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Question 1
Incorrect
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Who among the following prospective blood donors would be ineligible to donate whole blood or plasma?
Your Answer: A 42-year-old man with a history of hepatitis when he was aged 18 years
Correct Answer: A 32-year-old lady whose sister was diagnosed with Creutzfeldt-Jakob disease three years ago
Explanation:Blood Donation Guidelines
Blood donation guidelines set by the National Blood Service UK state that individuals who have a family member (parent or sibling) with Creutzfeldt-Jakob disease cannot donate blood. This is due to the risk of transmitting the disease through blood transfusion.
Other factors that may exclude individuals from donating blood include hepatitis or jaundice within the last 12 months, acupuncture within the last four months (unless performed by a registered professional), body piercing or tattoos within the last six months, any infection within the preceding two weeks, or a course of antibiotics within the last seven days.
It is important to follow these guidelines to ensure the safety of both the donor and the recipient. By excluding individuals who may have a higher risk of transmitting diseases or infections, the blood supply can remain safe and effective for those in need of transfusions.
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This question is part of the following fields:
- Haematology
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Question 2
Incorrect
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A 14-year-old girl presents with a history of easy bruising and excessive bleeding after a dental procedure. She is diagnosed with von Willebrand's disease and is scheduled for additional dental extractions. The physician prescribes DDAVP. What is the mechanism of action of DDAVP in treating von Willebrand's disease?
Your Answer: Acts as a substitute carrier for factor VIII
Correct Answer: Stimulates release of von Willebrand's factor from endothelial cells
Explanation:DDAVP for Increasing von Willebrand Factor
DDAVP is a medication that can be administered to increase the amount of von Willebrand factor in the body, which is necessary for surgical or dental procedures. This medication can increase plasma von Willebrand factor and factor VIII concentrations by two to five times. The mechanism of action involves the induction of cyclic adenosine monophosphate (cAMP)-mediated vWF secretion through a direct effect on endothelial cells. Overall, DDAVP is a useful tool for increasing von Willebrand factor levels in the body, allowing for safer and more successful surgical and dental procedures.
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This question is part of the following fields:
- Haematology
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Question 3
Correct
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A 35-year-old woman with type 1 diabetes and Addison's disease presents with a two-month history of increasing fatigue and shortness of breath during physical activity. She is currently using a progesterone-only oral contraceptive and taking hydrocortisone 10 mg twice daily and fludrocortisone 100 µg per day. Her glycaemic control has been reasonable, with a last HbA1c of 65 mmol/mol (20-46), and she is receiving mixed insulin twice daily. On examination, she appears pale. A full blood count reveals the following results: haemoglobin 52 g/L (120-160), MCV 115 fL (80-96), WCC 4.2 ×109/L (4-11), platelets 126 ×109L (150-400), and MCH 32 pg (28-32). The blood film shows multilobed nuclei in neutrophils and macrophages. What is the probable cause of her symptoms?
Your Answer: Vitamin B12 deficiency
Explanation:Megaloblastic Anemia and Pernicious Anemia
This patient is suffering from a macrocytic anemia, specifically a megaloblastic anemia, which is characterized by multilobed nuclei. The most probable cause of this condition is a deficiency in vitamin B12, which is commonly associated with pernicious anemia. Pernicious anemia is part of the autoimmune polyendocrine syndrome, which is linked to other autoimmune disorders such as Addison’s disease, type 1 diabetes, Sjögren’s disease, and vitiligo. Although there are other potential causes of macrocytosis, none of them are evident in this patient. Hypothyroidism, for example, does not cause megaloblastic anemia, only macrocytosis.
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This question is part of the following fields:
- Haematology
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Question 4
Incorrect
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A 17-year-old male patient comes in with a history of fevers, night sweats, and a weight loss of 20 pounds over the course of several months. Upon conducting a CT scan, mediastinal lymphadenopathy is discovered, and a biopsy is performed. The genetic analysis of the lesion reveals a translocation between chromosomes 14 and 18. What protein is expected to be overexpressed due to this translocation?
Your Answer: c-myc
Correct Answer: bcl-2
Explanation:Follicular lymphoma is a prevalent type of non-Hodgkin’s lymphoma that results from a chromosomal translocation between chromosome 14 and chromosome 18. This translocation causes the bcl-2 protein, which is anti-apoptotic, to be moved to the IgH promoter region, leading to overproduction of bcl-2. Consequently, abnormal B cells undergo clonal proliferation and are protected from apoptosis. Follicular lymphoma affects both genders equally, and its incidence increases with age. The disease typically presents with painless adenopathy that progresses over time. Systemic symptoms, such as fevers, night sweats, and weight loss, may occur later in the disease progression and can be associated with anaemia, thrombocytopenia, and lymphocytosis. Diagnosis requires a lymph node biopsy to demonstrate the expansion of follicles filling the node and chromosomal analysis of cells from bone marrow aspiration to detect t(14:18). Chemotherapy is the primary treatment, and rituximab, a monoclonal antibody against the CD20 protein found on B cells, is often used in combination with other agents. In Burkitt’s lymphoma, c-myc overexpression is caused by a translocation between chromosomes 8 and 14, while chronic myeloid leukaemia results from a translocation between chromosomes 9 and 22, forming the Philadelphia chromosome. Ewing’s sarcoma is caused by a translocation between chromosomes 11 and 12, leading to the formation of the fusion protein Ewsr1-fli1, which causes aberrant transcription of genes regulating cell growth and development. In a small subset of follicular lymphoma patients, translocations involving the bcl-6 gene and protein are found, which may increase the risk of transformation to a more aggressive form.
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This question is part of the following fields:
- Haematology
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Question 5
Incorrect
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A 67-year-old man presents to his General Practitioner with a 5-month history of night sweats and weight loss. He has no history of cough, shortness of breath or foreign travel. He denies any back or joint pain. He is normally fit and well, except for high blood pressure for which he takes enalapril. He continues to drink ten units of alcohol per week.
On examination, he is noted to have cervical and groin lymphadenopathy, with an enlarged spleen. The remainder of the examination and observations are normal.
Which of the following is most likely to be used in the management of this patient?Your Answer: Radiotherapy
Correct Answer: Rituximab
Explanation:The patient in question is displaying symptoms of non-Hodgkin’s lymphoma, including night sweats, weight loss, lymphadenopathy, and splenomegaly. While other symptoms may include pruritus, fever, and shortness of breath, the most common treatment for progressive non-Hodgkin’s lymphoma is a combination chemotherapy called R-CHOP, which includes rituximab. Rituximab is an anti-CD20 monoclonal antibody used for non-Hodgkin’s lymphoma and rheumatoid arthritis. The other options, including infliximab, lenalidomide, radiotherapy, and rifampicin, are used for different conditions such as Crohn’s disease, multiple myeloma, Hodgkin’s lymphoma, and tuberculosis, respectively.
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This question is part of the following fields:
- Haematology
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Question 6
Incorrect
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A 25-year-old patient presented with red rashes on their feet. Upon examination, they were found to be pale with purpuric spots on their lower legs. Their temperature was 38.3 °C and they also complained of nausea. On the second day of admission, their fever increased and they became disoriented. New bleeding spots started appearing on their face. Blood reports revealed low hemoglobin, high white cell count, low platelets, and high creatinine levels. A peripheral blood smear showed helmet cells and anisocytosis. The CSF study was normal. What test should be done next for this patient?
Your Answer:
Correct Answer: Urinary β-human chorionic gonadotrophin (hCG)
Explanation:The patient is presenting with thrombotic thrombocytopenic purpura (TTP), which is characterized by low platelet count due to clotting and platelet sequestration in small vessels. TTP is associated with haemolytic anaemia, thrombocytopenic purpura, fever, and neurological and renal abnormalities. The patient’s risk factors for TTP include being female, obese, pregnant, and of Afro-Caribbean origin. To determine the appropriate management, a urinary β-hCG test should be performed to establish pregnancy status. The first-line treatment for TTP is plasma exchange with fresh frozen plasma. Blood cultures should also be performed to check for underlying septicaemia. Antiplatelet antibody titres can be raised in idiopathic thrombocytopenic purpura (ITP), but ITP does not cause renal failure. A bone marrow study is appropriate to rule out leukaemia. Illicit drug use should also be considered as a cause of disseminated intravascular coagulation (DIC).
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This question is part of the following fields:
- Haematology
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Question 7
Incorrect
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The most common error in transfusion according to the SHOT (serious hazards of transfusion) analysis?
Your Answer:
Correct Answer: Wrong identification or mislabelling of patient or sample
Explanation:Common Causes of Transfusion Errors
Mislabelling of samples, requests, or misidentifying recipients are the most frequent causes of transfusion errors. This was confirmed by the SHOT study, which examined transfusion errors and near-misses in a nationwide audit in the United Kingdom. Although other errors, such as cross-match errors, incorrect storage, and transfusion reactions due to undetected antibodies, do occur, they are infrequent.
In summary, the SHOT study found that the most common causes of transfusion errors are related to labelling and identification. Therefore, it is crucial to implement strict protocols and procedures to ensure that samples and requests are correctly labelled and recipients are accurately identified to prevent these errors from occurring. While other errors may occur, they are rare and can be mitigated through proper training and adherence to established guidelines.
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This question is part of the following fields:
- Haematology
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Question 8
Incorrect
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A 5-year-old girl presents with purpura following a recent upper respiratory infection. Upon examination, her platelet count is found to be 20 ×109/L (normal range: 150-400) and a bone marrow examination reveals megakaryocyte hyperplasia. What is the correct statement regarding her condition?
Your Answer:
Correct Answer: A spontaneous remission is likely
Explanation:Treatment and Prognosis of Acute Temporary Thrombocytopenic Purpura
Acute temporary thrombocytopenic purpura is a condition that often occurs after a viral infection. Fortunately, 85% of children with this condition will recover within a year. Platelet transfusions are not helpful unless there is active bleeding or surgery is necessary. Instead, treatment typically involves immune suppression with medications like prednisolone or intravenous immune globulin infusions. The clotting time remains normal because the coagulation factors are not affected. However, detecting antiplatelet antibodies can be challenging with many assays. While splenectomy may be an option in some cases, it is not recommended early in the disease as it may resolve on its own within a year. Overall, with proper treatment and monitoring, most children with acute temporary thrombocytopenic purpura can expect a positive outcome.
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This question is part of the following fields:
- Haematology
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Question 9
Incorrect
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A middle-aged patient is newly diagnosed with a bleeding disorder after complaining of heavy periods. She presents with a prolonged APTT and bleeding time, but normal indices. The patient reports a family history of bleeding disorders on her mother's side. She denies any prior history of bleeding or bruising after minor surgical procedures. What is the most likely bleeding disorder in this patient?
Your Answer:
Correct Answer: von Willebrand’s disease
Explanation:The diagnosis in this scenario is von Willebrand’s disease, which is the most common hereditary bleeding disorder caused by a defective von Willebrand factor. This protein plays a crucial role in haemostasis by assisting in platelet adhesion and stabilising coagulation factor VIII. A deficiency in von Willebrand factor prolongs bleeding time and APTT, but does not affect platelet counts or PT. It is more pronounced in women and may present with menorrhagia. Treatment involves administration of recombinant von Willebrand factor. Haemophilia A, Bernard-Soulier syndrome, Glanzmann’s thrombasthenia, and vitamin K deficiency are other bleeding disorders with different causes and blood test results.
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This question is part of the following fields:
- Haematology
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Question 10
Incorrect
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Which statement about rhesus antibodies in pregnancy is correct?
Your Answer:
Correct Answer: Following delivery, the degree of fetomaternal haemorrhage should be calculated on a blood sample from a D negative mother
Explanation:Important Points to Remember about Fetomaternal Haemorrhage
Following the delivery of a baby, it is crucial to determine the degree of fetomaternal haemorrhage (FMH) in a D negative mother. This is done by analyzing a blood sample to adjust the dose of anti-D in the mother if she has delivered a D positive child. It is important to note that D positive and D negative women have the same likelihood of developing antibodies to other red cell antigens. Therefore, all pregnant women should undergo a blood group and antibody screen in their first trimester or at the time of presentation, whichever comes first. The fetal Rh type is determined by the Rh typing of both the mother and father. Additionally, maternal antibody titres are indicative of the degree of haemolytic disease of the newborn (HDN). For more information on the management of women with red cell antibodies during pregnancy, refer to the Royal College of Obstetricians and Gynaecologists (RCOG) Green-top Guideline No. 65.
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This question is part of the following fields:
- Haematology
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Question 11
Incorrect
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A 48-year-old man was admitted with unconsciousness.
On examination, his left plantar response was extensor, and deep tendon jerks were increased on the left side. A computerised tomography (CT) scan of the brain revealed a right-middle cerebral artery territory infarct. He was not known to have diabetes or hypertension. He was not receiving any drugs, either.
His blood count revealed:
Investigation Result Normal value
Haemoglobin 110 g/l 135–175 g/l
White cell count (WCC) 331 × 109/l 4–11 × 109/l
Neutrophil count 145 × 109/l 2.5–7.58 × 109/l
Metamyelocyte 3000/mm3
Platelet 490 × 109/l 150–400 × 109/l
Peripheral smear Many band forms, myelocytes, basophils
What is the next appropriate therapy?Your Answer:
Correct Answer: Leukapheresis
Explanation:Leukapheresis and Other Treatment Options for Chronic Myeloid Leukaemia with High White Blood Cell Count and Ischaemic Stroke
Chronic myeloid leukaemia can cause an extremely high white blood cell count, leading to hyperviscosity of the blood and an increased risk of ischaemic events such as stroke. While anticoagulation medications are important, they do not address the underlying issue of the high cell count. Leukapheresis is a procedure that can reduce the white cell volume by 30-60%, making it a crucial emergency treatment option. Other treatments, such as hydroxyurea and imatinib, can also be used to control disease burden. Imatinib is a tyrosine kinase inhibitor that is effective in treating chronic myeloid leukaemia with the Philadelphia chromosome translocation. Aspirin and heparin have limited roles in this scenario. While aspirin is recommended for long-term therapy after an ischaemic stroke, it does not address the hypercoagulable state caused by the high white blood cell count. Heparin is not used in the treatment of ischaemic strokes. Overall, leukapheresis should be the first step in emergency management for chronic myeloid leukaemia with a high white blood cell count and ischaemic stroke.
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This question is part of the following fields:
- Haematology
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Question 12
Incorrect
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A 32-year-old woman and her partner visit the clinic due to difficulty conceiving despite having regular sexual intercourse. The woman reports having a 28-day menstrual cycle, maintaining a normal diet, and not engaging in strenuous physical activity. During examination, her blood pressure is 122/72 mmHg, pulse is 68 and regular, and BMI is 24 without abnormalities found in her abdomen. What blood test would be most effective in determining if she is ovulating?
Your Answer:
Correct Answer: Progesterone
Explanation:Hormonal Tests for Ovulation and Pregnancy
In order to determine whether ovulation is occurring in a woman with a regular 28 day cycle, the most useful test is the measurement of day 21 progesterone levels. On the other hand, if a woman suspects she may be pregnant, a urinary pregnancy test can detect the presence of beta HCG hormone.
If a woman is experiencing absent periods and a pregnancy test is negative, measuring prolactin levels may be useful. This is especially true if there are other signs of hyperprolactinaemia, such as milk leakage on nipple stimulation.
It is important to note that oestrogen levels are not helpful in determining whether ovulation is occurring. However, if polycystic ovarian syndrome is suspected, measuring the LH/FSH ratio may be useful. By the different hormonal tests available, women can better monitor their reproductive health and seek appropriate medical attention when necessary.
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This question is part of the following fields:
- Haematology
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Question 13
Incorrect
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A 23-year-old female patient presents at the clinic for a check-up. She complains of irregular, heavy menstrual periods, has a BMI of 30 kg/m2, and experiences acne and excessive facial hair growth. She is not taking any medication. Her sister has been diagnosed with polycystic ovarian syndrome (PCOS), and she suspects that she may have the same condition.
During the physical examination, her blood pressure is 149/90 mmHg, and her pulse is regular at 78 bpm. She has excessive hair growth on her beard line and upper torso, and central obesity. However, the rest of her physical examination is normal.
Which blood test is the most diagnostic for PCOS?Your Answer:
Correct Answer: Total/free testosterone
Explanation:Diagnosis and Associated Risks of Polycystic Ovary Syndrome (PCOS)
Polycystic ovary syndrome (PCOS) is diagnosed when there is evidence of at least two out of three features, with other potential causes excluded. These features include oligoamenorrhoea, elevated levels of total/free testosterone (or clinical features suggestive of hyperandrogenism), or the presence of polycystic ovaries on ultrasound. While an extremely marked elevation in testosterone can suggest an androgen-secreting tumor, this is rare. Additionally, raised luteinising hormone (LH) with a normal follicle-stimulating hormone (FSH) can lead to an elevated LH/FSH ratio, but this is not diagnostic.
PCOS is associated with an increased risk of impaired glucose tolerance, although this may take a number of years to become apparent. Furthermore, there may be a reduction in levels of oestriol, although this is not always a consistent finding. It is important to diagnose PCOS early on to manage the associated risks and prevent potential complications.
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This question is part of the following fields:
- Haematology
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Question 14
Incorrect
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A 31-year-old man presented with weakness and fatigue. On examination, he was emaciated with a body weight of 40 kg. Blood tests revealed abnormalities including low haemoglobin, low MCV, low MCH, high platelet count, low albumin, and low calcium. His peripheral blood showed Howell-Jolly bodies. To which department should this patient be referred?
Your Answer:
Correct Answer: Gastroenterology Department
Explanation:Specialty Departments and Diagnosis of Coeliac Disease
The patient presents with microcytic, hypochromic anaemia, Howell-Jolly bodies, and splenic dysfunction, along with low albumin and calcium suggestive of malabsorption and emaciation. The most likely diagnosis is coeliac disease, which can be confirmed by antibody tests and a duodenal biopsy in the Gastroenterology department. Haematology can investigate the abnormal blood count, but treatment is not within their scope. Chronic kidney or liver disease is less likely, and there are no neurological symptoms.
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This question is part of the following fields:
- Haematology
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Question 15
Incorrect
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A frequent contributor complains of yellowing of the eyes and fever after donating blood for five days.
What should be the subsequent suitable step for the medical officer in charge of the blood bank?Your Answer:
Correct Answer: Recall blood products from this donor and arrange for retesting of this donor
Explanation:Managing Donor Complications and Blood Products
When a donor develops complications, it is important to assess how to manage both the donor and the blood products from the donation. In such cases, the blood products should be recalled until further testing and clarification of the donor’s illness. It is crucial to prevent the release of any of the blood products. However, the donor should not be immediately struck off the register until further testing results are available. It is important to take these precautions to ensure the safety of the blood supply and prevent any potential harm to recipients. Proper management of donor complications and blood products is essential to maintain the integrity of the blood donation system.
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This question is part of the following fields:
- Haematology
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Question 16
Incorrect
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A 65-year-old African-American has marked left-sided weakness and an expressive aphasia. He is diagnosed with a stroke. Blood results show the following:
Investigation Result Normal value
Haemoglobin (Hb) 90 g/l 135–175 g/l
Platelets 80 × 109/l 150–400 × 109/l
calcium 3.80 mmol/l 2.20–2.60 mmol/l
creatinine 128 µmol/l 50–120 µmol/l
Which investigation is likely to reveal the cause of this man’s stroke?Your Answer:
Correct Answer: Urinary Bence Jones proteins
Explanation:Investigations for a Patient with Stroke and Suspected Multiple Myeloma
When a patient presents with symptoms of stroke and possible multiple myeloma, several investigations may be necessary to confirm the diagnosis and determine the cause of the stroke. The mnemonic CRAB (high Calcium, Renal insufficiency, Anaemia and Bone lesions) can help identify the key symptoms of multiple myeloma.
One potential test is a urinary Bence Jones protein test, which can confirm the presence of multiple myeloma. However, this test alone cannot determine the cause of the stroke.
An MRI of the head may show areas of damage, but it will not reveal the cause of the stroke. A CT scan of the head, on the other hand, can identify changes caused by an ischaemic stroke, but it cannot differentiate the cause of the clot.
Liver function tests and X-rays of the spine are not first-line investigations for this condition, but they may be useful in identifying bone lesions associated with multiple myeloma.
Overall, a combination of tests and imaging may be necessary to diagnose and treat a patient with stroke and suspected multiple myeloma.
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This question is part of the following fields:
- Haematology
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Question 17
Incorrect
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A 15-year-old girl presents to the clinic with her mother, reporting an increase in abdominal size and no menstrual periods for the past three months. Despite denying any sexual activity, you suspect she may be pregnant. What is the specific measurement of a urinary pregnancy test?
Your Answer:
Correct Answer: Beta-HCG
Explanation:The Role of Hormone Assays in Confirming Pregnancy
Beta-HCG is a hormone produced by the placenta during pregnancy. There are highly sensitive assays available to detect the presence of beta-HCG, which can confirm pregnancy. In fact, some manufacturers of pregnancy tests claim that their tests are more accurate than ultrasound dating in determining gestation during the early stages of pregnancy.
While alpha-fetoprotein may also be elevated in pregnancy, particularly in cases of neural tube defects, it is not the primary focus of pregnancy testing. Hormone assays for oestrogen, progesterone, or testosterone levels are not reliable methods for confirming pregnancy. Therefore, beta-HCG remains the most reliable hormone to test for when confirming pregnancy.
It is important to note that while hormone assays can confirm pregnancy, they cannot determine the viability of the pregnancy or the presence of any complications. Ultrasound imaging and other diagnostic tests may be necessary to assess the health of the pregnancy and the developing fetus.
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This question is part of the following fields:
- Haematology
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Question 18
Incorrect
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A 65-year-old woman is two days postoperative, following a Hartmann’s procedure for bowel cancer. Her haemoglobin levels had dropped to 70 g/l, and as a result, she was started on a blood transfusion 12 hours ago. You are asked to review the patient, as she has suddenly become very agitated, pyrexial and hypotensive, with chest pain.
Which of the following transfusion reactions is most likely to be occurring in this patient?Your Answer:
Correct Answer: Acute haemolytic reaction
Explanation:An acute haemolytic reaction is a transfusion complication that can occur within 24 hours of receiving blood. It is often caused by ABO/Rh incompatibility and can result in symptoms such as agitation, fever, low blood pressure, flushing, pain in the abdomen or chest, bleeding from the site of the venepuncture, and disseminated intravascular coagulation (DIC). Treatment involves stopping the transfusion immediately. Iron overload, hepatitis B infection, graft-versus-host disease (GvHD), and human immunodeficiency virus (HIV) infection are all delayed transfusion reactions that may present after 24 hours.
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This question is part of the following fields:
- Haematology
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Question 19
Incorrect
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A 40-year-old man presents to his GP after discovering a low haemoglobin level during a routine blood donation. He has been experiencing fatigue and breathlessness during mild exertion for the past few weeks. He has donated blood twice before, with the most recent donation being a year ago. He has been taking 30 mg lansoprazole daily for several years to manage his acid reflux, which is well controlled. He also takes cetirizine for hay fever. He denies any nausea, vomiting, changes in bowel habits, or blood in his stools or urine. His diet is diverse, and he is not a vegetarian or vegan. Physical examinations of his chest and abdomen are normal, and urinalysis is unremarkable. The following are his blood test results:
- Haemoglobin: 100 g/l (normal range: 135-175 g/l)
- Mean corpuscular volume (MCV): 72.0 fl (normal range: 82-100 fl)
- White cell count (WCC): 6.1 × 109/l (normal range: 4-11 × 109/l)
- Platelets: 355 × 109/l (normal range: 150-400 × 109/l)
- Ferritin: 6.0 µg/l (normal range: 20-250 µg/l)
- Immunoglobulin A (IgA) tissue transglutaminase antibody (tTGA) is negative, and IgA level is normal.
What is the most appropriate initial management step?Your Answer:
Correct Answer: Referral to gastroenterology
Explanation:Management of Unexplained Microcytic Anemia with Low Ferritin
Unexplained microcytic anemia with low ferritin levels requires prompt investigation to identify the underlying cause. According to National Institute for Health and Care Excellence (NICE) guidelines, men with unexplained iron deficiency anemia and a hemoglobin level below 110 g/l should be urgently referred for upper and lower gastrointestinal investigations, regardless of age. A trial of oral iron may be appropriate in pregnant women or premenopausal women with a history of menorrhagia and without gastrointestinal symptoms or a family history of gastrointestinal cancer.
A faecal occult blood test is not recommended as it has poor sensitivity and specificity. Referral to haematology is not necessary as first-line investigations would be upper and lower gastrointestinal investigations, and thus a referral to gastroenterology would be warranted. It is important to rule out blood loss, in particular, through gastrointestinal investigations, before implicating poor dietary intake as the cause of the patient’s low iron stores and microcytic anemia.
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This question is part of the following fields:
- Haematology
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Question 20
Incorrect
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A 68-year-old woman presents with lethargy and poor exercise tolerance. She also mentions that, over the last month, she has had excessive thirst and polyuria.
Initial investigations show:
Investigation Result Normal value
Haemoglobin (Hb) 78 g/l 115–155 g/l
Erythrocyte sedimentation rate (ESR) 109 mm/h 0–10mm in the first hour
Sodium (Na+) 134 mmol/l 135–145 mmol/l
Potassium (K+) 5.8 mmol/l 3.5–5.0 mmol/l
Urea 26.5 mmol/l 2.5–6.5 mmol/l
Creatinine 268 µmol/l 50–120 μmol/l
Corrected calcium (Ca2+) 3.02 mmol/l 2.20–2.60 mmol/l
Glucose 5.2 mmol/l 3.5–5.5 mmol/l
Which of the following tests is the most appropriate to confirm the underlying diagnosis?Your Answer:
Correct Answer: Serum and urine electrophoresis
Explanation:Diagnostic Tests and Differential Diagnosis for a Patient with Multiple Derangements
The patient in question presents with several abnormalities in their blood tests, including anaemia, hypercalcaemia, electrolyte imbalances, and a significantly elevated ESR. These findings, along with the patient’s symptoms, suggest a diagnosis of malignancy, specifically multiple myeloma.
Multiple myeloma is characterized by the malignant proliferation of plasma cells, leading to bone marrow infiltration, pancytopenia, osteolytic lesions, hypercalcaemia, and renal failure. The ESR is typically elevated in this condition. To confirm a diagnosis of multiple myeloma, serum and urine electrophoresis can be performed to identify the presence of monoclonal antibodies and Bence Jones proteins, respectively. Bone marrow examination can also reveal an increased number of abnormal plasma cells.
Treatment for multiple myeloma typically involves a combination of chemotherapy and bisphosphonate therapy, with radiation therapy as an option as well. This condition is more common in men, particularly those in their sixth or seventh decade of life.
Other diagnostic tests that may be considered include an oral glucose tolerance test (to rule out diabetes as a cause of polydipsia and polyuria), a chest X-ray (to evaluate for a possible small cell carcinoma of the lung), and an abdominal CT scan (to assess the extent of disease and the presence of metastasis). A serum PTH level may also be useful in ruling out primary hyperparathyroidism as a cause of hypercalcaemia, although the patient’s symptoms and blood test results make malignancy a more likely diagnosis.
Diagnostic Tests and Differential Diagnosis for a Patient with Multiple Derangements
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This question is part of the following fields:
- Haematology
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Question 21
Incorrect
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A 67-year-old man presented with fever and body ache for 6 months. His blood tests revealed a haemoglobin level of 110 g/l and erythrocyte sedimentation rate (ESR) of 121 mm in the first hour. Serum protein electrophoresis revealed an M band in the gamma globulin region with a total IgG level of 70 g/l. Bone marrow biopsy shows plasma cells in the marrow of 11%. A skeletal survey reveals no abnormalities. Other blood tests revealed:
Test Parameter Normal range
Calcium 2.60 mmol/l 2.20–2.60 mmol/l
Creatinine 119 μmol/l 50–120 μmol/l
Phosphate 1.30 mmol/l 0.70–1.40 mmol/l
Potassium (K+) 4.6 mmol/l 3.5–5.0 mmol/l
Lactate dehydrogenase 399 IU/l 100–190 IU/l
His body weight was 80 kg.
What is his condition better known as?Your Answer:
Correct Answer: Smouldering myeloma
Explanation:Smouldering myeloma is a stage between monoclonal gammopathy of unknown significance (MGUS) and myeloma. To diagnose this condition, the patient must have a monoclonal protein in the serum of at least 30 g/l and monoclonal plasma cells of at least 10% in bone marrow or tissue biopsy, but no evidence of end-organ damage. Patients with smouldering myeloma should be closely monitored as they are at high risk of developing symptomatic myeloma.
Multiple myeloma is a malignant neoplasm where there is clonal proliferation of plasma cells in the bone marrow, leading to the secretion of a monoclonal antibody and light immunoglobulin chains that cause organ damage. Patients with multiple myeloma present with various symptoms, including lethargy, bone pain, pathological fractures, renal impairment, amyloidosis, and pancytopenia due to marrow infiltration. To diagnose multiple myeloma, the patient must have a monoclonal antibody in serum and/or urine, clonal plasma cells of at least 10% on bone marrow biopsy, and evidence of end-organ damage.
MGUS is a condition where low levels of paraprotein are detected in the blood, but they are not causing clinically significant symptoms or end-organ damage. To diagnose MGUS, the patient must have a monoclonal protein in the serum of less than or equal to 30 g/l, monoclonal plasma cells of less than or equal to 10% in bone marrow or tissue biopsy, and no evidence of end-organ damage.
Non-secretory myeloma is a rare variant of multiple myeloma where the bone marrow findings and end-organ damage are similar to myeloma, but there is no detectable monoclonal protein in the serum or urine. This makes it difficult to diagnose.
Plasma cell leukemia is a rare and aggressive form of multiple myeloma characterized by high levels of plasma cells circulating in the peripheral blood. It can occur as a primary condition or a secondary leukaemic transformation of multiple myeloma.
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This question is part of the following fields:
- Haematology
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Question 22
Incorrect
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In the monophyletic model of haematopoiesis, which mature cell in the peripheral circulation is a derivative of the CMP? This model suggests that the haematopoietic stem cell (HSC) can give rise to either the common lymphoid progenitor (CLP) or the common myeloid progenitor (CMP).
Your Answer:
Correct Answer: Monocyte
Explanation:Different Types of Blood Cells and their Progenitor Cells
Blood cells are formed from different types of progenitor cells. The common myeloid progenitor cell gives rise to myeloblasts, pro-erythroblasts, megakaryoblasts, and monoblasts. Myeloblasts produce granulocytes, while pro-erythroblasts produce red blood cells. Megakaryoblasts give rise to megakaryocytes and platelets, and monoblasts produce monocytes that can become tissue-specific macrophages. B cells, NK cells, and T cells are derivatives of the common lymphoid progenitor cell. Plasma cells, which are antibody-secreting cells, are derived from B cells. Understanding the different types of blood cells and their progenitor cells is important for studying blood disorders and developing treatments.
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This question is part of the following fields:
- Haematology
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Question 23
Incorrect
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A 30-year-old donor experiences a sudden fainting spell during blood donation. What should be the immediate course of action to manage this adverse event?
Your Answer:
Correct Answer: Temporarily cease the donation, consider fluid replacement and elevate the donor's legs
Explanation:Donor safety is important in blood donation. Fainting is a common adverse event and should be treated by stopping the donation and reviving the donor. Elevation of the legs and monitoring vitals is necessary. Donors should be counseled on pre-donation expectations and encouraged to drink fluids after recovery. Hemoglobin checks are no longer required.
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This question is part of the following fields:
- Haematology
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Question 24
Incorrect
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A 75-year-old woman who is in hospital for pneumonia begins to deteriorate on her third day of intravenous antibiotics. She develops purple bruises on her skin and on the inside of her mouth, and tells you that she feels short of breath and fatigued. Her platelet count has dropped from 165 × 109/l to 43 × 109/l over the last two days. She also complains of blurred vision in the last few hours.
Which of the following will form part of her initial management?Your Answer:
Correct Answer: Plasma exchange
Explanation:Treatment Options for Thrombotic Thrombocytopenic Purpura
Thrombotic thrombocytopenic purpura (TTP) is a medical emergency that requires prompt treatment. The most common initial management for TTP is plasma exchange, which aims to remove the antibodies that block the ADAMTS13 enzyme and replace the ADAMTS13 enzymes in the blood. Intravenous methylprednisone and rituximab may also be used in conjunction with plasma exchange.
Aspirin should only be considered when the platelet count is above 50 × 109/l, and even then, it is not an essential part of initial management and will depend on the patient’s comorbidities. Cryoprecipitate is not recommended for TTP treatment, as it is indicated for disseminated intravascular coagulation or fibrinogen deficiency.
Factor VIII infusion is used for haemophilia A, a C-linked-recessive disorder that presents with excessive bleeding and anaemia, and is less likely to be associated with thrombocytopenia and TTP. Platelet transfusions are relatively contraindicated in TTP and should only be considered in cases of catastrophic bleeding or urgent surgery that cannot wait until after plasma exchange. Platelet transfusions increase the risk of arterial thrombosis, which can lead to myocardial infarction and stroke.
In summary, plasma exchange is the most common initial management for TTP, and other treatment options should be carefully considered based on the patient’s individual circumstances. Early diagnosis and prompt treatment are crucial for a successful outcome.
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This question is part of the following fields:
- Haematology
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Question 25
Incorrect
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A 9-year-old boy presents to the clinic with complaints of fatigue and weight loss. Upon examination, he is found to be pale and has hepatosplenomegaly. A complete blood count shows the presence of immature white cells. What is the most probable diagnosis?
Your Answer:
Correct Answer: Leukaemia
Explanation:Probable Diagnosis of Acute Lymphoblastic Leukaemia in a Child
This child is likely to have acute lymphoblastic leukaemia (ALL) based on the presence of immature white cells on their full blood count (FBC). Hodgkin’s disease is unlikely as the patient is too young and typically presents with lymphadenopathy. HIV is also not a probable cause of the immature cells on the FBC.
Acute lymphoblastic leukaemia is a type of cancer that affects the white blood cells, specifically the lymphocytes. It is most commonly diagnosed in children and young adults. Symptoms may include fatigue, fever, and easy bruising or bleeding. Treatment typically involves chemotherapy and may also include radiation therapy or stem cell transplantation. Early diagnosis and treatment are important for improving outcomes in patients with ALL.
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This question is part of the following fields:
- Haematology
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Question 26
Incorrect
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In a 71-year-old man who is asymptomatic and attending clinic for an annual review, a full blood count result shows a mild lymphocytosis of 15 × 109/l with a few smear cells. What is the most crucial investigation to determine a diagnosis of chronic lymphocytic leukaemia (CLL)?
Your Answer:
Correct Answer: Peripheral blood flow cytometry
Explanation:Diagnosis and Staging of Chronic Lymphocytic Leukemia
Chronic lymphocytic leukemia (CLL) can be diagnosed through flow cytometry, which shows a specific pattern of monoclonal B cell proliferation. This pattern includes CD19/5 coexpressing, CD23 positive, and light chain restricted B cell population. However, smear cells, which are fragile lymphocytes that are smeared on the glass slide, can also be present in other lymphoproliferative disorders and benign lymphocytosis. Therefore, they do not necessarily indicate CLL.
While CT scan and LDH are not essential for diagnosis, they are necessary for staging CLL. These investigations help determine the extent of the disease and the organs affected. Additionally, cervical lymphadenopathy, which is the enlargement of lymph nodes in the neck, may be present in CLL. However, it can also be seen in other causes of lymphadenopathy, such as viral infections or adenopathy secondary to local dental infection.
In summary, flow cytometry is a crucial tool in diagnosing CLL, while CT scan and LDH are necessary for staging. Smear cells may be present but do not necessarily indicate CLL, and cervical lymphadenopathy can be seen in various conditions.
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This question is part of the following fields:
- Haematology
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Question 27
Incorrect
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A 35-year-old man visits the clinic seeking guidance. He has planned a long-distance trip to Australia and is worried about the risk of deep vein thrombosis (DVT). He has no significant medical history and is in good health.
What is the best advice to offer him?Your Answer:
Correct Answer: No aspirin, ankle exercises, aisle seat and no alcohol
Explanation:Venous Thromboembolism Prophylaxis for Long-Haul Flights
When it comes to preventing venous thromboembolism (VTE) during long-haul flights, the approach varies depending on the patient’s risk level. For low-risk patients without history of VTE, cardiac disease, major illness, or recent surgery, NICE recommends avoiding long periods of immobility by taking an aisle seat, performing ankle exercises, and wearing loose-fitting clothing. It’s also important to stay hydrated and avoid alcohol.
Moderate to high-risk patients, on the other hand, may benefit from compression stockings, especially if they have a history of VTE, cardiac disease, varicose veins, or are pregnant or postpartum. High-risk patients with thrombophilia, cancer, or recent surgery requiring general anesthesia should also consider compression stockings.
While some sources recommend a single dose of aspirin for VTE prophylaxis during long-haul flights, current NICE guidelines do not recommend this approach. Instead, patients should focus on staying mobile, wearing compression stockings if necessary, and staying hydrated.
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This question is part of the following fields:
- Haematology
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Question 28
Incorrect
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Which autoantibody is correctly matched with its associated disease in the following list?
Your Answer:
Correct Answer: Pernicious anaemia and anti-intrinsic factor antibodies
Explanation:Autoimmune Disorders and Associated Antibodies
Autoimmune disorders occur when the immune system mistakenly attacks healthy cells and tissues in the body. These disorders are often associated with the presence of specific antibodies that can help diagnose and monitor the disease. Here are some examples:
Pernicious Anaemia and Anti-Intrinsic Factor Antibodies
Pernicious anaemia is a type of anaemia caused by a deficiency in vitamin B12. It is associated with the presence of anti-intrinsic factor antibodies, which bind to intrinsic factor and prevent the absorption of vitamin B12 in the gut.Primary Biliary Cholangitis and Anti-Jo-1 Antibodies
Primary biliary cholangitis is an autoimmune disorder that affects the liver. It is associated with the presence of anti-mitochondrial antibodies, but not anti-Jo-1 antibodies, which are associated with other autoimmune disorders like polymyositis and dermatomyositis.Myasthenia Gravis and Voltage-Gated Calcium Channel Antibodies
Myasthenia gravis is a neuromuscular disorder that causes muscle weakness and fatigue. It is associated with the presence of anti-acetylcholine receptor antibodies, but not anti-striated muscle antibodies, which are found in other autoimmune disorders.Granulomatosis with Polyangiitis (GPA) and Anti-Myeloperoxidase (p-ANCA) Antibody
GPA is a type of vasculitis that affects small and medium-sized blood vessels. It is associated with the presence of cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), but not p-ANCA, which are found in other types of vasculitis.Hashimoto’s Thyroiditis and Thyroid-Stimulating Antibodies
Hashimoto’s thyroiditis is an autoimmune disorder that affects the thyroid gland. It is associated with the presence of anti-thyroglobulin and anti-thyroperoxidase antibodies, which attack the thyroid gland and cause inflammation. -
This question is part of the following fields:
- Haematology
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Question 29
Incorrect
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A 54-year-old white woman without past medical history presents with pallor, shortness of breath, palpitations and difficulty balancing.
On examination, her vitals are heart rate 110 bpm at rest and 140 bpm on ambulation, blood pressure 100/60 mmHg, respiratory rate 18 breaths/minute, temperature 37 ° C and oxygen saturation 98% on room air. She is pale. Her lungs are clear to auscultation; her heart rate is regular without murmurs, rubs or gallops; her abdomen is soft and non-tender; she is moving all extremities equally, and a stool guaiac test is heme-negative. Her gait is wide and she has difficulty balancing. She has decreased sensation to fine touch in her feet. Her mini-mental status exam is normal.
Blood work shows:
Haematocrit: 0.19 (0.35–0.55)
Mean cell volume: 110 fl (76–98 fl)
White blood cell count: 5 × 109/l (4–11 × 109/l)
Which one of the following findings would most likely lead to the correct diagnosis?Your Answer:
Correct Answer: Anti-intrinsic factor antibodies
Explanation:Causes and Symptoms of Vitamin B12 Deficiency
Vitamin B12 deficiency can lead to macrocytic anaemia and neurological symptoms. The most common cause of this deficiency is the presence of anti-intrinsic factor antibodies. Intrinsic factor is necessary for the absorption of dietary vitamin B12 in the terminal ileum. Without it, vitamin B12 cannot be absorbed, leading to deficiency and anaemia. Symptoms of vitamin B12 deficiency include fatigue, lethargy, dyspnoea on exertion, and neurological symptoms such as peripheral loss of vibration and proprioception, weakness, and paraesthesiae. If left untreated, it can lead to hepatosplenomegaly, heart failure, and demyelination of the spinal cord, causing ataxia.
Diagnosis can be made with a vitamin B12 level test, which reveals anaemia, often pancytopenia, and a raised MCV. A blood film reveals hypersegmented neutrophils, megaloblasts, and oval macrocytes. Treatment involves replacement of vitamin B12.
Other possible causes of vitamin B12 deficiency include intestinal tapeworm, which is rare, and gastrointestinal malignancy, which causes iron deficiency anaemia with a low MCV. Destruction of the anterior and lateral horns of the spinal cord describes anterolateral sclerosis (ALS), which is characterised by progressive muscle weakness and would not cause anaemia or loss of sensation. Enlargement of the ventricles on head CT indicates hydrocephalus, which could explain the wide-based gait but not the anaemia and other symptoms. A haemoglobin A1c of 12.2% is associated with diabetes, which could explain decreased peripheral sensation to fine touch but would not be associated with megaloblastic anaemia.
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This question is part of the following fields:
- Haematology
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Question 30
Incorrect
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A patient on the ward receiving a blood transfusion after knee replacement surgery is noted to have rigors.
On examination, their temperature is 39°C (baseline 36.5°C). They also complain of abdominal pain and their blood pressure is 90/55 mmHg (baseline 126/84 mmHg).
What is the next step in managing this patient?Your Answer:
Correct Answer: Stop the transfusion immediately and commence IV fluids
Explanation:Management of Acute Haemolytic Transfusion Reaction
When a patient experiences a temperature rise of more than 2°C, abdominal pain, and hypotension after a blood transfusion, an acute haemolytic transfusion reaction should be suspected. In such cases, the transfusion must be stopped immediately, and the set should be taken down. Saline infusion should be initiated to maintain the patient’s blood pressure.
The blood bank should be notified of the suspected reaction, and a sample may need to be collected for further investigation. However, the priority is to manage the patient’s symptoms and prevent further complications. If the reaction is severe, the transfusion should not be continued.
In summary, prompt recognition and management of acute haemolytic transfusion reactions are crucial to prevent serious complications. Healthcare providers should be vigilant in monitoring patients who receive blood transfusions and act quickly if any adverse reactions occur.
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This question is part of the following fields:
- Haematology
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Question 31
Incorrect
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A 43-year-old woman was diagnosed with acute myeloid leukaemia (AML) with 71% of bone marrow blasts. She declined bone marrow transplant and was started on appropriate chemotherapy. After 2 months, a repeat bone marrow revealed 8% of blasts. Peripheral blood was blast-free and blood tests revealed:
Investigation Result Normal value
Haemoglobin 106 g/l 115–155 g/l
White cell count (WCC) 8.1 × 109/l 4–11 × 109/l
Neutrophils 5.2 × 109/l 2.5–7.58 × 109/l
Lymphocytes 1.8 × 109/l 1.5–3.5 × 109/l
Platelets 131 × 109/l 150–400 × 109/l
What is her clinical status?Your Answer:
Correct Answer: Partial remission
Explanation:Partial remission occurs when a patient meets all the criteria for complete remission except for having more than 5% bone marrow blasts. To be diagnosed with partial remission, the blast cells can be between 5% and 25% and must have decreased by at least 50% from their levels before treatment.
Complete remission is achieved when a patient meets specific criteria, including having a neutrophil count of over 1.0 × 109/l and a platelet count of over 100 × 109/l, not requiring red cell transfusions, having normal cellular components on bone marrow biopsy, having less than 5% blasts in the bone marrow without Auer rods present, and having no signs of leukemia anywhere else in the body.
Complete remission with incomplete recovery is when a patient meets all the criteria for complete remission except for continuing to have neutropenia or thrombocytopenia.
Resistant disease occurs when a patient fails to achieve complete or partial remission and still has leukemia cells in their peripheral blood or bone marrow seven days after completing initial therapy.
A morphologic leukemia-free state is when a patient has less than 5% bone marrow blasts without blasts with Auer rods present and no extramedullary disease, but they do not meet the criteria for neutrophils, platelets, and blood transfusions.
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This question is part of the following fields:
- Haematology
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Question 32
Incorrect
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By how much does the haemoglobin concentration increase with the infusion of one unit of fresh blood?
Your Answer:
Correct Answer: 10 g/L
Explanation:The Effect of Fresh Blood on Haemoglobin Levels
When one unit of fresh blood is transfused, it increases the haemoglobin levels in the body by approximately 10 g/L. This is equivalent to the effect of one unit of red cell concentrate. Both fresh blood and red cell concentrate contain red blood cells, which are responsible for carrying oxygen throughout the body. Therefore, the increase in haemoglobin levels is due to the additional red blood cells that are introduced into the bloodstream. This information is important for medical professionals who need to monitor and manage the haemoglobin levels of their patients, particularly those who have undergone significant blood loss or have conditions that affect their red blood cell count.
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This question is part of the following fields:
- Haematology
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Question 33
Incorrect
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A 70-year-old man presents with fatigue, pallor and shortness of breath. He has been battling with an indolent colon carcinoma for the past 5 years. He also suffers from insulin-dependent diabetes, hypertension, coronary artery disease and rheumatoid arthritis. He has been feeling unwell for the past few weeks. He denies any history of melaena or haematochezia and has been amenorrhoeic for decades. A bedside stool guaiac test is negative for any blood in the stool. He is well nourished, reports taking daily supplements and is not a vegetarian. He reports that his haematocrit is 0.28 (0.35–0.55) and haemoglobin level 100 g/l (115–155 g/l).
What additional findings would you expect to observe in his full blood count?Your Answer:
Correct Answer: Increased ferritin
Explanation:Understanding Anaemia of Chronic Disease: Increased Ferritin and Decreased TIBC
Anaemia of chronic disease is a type of anaemia that is commonly seen in patients with chronic inflammatory conditions. It is characterised by a low haemoglobin level and low haematocrit, but unlike iron deficiency anaemia, it is associated with increased ferritin levels and decreased total iron-binding capacity (TIBC). This is because ferritin is a serum reactive protein that is elevated in response to the underlying inflammatory process.
Diagnosis of anaemia of chronic disease requires the presence of a chronic inflammatory condition and anaemia, which can be either normocytic or microcytic. It is important to note that a haemoglobin level of <80 g/l is very rarely associated with this type of anaemia. Treatment involves addressing the underlying disorder causing the anaemia and monitoring the haemoglobin level. Blood transfusion is only used in severe cases. It is important to differentiate anaemia of chronic disease from other types of anaemia. For example, it is characterised by a low reticulocyte count, and not reticulocytosis. Serum transferrin receptor is not affected in anaemia of chronic disease and would therefore be normal. Additionally, TIBC is reduced in anaemia of chronic disease, whereas it is increased in iron deficiency anaemia. Finally, anaemia of chronic disease is associated with either microcytosis or normocytosis, whereas macrocytosis is associated with other types of anaemia such as folate deficiency, vitamin B12 deficiency, alcohol excess, and myelodysplastic disease. In summary, understanding the unique features of anaemia of chronic disease, such as increased ferritin and decreased TIBC, can aid in its diagnosis and management.
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This question is part of the following fields:
- Haematology
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Question 34
Incorrect
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A 14-year-old boy with thalassaemia major, receiving regular blood transfusions, has been added to the transplant waiting list for chronic heart failure. What is the probable reason for his heart failure?
Your Answer:
Correct Answer: Transfusion haemosiderosis
Explanation:Complications of Blood Transfusions: Understanding the Risks
Blood transfusions are a common medical intervention used to treat a variety of conditions, from severe bleeding to anaemia. While they can be life-saving, they also carry certain risks and potential complications. Here are some of the most common complications associated with blood transfusions:
Transfusion haemosiderosis: Repeated blood transfusions can lead to the accumulation of iron in the body’s organs, particularly the heart and endocrine system. This can cause irreversible heart failure if left untreated.
High-output cardiac failure: While anaemia on its own may not be enough to cause heart failure, it can exacerbate the condition in those with reduced left ventricular systolic dysfunction.
Acute haemolytic transfusion reaction: This occurs when there is a mismatch between the major histocompatibility antigens on blood cells, such as the ABO system. It can cause severe intravascular haemolysis, disseminated intravascular coagulation, renal failure, and shock, and has a high mortality rate if not recognized and treated quickly.
Pulmonary oedema: While rare in patients with normal left ventricular systolic function, blood transfusions can cause fluid overload and pulmonary oedema, which can exacerbate chronic heart failure.
Transfusion-related bacterial endocarditis: While rare, bacterial infections can occur from blood transfusions. Platelet pools, which are stored at room temperature, have a slightly higher risk of bacterial contamination that can cause fulminant sepsis.
Understanding the potential complications of blood transfusions is important for both patients and healthcare providers. By recognizing and addressing these risks, we can ensure that blood transfusions remain a safe and effective treatment option for those who need them.
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This question is part of the following fields:
- Haematology
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Question 35
Incorrect
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What is the correct information regarding the storage requirements and lifespan of blood products?
Your Answer:
Correct Answer: Fresh frozen plasma is stored at −25°C for up to 36 months
Explanation:Storage Guidelines for Blood Products
Blood products such as fresh frozen plasma, red cells, and platelets have specific storage guidelines to ensure their safety and efficacy. Fresh frozen plasma can be stored for up to 36 months at a temperature of −25°C. On the other hand, red cells are stored at a temperature of 4°C for a maximum of 35 days, while platelets are stored at a temperature of 22°C for up to 5 days on a platelet shaker/agitator.
These guidelines are important to follow to maintain the quality of blood products and prevent any adverse reactions in patients who receive them. It is crucial to store blood products at the appropriate temperature and for the recommended duration to ensure their effectiveness when used in transfusions. Healthcare professionals should be aware of these guidelines and ensure that they are followed to provide safe and effective blood transfusions to patients.
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This question is part of the following fields:
- Haematology
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Question 36
Incorrect
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A 28-year-old woman on vacation complained of a swollen left calf. She mentioned having the 'factor V Leiden mutation'. Can you explain the pathophysiology of this mutation?
Your Answer:
Correct Answer: Activated protein C resistance
Explanation:Factor V Leiden: A Genetic Condition Affecting Blood Clotting
Factor V is a protein that acts as a cofactor to allow the generation of an enzyme called thrombin, which is responsible for cleaving fibrinogen to fibrin. This process leads to the formation of a dense meshwork that makes up the majority of a clot. Activated protein C (aPC) is a natural anticoagulant that limits clotting by degrading factor V. However, in individuals with Factor V Leiden, a genetic condition that is inherited in an autosomal dominant manner, the coagulation factor cannot be destroyed by aPC.
Factor V Leiden is caused by a single nucleotide substitution of adenine for guanine in the gene encoding factor V. This mutation changes the protein’s 506th amino acid from arginine to glutamine, which prevents efficient inactivation of factor V. As a result, factor V remains active, leading to overproduction of thrombin and excess fibrin generation, which in turn causes excess clotting.
In summary, Factor V Leiden is a genetic condition that affects blood clotting by preventing the efficient inactivation of factor V. This leads to excess clotting, which can increase the risk of developing blood clots and related complications.
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This question is part of the following fields:
- Haematology
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Question 37
Incorrect
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A 10-year-old Afro-Caribbean boy has been brought to the paediatric Emergency Department by his parents, both of whom are known to suffer from sickle cell disease. They have brought him in to see you because they are worried he has developed ‘septicaemia’. Upon further questioning, he reveals that he has developed extreme fatigue, shortness of breath on exertion, coryzal symptoms and bleeding gums, all over the last two days.
Upon examination, you note an erythematosus rash on both cheeks, a small purpuric rash on the left arm, pale conjunctivae, pale skin and well-perfused peripheries. Brudzinski’s sign is negative.
You take some basic observations, which are as follows:
Temperature: 37.8 ˚C
Heart rate: 100 bpm (normal 55–85 bpm)
Respiratory rate: 20 breaths/min (normal 12–18 breaths/min)
Blood pressure: 130/86 mmHg (lying), 132/84 mmHg (standing)
Oxygen saturation: 98% on room air
His initial investigation findings are as follows:
Investigation Result Normal
White cell count (WCC) 11.4 × 109/l 4–11 × 109/l
Neutrophils 3800 × 106/l 3000–5800 × 106/l
Lymphocytes 7200 × 106/l 1500–3000 × 106/l
Haemoglobin (Hb) 84 g/dl 135–175 g/l
Mean corpuscular volume
(MCV) 94 fl 76–98 fl
Platelets 200 × 109/l 150–400 × 109/l
Given the likely diagnosis, how should the patient be managed?Your Answer:
Correct Answer: Cross-match, giving blood as soon as it is available
Explanation:The patient in question is at a high risk of sickle cell disease due to their ethnicity and family history. They are showing signs of parvovirus B19 infection, which is causing bone marrow failure and a decrease in erythropoiesis. This condition, known as aplastic crisis, is usually managed conservatively but may require a blood transfusion if the patient is experiencing symptomatic anemia. Granulocyte colony-stimulating factor (G-CSF) is not recommended in this case as it will not address the patient’s severe anemia. IV ceftriaxone and a lumbar puncture would be the correct initial management for meningococcal disease, but it is not the most likely diagnosis in this case. Oral benzylpenicillin and transfer to a pediatric ward is also not recommended as it is not the correct management for meningococcal disease and is not relevant to the patient’s condition. While sepsis is a possible differential diagnosis, the most likely cause of the patient’s symptoms is a viral infection causing aplastic crisis in a patient with sickle cell disease. Therefore, the appropriate management would be to investigate for viral infection and provide supportive therapies.
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This question is part of the following fields:
- Haematology
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Question 38
Incorrect
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A 50-year-old male has a history of severe rheumatoid arthritis for the past four years. Despite being compliant with therapy (NSAIDs and methotrexate), the disease remains poorly controlled. Recently, he has been experiencing extreme fatigue. Upon conducting an FBC, the following results were obtained:
- Haemoglobin 70 g/L (120-160)
- White cell count 1.5 ×109/L (4-11)
- Platelet count 40 ×109/L (150-400)
What could be the possible cause of his pancytopenia?Your Answer:
Correct Answer: Methotrexate
Explanation:Pancytopenia in a Patient with Erosive Rheumatoid Arthritis
This patient is showing signs of pancytopenia, a condition where there is a decrease in all three blood cell types (red blood cells, white blood cells, and platelets). Given her history of erosive rheumatoid arthritis for the past three years, it is likely that she has been on immunosuppressive therapy, which can lead to this type of blood disorder.
Immunosuppressive drugs such as methotrexate, sulfasalazine, penicillamine, and gold can all have an impact on blood cell production and lead to pancytopenia. It is important to monitor patients on these medications for any signs of blood disorders and adjust treatment accordingly. Early detection and management can prevent further complications and improve patient outcomes.
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This question is part of the following fields:
- Haematology
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Question 39
Incorrect
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A 45-year-old Afro-Caribbean man presents to the Emergency Department with acute severe chest pain, fever and a cough, which he has had for five days. Examination revealed signs of jaundice and the spleen was not big enough to be palpable.
You take some basic observations:
Temperature: 38 °C
Respiratory rate: 26 breaths/min
O2 saturation: 86%
Heart rate: 134 bpm (regular)
Blood pressure (lying): 134/86 mmHg
Blood pressure (standing): 132/90 mmHg
His initial investigation findings are as follows:
Investigation Result Normal
White cell count (WCC) 13.8 × 109/l 4–11.0 × 109/l
Neutrophils 7000 × 106/l 3000–5800 × 106/l
Lymphocytes 2000 × 106/l 1500–3000 × 106/l
Haemoglobin (Hb) 105 g/l 135–175 g/l
Mean corpuscular volume (MCV) 110 fl 76–98 fl
Platelets 300 × 109/l 150–400 × 109/l
Troponin l 0.01 ng/ml < 0.1 ng/ml
D-dimer 0.03 μg/ml < 0.05 μg/ml
Arterial blood gas (ABG) showed type 1 respiratory failure with a normal pH. Chest X-ray showed left lower lobe consolidation.
The patient was treated successfully and is due for discharge tomorrow.
Upon speaking to the patient, he reveals that he has suffered two similar episodes this year.
Given the likely diagnosis, what medication should the patient be started on to reduce the risk of further episodes?Your Answer:
Correct Answer: Hydroxycarbamide (hydroxyurea)
Explanation:Treatment Options for a Patient with Sickle Cell Disease and Acute Chest Pain Crisis
A patient with sickle cell disease is experiencing an acute chest pain crisis, likely due to a lower respiratory tract infection. Hydroxycarbamide is recommended as a preventative therapy to reduce the risk of future crises by increasing the amount of fetal hemoglobin and reducing the percentage of red cells with hemoglobin S. Granulocyte colony-stimulating factor (G-CSF) is not necessary as the patient has a raised white blood cell count. Inhaled beclomethasone is not appropriate as asthma or COPD are not likely diagnoses in this case. Oral prednisolone may be used as a preventative therapy for severe asthma, but is not recommended for COPD and is not appropriate for this patient’s symptoms. A tuberculosis (TB) vaccination may be considered for primary prevention, but would not be useful for someone who has already been infected.
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This question is part of the following fields:
- Haematology
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Question 40
Incorrect
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These results were obtained on a 65-year-old male:
Hb 110 g/L (120-170)
RBC 4.8 ×1012/L (4.2-5.8)
Hct 0.365 (0.37-0.49)
MCV 75 fL (82-98)
MCH 33.2 pg (28-33)
Platelets 310 ×109/L (140-450)
WBC 8.21 ×109/L (4.5-11)
Neutrophils 6.45 ×109/L (1.8-7.5)
Lymphocytes 1.23 ×109/L (1.0-4.5)
Monocytes 0.28 ×109/L (0-0.8)
Eosinophils 0.18 ×109/L (0.02-0.5)
Basophils 0.09 ×109/L (0-0.1)
Others 0.18 ×109/L -
What could be the possible reason for these FBC results in a 65-year-old male?Your Answer:
Correct Answer: Gastrointestinal blood loss
Explanation:Microcytic Anaemia in a 63-Year-Old Female
A Full Blood Count (FBC) analysis has revealed that a 63-year-old female is suffering from microcytic anaemia, which is characterized by low mean corpuscular volume (MCV) and low haemoglobin (Hb) levels. This type of anaemia is typically caused by iron deficiency, which is often the result of blood loss. However, in this case, menorrhagia can be ruled out as the patient is postmenopausal. Therefore, the most likely cause of the microcytic anaemia is peptic ulceration. It is important to note that pernicious anaemia or folate deficiency can cause macrocytosis, which is characterized by elevated MCV levels. Proper diagnosis and treatment are necessary to address the underlying cause of the microcytic anaemia and prevent further complications.
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This question is part of the following fields:
- Haematology
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Question 41
Incorrect
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A young man comes to the clinic complaining of fatigue, tiredness, and pallor. Upon conducting a full blood count, it is revealed that he has acute lymphoblastic leukemia. Among the following options, which is linked to the poorest prognosis in ALL?
Your Answer:
Correct Answer: Philadelphia chromosome present
Explanation:Prognostic Factors in Acute Lymphoblastic Leukaemia
Acute lymphoblastic leukaemia (ALL) is a type of cancer that affects the blood and bone marrow. There are several factors that can affect the prognosis of a patient with ALL. Good prognostic factors include having the FAB L1 type, common ALL, a pre-B phenotype, and a low initial white blood cell count. On the other hand, poor prognostic factors include having the FAB L3 type, B or T cell type, the Philadelphia translocation (t(9;22)), increasing age at diagnosis, male sex, CNS involvement, and a high initial white blood cell count (e.g. > 100).
It is important for healthcare professionals to consider these prognostic factors when diagnosing and treating patients with ALL. By identifying these factors, they can better predict the outcome of the disease and tailor treatment plans accordingly. Patients with good prognostic factors may have a better chance of survival and may require less aggressive treatment, while those with poor prognostic factors may need more intensive therapy. Overall, the prognostic factors in ALL can help healthcare professionals provide the best possible care for their patients.
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This question is part of the following fields:
- Haematology
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Question 42
Incorrect
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What is true regarding the production of pooled plasma derivatives?
Your Answer:
Correct Answer: The end product is a freeze dried product
Explanation:Preparation of Plasma Derivatives
The preparation of plasma derivatives, such as factor VIII, involves pooling several thousand plasma donations, typically 20,000 or 5,000 kg of plasma at a time. To avoid the risk of vCJD, pooled plasma has been sourced from outside the UK since 1999. The process includes several chemical steps, including ethanol extraction, chromatography, and viral inactivation, resulting in a freeze-dried product. These products have a long shelf life of several months to years.
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This question is part of the following fields:
- Haematology
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Question 43
Incorrect
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A 55-year-old woman presents at the clinic for evaluation. She has not experienced menstrual periods for the past six months and has taken two pregnancy tests, both of which were negative. Upon clinical examination, no abnormalities were found. The patient desires a blood test to determine if she has entered menopause. What is the most sensitive hormone to test for this purpose?
Your Answer:
Correct Answer: FSH
Explanation:Hormone Levels and Menopausal Status
Follicle-stimulating hormone (FSH) levels that are greater than 30 IU/l, repeated over a period of four to eight weeks, are typically indicative of menopause. It is important to ensure that FSH is tested when the patient is not on contraception, although this is not relevant in the current scenario. While oestrogen and progesterone levels decrease after menopause, their assay is less reliable in determining menopausal status compared to FSH levels. Beta-HCG levels are elevated during pregnancy and trophoblastic disease, while prolactin levels increase in response to certain drug therapies and the presence of a pituitary tumour.
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This question is part of the following fields:
- Haematology
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Question 44
Incorrect
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A 14-year-old girl with known sickle-cell disease presents to the Emergency Department with severe abdominal pain.
On examination, she is found to have a tachycardia of 130 bpm, with generalised abdominal tenderness and 3 cm splenomegaly. Blood tests reveal marked anaemia, and a diagnosis of splenic sequestration crisis is considered.
Which blood vessel in the spleen is most responsible for monitoring the quality of red blood cells and removing aged ones from circulation?Your Answer:
Correct Answer: Splenic sinusoid
Explanation:The Anatomy of the Spleen: Splenic Sinusoids, Trabecular Veins, Arteries, and Sheathed Capillaries
The spleen is an important organ in the immune system, responsible for filtering blood and removing old or damaged red blood cells. Its unique anatomy allows it to perform this function effectively.
One key component of the spleen is the splenic sinusoid. These sinusoids are lined with elongated, cuboidal endothelial cells that are closely associated with macrophages. The gaps between the endothelial cells and incomplete basement membrane allow for the passage of red blood cells, with younger and more deformable cells passing through easily while older or abnormal cells are more readily destroyed by the macrophages.
The trabecular veins receive blood from the splenic sinusoids, while the trabecular arteries are branches of the afferent splenic artery. These arteries pass deep into the spleen along connective tissue trabeculae and branch into central arteries that pass through the white pulp of the spleen.
The central arteries then lead to sheathed capillaries, which are branches of the central arteries. These capillaries open directly into the red pulp of the spleen, allowing for further filtration and removal of old or damaged red blood cells.
Overall, the anatomy of the spleen is complex and specialized, allowing it to perform its important functions in the immune system.
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This question is part of the following fields:
- Haematology
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Question 45
Incorrect
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Which structure, containing both white and red pulp, is responsible for trapping foreign substances present in the blood?
Your Answer:
Correct Answer: Spleen
Explanation:Comparison of Lymphoid Organs and Non-Lymphoid Organs
The human body contains various organs that serve different functions. Among these are the lymphoid organs, which play a crucial role in the immune system. In this article, we will compare the characteristics of three lymphoid organs (spleen, lymph node, and thymus) with two non-lymphoid organs (heart and thyroid gland).
Spleen
The spleen is the largest secondary lymphoid organ. It is located in the left upper quadrant of the abdomen and contains both white pulp (lymphatic tissue and macrophages) and red pulp (sinusoids and red blood cells). One of its functions is to filter foreign substances from the blood.Lymph Node
Lymph nodes are secondary lymphoid organs that contain structures where mature lymphocytes are stimulated by antigens to undergo further division and differentiation. They do not contain white and red pulp.Thymus
The thymus is a primary lymphoid organ where T lymphocytes mature, differentiate, and proliferate. It does not contain white and red pulp.Heart
The heart is the main organ of the circulatory system and does not contain white and red pulp.Thyroid Gland
The thyroid gland is located in the anterior neck and is part of the endocrine system. It does not contain white and red pulp.In summary, lymphoid organs play a crucial role in the immune system, while non-lymphoid organs serve other functions. Understanding the characteristics of these organs can help us appreciate the complexity and diversity of the human body.
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This question is part of the following fields:
- Haematology
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Question 46
Incorrect
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A 25-year-old woman with sickle-cell disease is being evaluated in Haematology Outpatients. She has been admitted several times due to sickle-cell crisis and abdominal pain, and there is suspicion of multiple splenic infarcts. What blood film abnormalities would indicate hyposplenism?
Your Answer:
Correct Answer: Howell–Jolly bodies
Explanation:Blood Film Abnormalities and Their Significance
Blood film abnormalities can provide important diagnostic information about a patient’s health. One such abnormality is Howell-Jolly bodies, which are nuclear remnants found in red blood cells and indicate hyposplenism. Other abnormalities seen in hyposplenism include target cells, Pappenheimer cells, increased red cell anisocytosis and poikilocytosis, and spherocytes. Patients with hyposplenism are at increased risk of bacterial infections and should be vaccinated accordingly.
Rouleaux formation, on the other hand, is a stack of red blood cells that stick together, forming a rouleau. This occurs in conditions where plasma protein is high, such as multiple myeloma, some infections, Waldenström’s macroglobulinemia, and some cancers.
Schistocytes are irregular and jagged fragments of red blood cells that occur due to mechanical destruction of red blood cells in conditions such as hemolytic anemia. They are not typically seen in hyposplenism.
Tear drop cells, which are seen in conditions where there is abnormality of bone marrow function, such as myelofibrosis, are also not seen in hyposplenism.
Finally, toxic granulation occurs during inflammatory processes such as bacterial infection or sepsis and refers to neutrophils which contain dark, coarse granules. It is not present in hyposplenism.
In summary, understanding blood film abnormalities and their significance can aid in the diagnosis and management of various medical conditions.
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This question is part of the following fields:
- Haematology
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Question 47
Incorrect
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A 6-year-old child of African descent presents with a 2-day history of increasing abdominal pain. It is not associated with nausea or vomiting, and the bowels are opening regularly. The child’s mother tells you that he has had a few episodes of similar symptoms in the past, but none as severe.
On examination, the child has generalised abdominal tenderness, and splenomegaly is noted. After further investigation, the child is diagnosed with sickle-cell anaemia.
Which organ is most responsible for the removal of sickled red blood cells from the circulatory system?Your Answer:
Correct Answer: Spleen
Explanation:The Role of Organs in Immune Surveillance and Blood Production
The human body has several organs that play a crucial role in immune surveillance and blood production. The spleen, for instance, is responsible for removing abnormal and aged red blood cells from circulation and monitoring the blood for immune purposes. However, in sickle-cell anaemia patients, the spleen can become non-functional due to continuous hypoxic and thrombotic insults, leading to a process called autosplenectomy.
Lymph nodes, on the other hand, are involved in immunological surveillance of the lymph. They can swell in response to severe bacterial infections in specific body parts, such as the axillary lymph nodes in the case of a hand infection.
The thymus is responsible for programming pre-T cells to differentiate into T cells, which are responsible for the cellular immune response against pathogenic viruses and fungi and the destruction of malignant cells. It is most active during neonatal and pre-adolescent life.
The bone marrow is responsible for erythropoiesis, the production of red blood cells. In sickle-cell anaemia patients, erythropoiesis in the bone marrow is stimulated.
Finally, the liver can become a site of extramedullary erythropoiesis, which means it can produce red blood cells outside of the bone marrow.
Overall, these organs work together to maintain a healthy immune system and blood production in the body.
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This question is part of the following fields:
- Haematology
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Question 48
Incorrect
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A woman has some blood tests taken by her general practitioner (GP). The results show a Hb of 10.0, MCV of 69 and a ferritin of 9.
Which is the most appropriate management plan for this patient?Your Answer:
Correct Answer: Treat with ferrous sulphate 200 mg three times a day and repeat bloods in three months
Explanation:Understanding Iron Deficiency Anaemia and Treatment Options
Iron deficiency anaemia is a common condition that can present with symptoms such as lethargy, tiredness, and shortness of breath on exertion. It is often seen in women due to menstruation and blood loss associated with it, as well as in pregnant women. However, it is not a common finding in men and should be investigated further if present.
Treatment for iron deficiency anaemia involves the use of ferrous sulfate, typically at a dose of 200 mg two to three times a day for at least three months. Blood tests should be repeated after this time to assess the effectiveness of therapy. Folic acid supplementation may also be necessary in cases of folate deficiency anaemia, which presents with a raised MCV.
It is important to investigate persistent anaemia despite adequate iron supplementation, as it may indicate an underlying malignancy. Men with unexplained iron deficiency anaemia and a haemoglobin level of < 110 g/l should be referred urgently to the gastroenterology team for investigation of upper or lower gastrointestinal malignancy. Overall, understanding the causes and treatment options for iron deficiency anaemia can help improve patient outcomes and prevent complications.
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This question is part of the following fields:
- Haematology
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Question 49
Incorrect
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A 67-year-old man was admitted with pain in the left groin. He had fallen from his chair, after which the pain started. He was unable to walk and his left leg was externally rotated. X-rays revealed a neck of femur fracture. He lives alone in a flat and is a vegetarian. His past medical history includes shortness of breath, body itching and occasional swelling of legs. His blood tests revealed:
Investigation Result Normal range
Haemoglobin 120 g/l 135–175 g/l
Calcium 3.5 mmol/l 2.20–2.60 mmol/l
Phosphate 1.52 mmol/l 0.70–1.40 mmol/l
Erythrocyte sedimentation rate (ESR) 184 mm/h 0–15 mm/h
What is the most likely diagnosis?Your Answer:
Correct Answer: Multiple myeloma
Explanation:Diagnosis of Multiple Myeloma in a Patient with Pathological Fracture
A man has sustained a pathological fracture after a minor trauma, which is likely due to lytic bone lesions. He also presents with anemia, raised calcium, and ESR, all of which are consistent with a diagnosis of multiple myeloma. This is further supported by his age group for presentation.
Other possible diagnoses, such as osteoporosis, vitamin D deficiency, acute leukemia, and malignancy with metastasis, are less likely based on the absence of specific symptoms and laboratory findings. For example, in osteoporosis, vitamin D and phosphate levels are normal, and ESR and hemoglobin levels are not affected. In vitamin D deficiency, calcium and phosphate levels are usually normal or low-normal, and ESR is not raised. Acute leukemia typically presents with systemic symptoms and normal serum calcium levels. Malignancy with metastasis is possible but less likely without preceding symptoms suggestive of an underlying solid tumor malignancy.
In summary, the patient’s clinical presentation and laboratory findings suggest a diagnosis of multiple myeloma.
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This question is part of the following fields:
- Haematology
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Question 50
Incorrect
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A 28-year-old woman who is 30 weeks pregnant arrives at the emergency assessment unit with concerns of fluid leakage from her vagina. Upon examination, her pad is saturated with what appears to be amniotic fluid. What component of the fluid may indicate premature rupture of membranes (PROM)?
Your Answer:
Correct Answer: Alpha fetoprotein
Explanation:Using Biomarkers to Detect Premature Rupture of Membranes
Premature rupture of membranes (PROM) can be difficult to diagnose in some cases. In 2006, a study was conducted to determine if measuring certain biomarkers in vaginal fluid could be used as an indicator of membrane rupture. The study found that alpha-fetoprotein (AFP) had the highest accuracy in predicting PROM, with a specificity and sensitivity of 94%. This suggests that AFP could be used as a marker in cases where diagnosis is uncertain.
In addition to AFP, other biomarkers have been identified for different purposes. Carcinoembryonic antigen (CEA) is a tumor marker for colon cancer, while cancer antigen 125 (CA125) is a tumor marker for ovarian cancer. By measuring these biomarkers, doctors can detect the presence of cancer and monitor its progression. Overall, biomarkers have proven to be a valuable tool in diagnosing and monitoring various medical conditions.
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This question is part of the following fields:
- Haematology
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