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Question 1
Correct
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A 35-year-old woman is 10 weeks pregnant. She plans to undergo a Down's syndrome screening test around 15 weeks into her pregnancy.
What is included in the measurement of a Down's screening blood test?Your Answer: Alpha-fetoprotein
Explanation:AFP Measurement for Detecting Birth Defects and Chromosomal Abnormalities
When a woman is 15 weeks pregnant, a blood test called AFP measurement can be performed to determine if there is an increased risk of certain birth defects and chromosomal abnormalities. This test can detect open neural tube or abdominal wall defects, as well as Down’s syndrome and trisomy 18. In the past, if the results of the AFP measurement were abnormal, an ultrasound scan would be performed. However, it is possible that in the future, mid-trimester anomaly scanning may replace the use of AFP measurement altogether.
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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 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: Complete remission with incomplete recovery
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 3
Correct
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A 72-year-old man is receiving a 2 units of blood transfusion for anaemia of unknown cause – haemoglobin (Hb) 65 g/l (normal 135–175 g/l). During the third hour of the blood transfusion he spikes a temperature of 38.1°C (normal 36.1–37.2°C). Otherwise the patient is asymptomatic and his other observations are normal.
Given the likely diagnosis, what should you do?Your Answer: Temporarily stop transfusion, repeat clerical checks. Then treat with paracetamol and repeat observations more regularly (every 15 minutes)
Explanation:Treatment Options for Non-Haemolytic Febrile Transfusion Reaction
Non-haemolytic febrile transfusion reaction is a common acute reaction to plasma proteins during blood transfusions. If a patient experiences this reaction, the transfusion should be temporarily stopped, and clerical checks should be repeated. The patient should be treated with paracetamol, and observations should be repeated more regularly (every 15 minutes).
If the patient’s temperature is less than 38.5 degrees, and they are asymptomatic with normal observations, the transfusion can be continued with more frequent observations and paracetamol. However, if the patient experiences transfusion-associated circulatory overload, furosemide is a suitable treatment option.
Adrenaline is not needed unless there are signs of anaphylaxis, and antihistamines are only suitable for urticaria during blood transfusions. Therefore, it is essential to identify the specific type of transfusion reaction and provide appropriate treatment accordingly.
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This question is part of the following fields:
- Haematology
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Question 4
Correct
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Which statement about rhesus antibodies in pregnancy is correct?
Your 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 5
Correct
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A 35-year-old man comes to you seeking advice. He had a splenectomy ten years ago after a cycling accident and has been in good health since. However, a friend recently told him that he should be receiving treatment for his splenectomy. He is currently not taking any medication.
What would you recommend to him?Your Answer: Pneumococcal vaccination
Explanation:Asplenic Patients and the Importance of Vaccination
Asplenic patients are individuals who have had their spleen removed, leaving them at risk of overwhelming bacterial infections, particularly from pneumococcus and meningococcus. To prevent such infections, it is recommended that these patients receive the Pneumovax vaccine two weeks before surgery or immediately after emergency surgery. This vaccine should be repeated every five years. Additionally, influenzae vaccination is also recommended to prevent super added bacterial infections.
While oral penicillin is recommended for children, its long-term use in adults is a topic of debate. However, current guidance suggests that splenectomized patients should receive both antibiotic prophylaxis and appropriate immunization. It is crucial to take these preventative measures to protect asplenic patients from potentially life-threatening infections.
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This question is part of the following fields:
- Haematology
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Question 6
Incorrect
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This is the full blood count result of a 72-year-old male who presents with fatigue following his retirement 6 months ago:
Hb 130 g/L (120-160)
RBC 4.5 ×1012/L -
Haematocrit 0.39 (0.36-0.46)
MCV 86.5 fL (80-100)
MCH 28.1 pg (27-32)
Platelets 180 ×109/L (150-400)
WBC 6.5 ×109/L (4-11)
Neutrophils 3.8 ×109/L (2-7)
Lymphocytes 1.9 ×109/L (1-4)
Monocytes 0.5 ×109/L (0.2-1)
Eosinophils 0.2 ×109/L (0-0.5)
Basophils 0.1 ×109/L (0-0.1)
He is brought into the clinic by his wife who is concerned that her husband is constantly tired, has lost interest in his hobbies and has trouble sleeping.
Examination is pretty much normal except that he appears fatigued. There are no abnormalities on chest, abdominal or respiratory examination. Neurological examination is normal.
What is the most likely cause of this blood picture?Your Answer: Hypothyroidism
Correct Answer: Alcohol excess
Explanation:Delayed Grief Reaction and Elevated MCV in a Patient
Explanation:
The patient in question is displaying a delayed grief reaction following the recent death of her husband. Her FBC shows a normal picture except for an elevated MCV, which suggests alcohol excess. If the cause of macrocytosis were folate or B12 deficiency, it would be expected to cause anemia in association with the macrocytosis. Hypothyroidism may also cause macrocytosis, but the patient’s weight loss argues against this diagnosis. For further information on macrocytosis, refer to the BMJ Practice article Macrocytosis: pitfalls in testing and summary of guidance, the BMJ Endgames case report A woman with macrocytic anemia and confusion, and the BMJ Best Practice article Assessment of anemia. -
This question is part of the following fields:
- Haematology
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Question 7
Correct
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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: 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 8
Correct
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A 70-year-old woman comes to the clinic complaining of fatigue and overall weakness. She denies any other symptoms. Upon examination, the patient has conjunctival pallor and an inflamed, red tongue. Initial blood tests show a macrocytic anemia. Further testing reveals positive antiparietal cell antibodies.
What result is most likely to be found upon further investigation?Your Answer: Low vitamin B12
Explanation:Differential diagnosis of a patient with low vitamin B12 and related symptoms
Pernicious anaemia, coeliac disease, Crohn’s disease, and multiple myeloma are among the possible conditions that may cause low vitamin B12 levels and related symptoms. Pernicious anaemia is an autoimmune disorder that affects the gastric mucosa and impairs the production of intrinsic factor, leading to vitamin B12 deficiency and anaemia. Coeliac disease is a chronic immune-mediated enteropathy that affects the small intestine and causes malabsorption of nutrients, including vitamin B12. Crohn’s disease is a chronic inflammatory bowel disease that can affect any part of the gastrointestinal tract and cause various symptoms, including diarrhoea, abdominal pain, and weight loss. Multiple myeloma is a malignant plasma cell disorder that can cause bone pain, anaemia, and other symptoms, but is less likely to present with low vitamin B12 levels as the primary feature.
The differential diagnosis of these conditions may involve various tests and procedures, such as blood tests for antibodies and vitamin B12 levels, endoscopy with biopsies of the duodenum or colon, and bone marrow examination. The specific findings on these tests can help to distinguish between the different conditions and guide further management. For example, the presence of parietal cell antibodies and intrinsic factor antibodies in the blood may support a diagnosis of pernicious anaemia, while villous atrophy and crypt hyperplasia in the duodenal biopsies may suggest coeliac disease. Transmural inflammation with granuloma formation in the colon biopsies may indicate Crohn’s disease, while plasma cell infiltration in the bone marrow may suggest multiple myeloma.
Overall, the diagnosis of a patient with low vitamin B12 and related symptoms requires a thorough evaluation of the clinical history, physical examination, and laboratory findings, as well as consideration of the possible differential diagnoses.
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This question is part of the following fields:
- Haematology
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Question 9
Correct
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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: 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 10
Correct
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A 50-year-old female patient visits the clinic with complaints of abdominal discomfort and bloating that have been progressively worsening over the past few months. Upon examination, her GP discovered a complex left ovarian cyst. Which blood test would be the most useful in determining if the cyst is cancerous?
Your Answer: CA125
Explanation:Tumor Markers and Their Association with Different Cancers
Tumor markers are substances that are produced by cancer cells or by normal cells in response to cancer. These markers can be used to detect the presence of cancer, monitor the progress of treatment, and detect the recurrence of cancer. One such tumor marker is CA125, which is associated with ovarian carcinoma. However, elevations in CA125 can also be seen in uterine cancer, pancreas, stomach, and colonic tumors. In some cases, benign ovarian tumors can also cause a rise in CA125 levels. If a patient has a CA125 level of over 200U/ml and an abnormal ultrasound scan, it is highly likely that they have ovarian carcinoma.
Other tumor markers include CA19-9, which is primarily associated with pancreatic tumors, beta-HCG, which is associated with germ cell tumors, alpha-fetoprotein, which is associated with hepatocellular carcinoma, and carcinoembryonic antigen, which is associated with colonic carcinoma. While AFP and beta-HCG can rarely be secreted by ovarian tumors, it is important to check CA125 levels first, as they are much more frequently elevated. By monitoring tumor markers, doctors can detect cancer early and provide appropriate treatment.
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This question is part of the following fields:
- Haematology
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Question 11
Correct
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A 70-year-old man is referred to the medical team on call. He has been feeling fatigued for two months and is now experiencing shortness of breath with minimal exertion. He has also had several episodes of syncope with postural hypotension. The GP conducted a blood count and the results showed:
- Haemoglobin 64 g/L (120-160)
- MCV 62 fL (80-96)
- WCC 11.6 ×109L (4-11)
- Platelets 170 ×109L (150-400)
- MCH 22 pg (28-32)
What is the most appropriate next step?Your Answer: Transfuse packed red cells
Explanation:Microcytic Hypochromic Anaemia and the Importance of Blood Transfusion
This patient is presenting with a microcytic hypochromic anaemia, which is commonly caused by iron deficiency due to occult gastrointestinal (GI) blood loss in a Caucasian population. To determine the cause of the anaemia, a full history and examination should be conducted to look for clues of GI blood loss. Given the microcytic hypochromic picture, it is likely that blood loss has been ongoing for some time.
Although there is no evidence of haemodynamic compromise or congestive cardiac failure (CCF), the patient is experiencing breathlessness on minimal exertion. This justifies an upfront transfusion to prevent the patient from going into obvious cardiorespiratory failure. At a Hb of 64 g/L in a 72-year-old, the benefits of transfusion outweigh the risks.
While haematinics such as ferritin, vitamin B12, and folate are important investigations, the most crucial management step is organising a blood transfusion. This will help to address the immediate issue of anaemia and prevent further complications.
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This question is part of the following fields:
- Haematology
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Question 12
Correct
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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: 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 13
Incorrect
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A patient who is seen in the Renal Outpatient Department for glomerulonephritis presents to the Emergency Department with a swollen, erythematosus right leg with a 4-cm difference in circumference between the right and left leg. Routine blood tests show:
Investigation Result Normal value
Sodium (Na+) 143 mmol 135–145 mmol/l
Potassium (K+) 4.2 mmol 3.5–5.0 mmol/l
Urea 10.1 mmol 2.5–6.5 mmol/l
Creatinine 120 μmol 50–120 µmol/l
eGFR 60ml/min/1.73m2
Corrected calcium (Ca2+) 2.25 mmol 2.20–2.60 mmol/l
Bilirubin 7 μmol 2–17 µmol/l
Albumin 32 g/l 35–55 g/l
Alkaline phosphatase 32 IU/l 30–130 IU/l
Aspartate transaminase (AST) 15 IU/l 10–40 IU/l
Gamma-Glutamyl transferase (γGT) 32 IU/l 5–30 IU/l
C-reactive protein (CRP) 15 mg/l 0–10 mg/l
Haemoglobin 78 g/l
Males: 135–175 g/l
Females: 115–155 g/l
Mean corpuscular volume (MCV) 92 fl 76–98 fl
Platelets 302 x 109/l 150–400 × 109/l
White cell count (WCC) 8.5 x 109/l 4–11 × 109/l
Which of the following should be commenced after confirmation of the diagnosis?Your Answer: Low molecular weight heparin
Correct Answer: Apixaban
Explanation:According to NICE guidance, the first-line treatment for a confirmed proximal deep vein thrombosis is a direct oral anticoagulant such as apixaban or rivaroxaban. When warfarin is used, an initial pro-coagulant state occurs, so heparin is needed for cover until the INR reaches the target therapeutic range and until day 5. Low-molecular-weight heparin is typically used with warfarin in the initial anticoagulation phase, but it can accumulate in patients with renal dysfunction. Unfractionated heparin infusion is used in these cases. For patients with normal or slightly deranged renal function, low-molecular-weight heparin can be given once per day as a subcutaneous preparation. However, warfarin is not the first-line treatment according to NICE guidance.
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This question is part of the following fields:
- Haematology
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Question 14
Correct
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Which autoantibody is correctly matched with its associated disease in the following list?
Your 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 15
Correct
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A 29-year-old female patient presents to the clinic with a complaint of missed periods for the past four months despite negative pregnancy tests. She is also distressed about the loss of her libido and has noticed milk leakage with minimal nipple stimulation during intercourse. On physical examination, her blood pressure is 122/70 mmHg, pulse is 70 and regular, and general physical examination is unremarkable. Which blood test is most likely to show elevated levels?
Your Answer: Prolactin
Explanation:Symptoms and Diagnosis of Hyperprolactinaemia
Hyperprolactinaemia is a condition characterized by elevated levels of prolactin in the body. This condition is typically associated with symptoms such as milk production, decreased libido, and absence of menstruation. However, visual disturbances are not always present, as many cases of hyperprolactinaemia are related to a microprolactinoma.
When diagnosing hyperprolactinaemia, it is important to assess thyroid status as this condition is often associated with hypothyroidism. Thyroxine levels are usually low in individuals with hyperprolactinaemia. Additionally, beta-HCG levels are elevated in pregnancy, so it is important to rule out pregnancy as a potential cause of elevated prolactin levels.
In summary, hyperprolactinaemia is a condition that can present with a variety of symptoms, but is typically characterized by elevated prolactin levels. Diagnosis involves assessing thyroid status and ruling out pregnancy as a potential cause.
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This question is part of the following fields:
- Haematology
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Question 16
Correct
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A 65-year-old man comes to the clinic with complaints of haematuria. He has a history of chronic atrial fibrillation and is currently taking warfarin. His blood tests reveal a Hb level of 112g/L and an INR of 9, but he is stable hemodynamically. The consulting physician recommends reversing the effects of warfarin. What blood product/s would be the most appropriate choice for this patient?
Your Answer: Prothrombin concentrate ('Octaplex')
Explanation:Treatment Options for Warfarin Reversal
Prothrombin concentrates are the preferred treatment for reversing the effects of warfarin in cases of active bleeding and a significantly elevated INR. While packed cells are important for managing severe bleeding, they are not the recommended treatment for warfarin reversal. Cryoprecipitate, recombinant factor VII, and platelets are also not indicated for reversing the effects of warfarin. It is important to choose the appropriate treatment option based on the patient’s individual needs and medical history. Proper management of warfarin reversal can help 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 17
Correct
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The most common error in transfusion according to the SHOT (serious hazards of transfusion) analysis?
Your 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 18
Correct
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A plasma donor on a continuous flow separator machine experiences light headedness, muscle cramps, and circumoral paraesthesia. What should be the next course of action in managing this patient?
Your Answer: Investigate and treat citrate toxicity
Explanation:Citrate Toxicity and Hypocalcaemia in Apheresis Patients
This patient is experiencing symptoms of citrate toxicity, which has led to hypocalcaemia. While it is possible for haemorrhage to occur at the site of venepuncture or venous access, this is typically easy to identify through clinical examination. Sepsis is an uncommon occurrence if proper aseptic precautions have been taken, and the symptoms described here are not indicative of an infection. Immediate treatment is necessary, and this can be achieved by slowing or stopping the apheresis process. Treatment options include the administration of oral or intravenous calcium replacement.
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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 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: Platelet transfusions
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 20
Correct
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A 19-year-old Afro-Caribbean male with sickle cell disease complains of right upper abdominal pain and exhibits tenderness upon palpation. What diagnostic test should be performed to confirm the diagnosis?
Your Answer: Abdominal ultrasound scan
Explanation:Pigment Gallstones and High Haem Turnover
In cases of chronic haemolysis, such as sickle cell disease or thalassaemia, the presence of unconjugated bilirubin in bile can lead to the formation of pigment gallstones. These stones are black in color and are caused by the precipitation of calcium bilirubinate from solution. The high concentration of unconjugated bilirubin in bile is a result of the increased turnover of haemoglobin. This can cause pain and discomfort for the patient. It is important to manage the underlying condition causing the high haem turnover to prevent the formation of pigment gallstones.
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This question is part of the following fields:
- Haematology
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Question 21
Correct
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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: 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 22
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 23
Correct
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A 36-year-old woman, who has received a 3-unit blood transfusion following a hysterectomy, begins to feel unwell with a fever and chills 3 or 4 days later. There are no obvious signs of active bleeding. On examination, you note that she is mildly jaundiced.
Investigations:
Investigation Result Normal value
Pre-transfusion haemoglobin (Hb) 92 g/l 115–155 g/l
Post-transfusion Hb 98 g/l 115–155 g/l
Platelets 180 × 109/l 150–400 × 109/l
White cell count (WCC) 7.2 × 109/l 4–11 × 109/l
Sodium (Na+) 138 mmol/l 135–145 mmol/l
Potassium (K+) 4.9 mmol/l 3.5–5.0 mmol/l
Creatinine 130 μmol/l 50–120 µmol/l
Bilirubin 90 μmol/l 2–17 µmol/l
Alanine aminotransferase (ALT) 50 IU/l 5–30 IU/l
What is the most likely diagnosis for this 36-year-old woman who has received a 3-unit blood transfusion following a hysterectomy and is experiencing fever, chills, and mild jaundice without signs of active bleeding?Your Answer: Delayed transfusion haemolysis
Explanation:Delayed Transfusion Haemolysis: A Possible Cause for Minimal Increase in Haemoglobin
A patient who has received a 3-unit transfusion has only shown a minimal increase in haemoglobin levels, which is a cause for concern. The rise in bilirubin without a corresponding increase in liver enzymes suggests haemolysis from a delayed transfusion haemolysis reaction. This type of reaction occurs when a patient without certain red cell antigens is exposed to these antigens through blood transfusion, resulting in the development of new antibodies and haemolysis after 3-14 days. Symptoms include fevers, rigors, rash, and jaundice, which are less severe and more gradual in onset than acute haemolytic reactions.
Medical management of this patient will involve screening for a wider range of possible antigens and access to a blood bank with a sufficient number of available units for a clean transfusion. Serious complications of blood transfusion are rare due to screening techniques, leukocyte depletion, and improved collection and storage.
Other potential transfusion-related reactions and their approximate time-course include hyperacute (minutes to hours), acute (hours to days), and late (days or longer). Bacterial sepsis is a possible reaction that would occur most likely in the acute time course, but it does not explain the minimal increase in haemoglobin following a 3-unit transfusion. Further haemorrhage, cholestasis of pregnancy, and disseminated intravascular coagulation (DIC) are also unlikely causes.
In conclusion, delayed transfusion haemolysis is a possible cause for the minimal increase in haemoglobin levels in this patient. It is important to consider this reaction and manage it appropriately to prevent further complications.
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This question is part of the following fields:
- Haematology
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Question 24
Correct
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A 25-year-old female presents with severe anaemia and a skull x-ray shows a 'hair on end' appearance. Where is this appearance most commonly observed on a skull x-ray?
Your Answer: Thalassaemia
Explanation:Skeletal Abnormalities in Thalassaemia Major
Patients with thalassaemia major often exhibit skeletal abnormalities, which can be observed in various parts of the body. One of the most notable changes is an expanded bone marrow space, which leads to the thinning of the bone cortex. This can be particularly striking in the skull, where it can cause a hair on end appearance. While this phenomenon can also occur in sickle cell disease, it is much less common.
In addition to changes in the skull, bone abnormalities can also be seen in the long bones, vertebrae, and pelvis. These changes can have a significant impact on a patient’s quality of life, as they can cause pain, deformities, and other complications. As such, it is important for healthcare providers to be aware of these skeletal abnormalities and to monitor patients for any signs of progression or deterioration. With proper management and treatment, many of these complications can be mitigated or prevented, allowing patients with thalassaemia major to lead healthy and fulfilling lives.
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This question is part of the following fields:
- Haematology
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Question 25
Correct
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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: 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 26
Correct
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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: 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 27
Correct
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A 70-year-old man presents with increasing shortness of breath on exertion. He also notes he has been bruising more easily of late. He is noted to be in sinus tachycardia but otherwise is haemodynamically stable. Examination reveals conjunctival pallor and hepatosplenomegaly. No definite lymphadenopathy is palpable. A full blood count is performed:
Investigation Result Normal value
Haemoglobin 69 g/l 135–175 g/l
White cell count (WCC) 0.7 × 109/l 4.0–11.0 × 109/l
Platelets 14 × 109/l 150–400 × 109/l
Blood film is reported as a leukoerythroblastic picture with teardrop-shaped erythrocytes. A bone marrow aspirate is attempted, but this is unsuccessful.
What is the likely diagnosis?Your Answer: Myelofibrosis
Explanation:Understanding Myelofibrosis: A Comparison with Other Bone Marrow Disorders
Myelofibrosis is a rare disorder that primarily affects older patients. It is characterized by bone marrow failure, which can also be found in other diseases such as advanced prostate cancer, acute lymphoblastic leukemia, acute myelocytic leukemia, and chronic myeloid leukemia. However, myelofibrosis can be distinguished from these other disorders by specific diagnostic clues.
One of the key diagnostic features of myelofibrosis is the presence of a leukoerythroblastic picture with teardrop-shaped red blood cells, which is also seen in advanced prostate cancer. However, in myelofibrosis, a failed bone marrow aspirate, or dry tap, is frequent and a bone marrow trephine biopsy is needed for diagnosis. This is not the case in other bone marrow disorders.
Myelofibrosis is caused by the proliferation of megakaryocytes, which leads to intense bone marrow fibrosis, marrow failure, and secondary hepatosplenomegaly due to extramedullary hematopoiesis. Patients may present with systemic upset, symptoms of marrow failure, or abdominal discomfort from hepatosplenomegaly. Treatment is supportive, with bone marrow transplant reserved for younger patients. The median survival is 4-5 years, and transformation to acute myeloid leukemia is relatively common.
In contrast, acute lymphoblastic leukemia is a disease of childhood that presents with elevated white cell count and blasts on peripheral blood film. Acute myelocytic leukemia and chronic myeloid leukemia both present with raised white cell counts and blasts on blood film, but are more common in younger patients. Advanced prostate cancer may cause bone marrow failure if there is replacement of enough bone marrow by metastases, but patients would also complain of bone pain.
In summary, while bone marrow failure may be found in various diseases, specific diagnostic clues such as a leukoerythroblastic picture with teardrop-shaped red blood cells and a failed bone marrow aspirate can help distinguish myelofibrosis from other bone marrow disorders.
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This question is part of the following fields:
- Haematology
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Question 28
Correct
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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: 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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Question 29
Correct
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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: 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 30
Correct
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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: 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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