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Question 1
Correct
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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: 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 2
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: His clotting time will be prolonged
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 3
Incorrect
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A 58-year-old male presents with macrocytic anaemia and a megaloblastic bone marrow. What is the most probable cause of his macrocytosis?
Your Answer: Alcohol
Correct Answer: Folate deficiency
Explanation:Megaloblastic Bone Marrow and Its Causes
A megaloblastic bone marrow is a condition that occurs due to a deficiency in vitamin B12 or folate, as well as some cytotoxic drugs. This condition is characterized by the presence of large, immature red blood cells in the bone marrow. However, other causes of macrocytosis, which is the presence of abnormally large red blood cells in the bloodstream, do not result in a megaloblastic bone marrow appearance. It is important to identify the underlying cause of macrocytosis to determine the appropriate treatment.
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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 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: Upper GI endoscopy
Correct 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 5
Correct
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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: 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 6
Correct
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A 68-year-old man who has been diagnosed with severe Gram-positive sepsis is admitted to the Intensive Care Unit (ICU). However, he is starting to deteriorate despite being on the Sepsis Six Bundle. He is pyrexial and appears very unwell. On examination:
Investigation Results Normal value
Respiratory rate (RR) 30 breaths/minute 12–18 breaths/minute
Heart rate (HR) 120 bpm 60–100 bpm
Blood pressure (BP) 88/40 mmHg < 120/80 mmHg
You noted some bleeding along the cannulation site and on his gums. The coagulation profile showed prolonged prothrombin time, a decrease in fibrinogen level and marked elevation of D-dimer. He has some purpuric rash on his extremities.
Which of the following is the most likely cause of the above condition?Your Answer: Disseminated intravascular coagulopathy (DIC)
Explanation:Comparison of DIC, von Willebrand’s Disease, Liver Failure, Haemophilia, and Heparin Administration
Disseminated intravascular coagulopathy (DIC) is a serious complication of severe sepsis that can lead to multiorgan failure and widespread bleeding. It is characterized by high prothrombin time and the use of fibrinogen for widespread clot formation, resulting in high levels of D-dimer due to intense fibrinolytic activity. DIC is a paradoxical state in which the patient is prone to clotting but also to bleeding.
Von Willebrand’s disease is an inherited disorder of coagulation that is usually autosomal dominant. There is insufficient information to suggest that the patient in this case has von Willebrand’s disease.
Liver failure could result in excessive bleeding due to disruption of liver synthetic function, but there is no other information to support liver failure in this case. Signs of hepatic encephalopathy or jaundice would also be expected.
Haemophilia is an X-linked recessive disorder of coagulation that is characterized by prolonged activated partial thromboplastin time (APTT) and normal prothrombin time.
There is no information to suggest that heparin has been administered, and the bleeding time and platelet count would be normal.
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This question is part of the following fields:
- Haematology
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Question 7
Correct
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A 91-year-old woman with known vascular dementia presents to the Emergency Department after a routine blood test uncovered that the patient had a sodium level of 149 mmol/l. Her carer comments that the patient’s fluid intake has been minimal over the past week. The patient’s past medical history includes a previous lacunar stroke and peripheral vascular disease. Her regular medications are atorvastatin, ramipril, amlodipine and clopidogrel. She currently is fully dependent on the assistance provided by carers. The patient’s National Early Warning (NEWS) score is 0, and her Glasgow Coma Scale (GCS) score is 15.
A physical examination does not reveal any source of infection. A bedside capillary glucose is recorded as 5.8 mmol/mmol. A full set of blood tests are repeated, reported as follows:
Full blood count and urea and electrolytes (U&Es):
Investigations Results Normal Values
Haemoglobin (Hb) 131 g/l 115–155 g/l
Mean corpuscular volume (MCV) 91 fl 76–98 fl
White cell count (WCC) 5 × 109/l 4–11 × 109/l
Platelets (Plt) 215 × 109/l 150–400 × 109/l
Sodium (Na+) 148 mmol/l 135–145 mmol/l
Potassium (K+) 4.8 mmol/l 3.5–5.0 mmol/l
Urea 3.3 mmol/l 2.5–6.5 mmol/l
Creatinine 66 mmol/l 50–120 mmol/l
A routine chest X-ray and urinalysis are performed and show no abnormalities.
Which of the following is the most suitable to correct the patient’s hypernatraemia?Your Answer: Oral water
Explanation:Treatment Options for Hypernatraemia: A Case Study
Hypernatraemia is a condition characterized by an elevated sodium concentration in the blood. In this case study, the patient’s hypernatraemia is mild and caused by insufficient free water intake. It is important to rule out infection as a cause of hypernatraemia, which can increase free water loss. Mild calcification of the aortic arch is a common finding in the elderly and unrelated to the patient’s current complaint.
Hypertonic saline infusion is not recommended as it would further increase the sodium concentration. Standard dialysis is not necessary in this case as the hypernatraemia is not profound enough. 500 ml of 10% dextrose is not appropriate as it is not equivalent to giving free water and is used to reverse hypoglycaemia. 500 ml of 0.9% saline is not the correct option for this patient, but it may be appropriate for hypovolaemic and hypotensive patients to restore circulating volume.
The most appropriate treatment option for this patient is to provide free water, which can be achieved by administering 5% dextrose. It is important to monitor the patient’s sodium levels and fluid intake to prevent further complications. The decision to start hypertonic saline infusion or dialysis should be made by a consultant in severe cases of hypernatraemia.
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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 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: Hypothyroidism
Correct 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 9
Incorrect
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A 50-year-old woman presents with headaches and nosebleeds and is found to have a raised platelet count. She is diagnosed with essential thrombocytosis by the haematologist.
Which of the following might be used to treat essential thrombocytosis?Your Answer: Interferon gamma
Correct Answer: Hydroxyurea
Explanation:Common Medications and Their Uses
Thrombocytosis and Hydroxyurea
Thrombocytosis is a condition characterized by an elevated platelet count, which can lead to bleeding or thrombosis. Primary or essential thrombocytosis is a myeloproliferative disorder that results in overproduction of platelets by the bone marrow. Hydroxyurea is the first-line treatment for essential thrombocytosis, as it inhibits an enzyme involved in DNA synthesis and reduces the rate of platelet production.Interferon Gamma for Immunomodulation
Interferon gamma is an immunomodulatory medication used to reduce the frequency of infections in patients with chronic granulomatous disease and severe malignant osteopetrosis. It is administered by subcutaneous injection.Cromoglycate for Inflammation
Sodium cromoglycate is a synthetic non-steroidal anti-inflammatory drug used in the treatment of asthma, allergic rhinitis, and various food allergies.Interferon β for Multiple Sclerosis
Interferon β is a cytokine used in the treatment of relapsing-remitting multiple sclerosis. It is administered subcutaneously.Ranitidine for Acid Reduction
Ranitidine is a H2 (histamine) receptor blocker that inhibits the production of acid in the stomach. It can be used in the treatment of gastro-oesophageal reflux disease, peptic ulcer disease, and gastritis. -
This question is part of the following fields:
- Haematology
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Question 10
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 11
Incorrect
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A diabetic patient with idiopathic thrombocytopenic purpura presents with a leg ulcer which needs debridement. His platelet counts are 15 ×109/l. His blood sugars are poorly controlled and he has been started on a sliding scale insulin. He has previously responded to steroids and immunoglobulin infusions.
What is the recommended product to increase platelet counts to a safe level for debridement surgery in a diabetic patient with idiopathic thrombocytopenic purpura who has previously responded to steroids and immunoglobulin infusions and has poorly controlled blood sugars, and is slightly older?Your Answer: Single donor platelets
Correct Answer: Intravenous immunoglobulin
Explanation:Treatment options for ITP patients
Intravenous immunoglobulin is the preferred treatment for patients with immune thrombocytopenia (ITP) who also have diabetes. Steroids may be used as a trial treatment if the patient does not have any contraindications for steroid-related complications. Platelets are not typically effective in raising platelet counts in ITP patients because they are destroyed by the antibodies. However, they may be used in emergency situations to treat major bleeding. It is important for healthcare providers to carefully consider the individual patient’s medical history and current condition when selecting a treatment plan for ITP. Proper treatment can help manage symptoms and improve quality of life for patients with this condition.
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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 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: 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 13
Correct
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A 14-year-old girl presents to the clinic with her parents. She is worried about not having started her periods yet, while many of her peers have. Her medical history includes a lack of sense of smell, which she has had since childhood. On examination, she has normal height, early breast development, and minimal secondary sexual hair. Her BMI is 22. What blood test would be most helpful in determining the underlying cause of her amenorrhea?
Your Answer: FSH
Explanation:Kallmann’s Syndrome and its Differential Diagnosis
Anosmia and primary amenorrhoea are two symptoms that may indicate the presence of Kallmann’s syndrome. This condition is characterized by the underdevelopment of the olfactory bulb, which leads to a loss of the sense of smell, and the failure to produce gonadotrophin releasing hormone. As a result, low levels of follicle-stimulating hormone and luteinising hormone may cause a partial or complete failure to enter puberty in women.
Congenital adrenal hyperplasia, on the other hand, may cause electrolyte imbalances, but it is typically associated with abnormal female virilization. Prolactinoma, a type of pituitary tumor, is usually linked to secondary amenorrhoea. Meanwhile, thyrotoxicosis, a condition characterized by an overactive thyroid gland, may cause menstrual cessation, but it is less likely to be the cause of primary amenorrhoea, especially in the absence of hyperthyroidism symptoms.
In summary, Kallmann’s syndrome should be considered as a possible diagnosis in patients presenting with anosmia and primary amenorrhoea. However, other conditions such as congenital adrenal hyperplasia, prolactinoma, and thyrotoxicosis should also be ruled out through proper evaluation and testing.
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This question is part of the following fields:
- Haematology
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Question 14
Incorrect
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By how much does the haemoglobin concentration increase with the infusion of one unit of fresh blood?
Your Answer: 25 g/L
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 15
Correct
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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: 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 16
Correct
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What is the diagnostic tool for beta thalassaemia?
Your Answer: Haemoglobin electrophoresis
Explanation:Diagnosis of Beta Thalassaemia
Beta thalassaemia can be diagnosed through the presence of mild microcytic anaemia, target cells on the peripheral blood smear, and a normal red blood cell count. However, the diagnosis is confirmed through the elevation of Hb A2, which is demonstrated by electrophoresis. In beta thalassaemia patients, the Hb A2 level is typically around 4-6%.
It is important to note that in rare cases where there is severe iron deficiency, the increased Hb A2 level may not be observed. However, it becomes evident with iron repletion. Additionally, patients with the rare delta-beta thalassaemia trait do not exhibit an increased Hb A2 level.
In summary, the diagnosis of beta thalassaemia can be suggested through certain symptoms and blood tests, but it is confirmed through the measurement of Hb A2 levels.
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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 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: bcr-abl
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 18
Incorrect
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A 70-year-old man presents to the clinic with a four-month history of abdominal swelling and discomfort along with breathlessness. Upon examination, he appears unwell and pale. The liver is palpable 12 cm below the right costal margin, and the spleen is palpable 15 cm below the left costal margin. No lymphadenopathy is detected. The following investigations were conducted:
Hb 59 g/L (130-180)
RBC 2.1 ×1012/L -
PCV 0.17 l/l -
MCH 30 pg (28-32)
MCV 82 fL (80-96)
Reticulocytes 1.4% (0.5-2.4)
Total WBC 23 ×109/L (4-11)
Normoblasts 8% -
Platelets 280 ×109/L (150-400)
Neutrophils 9.0 ×109/L (1.5-7)
Lymphocytes 5.2 ×109/L (1.5-4)
Monocytes 1.3 ×109/L (0-0.8)
Eosinophils 0.2 ×109/L (0.04-0.4)
Basophils 0.2 ×109/L (0-0.1)
Metamyelocytes 5.1 ×109/L -
Myelocytes 1.6 ×109/L -
Blast cells 0.4 ×109/L -
The blood film shows anisocytosis, poikilocytosis, and occasional erythrocyte tear drop cells. What is the correct term for this blood picture?Your Answer: Anaemia of chronic disease
Correct Answer: Leukoerythroblastic anaemia
Explanation:Leukoerythroblastic Reactions and Myelofibrosis
Leukoerythroblastic reactions refer to a condition where the peripheral blood contains immature white cells and nucleated red cells, regardless of the total white cell count. This means that even if the overall white cell count is normal, the presence of immature white cells and nucleated red cells can indicate a leukoerythroblastic reaction. Additionally, circulating blasts may also be seen in this condition.
On the other hand, myelofibrosis is characterized by the presence of tear drop cells. These cells are not typically seen in other conditions and are therefore considered a hallmark of myelofibrosis. Tear drop cells are red blood cells that have been distorted due to the presence of fibrous tissue in the bone marrow. This condition can lead to anemia, fatigue, and other symptoms.
Overall, both leukoerythroblastic reactions and myelofibrosis are conditions that can be identified through specific characteristics in the peripheral blood. It is important for healthcare professionals to be aware of these findings in order to properly diagnose and treat patients.
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This question is part of the following fields:
- Haematology
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Question 19
Correct
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A 36-year-old Afro-Caribbean woman presents to the Emergency Department complaining of shortness of breath, fever and a productive cough. She has suffered multiple severe infections over the past five years; however, she has no other past medical history.
On examination, you note intercostal recessions and the use of accessory muscles of respiration. She has significant coarse crepitations in her right lower lobe.
You take some basic observations, which are as follows:
Temperature: 39.8 °C
Heart rate: 120 bpm
Respiratory rate: 26 breaths/min
Blood pressure: 150/94 mmHg (lying) 146/90 mmHg (standing)
Oxygen saturation: 86% on room air
Her initial investigation findings are as follows:
Investigation Result Normal value
White cell count (WCC) 14.4 × 109/l 4–11. × 109/l
Neutrophils 12800 × 106/l 3000–5800 × 106/l
Lymphocytes 1400 × 106/l 1500–3000 × 106/l
Haemoglobin (Hb) 110 g/dl 115–155 g/dl
Mean corpuscular volume (MCV) 94 fl 76–98 fl
Platelets 360 × 109/l 150–400 × 109/l
Her chest X-ray shows significant consolidation in the right lower lobe.
A blood film comes back and shows the following: sickled erythrocytes and Howell–Jolly bodies.
A sputum culture is grown and shows Streptococcus pneumoniae, and the patient’s pneumonia is managed successfully with antibiotics and IV fluid therapy.
What condition is predisposing this patient to severe infections?Your Answer: Splenic dysfunction
Explanation:The patient has sickle cell disease and a history of recurrent infections, indicating long-term damage to the spleen. The blood film shows signs of splenic disruption, such as Howell-Jolly bodies, and a low lymphocyte level, which may be due to reduced lymphocyte storage capacity in the shrunken spleen. This is different from a splenic sequestration crisis, which is an acute pediatric emergency. The current admission may be an acute chest pain crisis, but it is not the cause of the recurrent infections. The patient does not have acute lymphoblastic leukemia, as there is no evidence of blastic cells or pancytopenia. Advanced HIV is a possibility, but the blood film suggests sickle cell disease. While the patient is at risk of an aplastic crisis, it typically occurs in younger patients after a parvovirus B19 infection, which is not present in this case.
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This question is part of the following fields:
- Haematology
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Question 20
Incorrect
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An 80-year-old man comes to the clinic with painless, symmetrical swellings in his neck. He reports no other symptoms. Laboratory tests show a haemoglobin level of 100 g/l and a white cell count of 23 × 109/l. A blood film reveals smear cells, with more than 60% of the cells being small mature lymphocytes. What is the probable diagnosis?
Your Answer: Chronic myeloid leukaemia
Correct Answer: Chronic lymphocytic leukaemia
Explanation:Differentiating Leukaemia and Lymphoma: Understanding CLL and Other Types
Leukaemia and lymphoma are two types of blood cancers that can present with similar symptoms. However, each type has distinct characteristics that can help differentiate them. Among the different types of leukaemia and lymphoma, B-cell chronic lymphocytic leukaemia (B-CLL) is the most common leukaemia in adults. It is characterized by peripheral blood lymphocytosis and uncontrolled proliferation of B cell lymphocytes in the bone marrow, lymph nodes, and splenomegaly. Patients with CLL are often asymptomatic, and the condition is often picked up incidentally.
In contrast, acute lymphoblastic leukaemia is a common leukaemia of children aged 2–5 years and is very rare in adults. Multiple myeloma, on the other hand, is the uncontrolled proliferation of plasma cells and presents with bone pain, hypercalcaemia, renal failure, and neutropenia. Chronic myeloid leukaemia tends to present with more systemic, B symptoms in a slightly younger age group, and a classic symptom is massive hepatosplenomegaly.
While lymphoma is a possibility in this age group, CLL is the most likely diagnosis as it is more common in this age group and in the western world. Further investigation would be used to confirm the diagnosis. Understanding the characteristics of each type of leukaemia and lymphoma can aid in accurate diagnosis and appropriate treatment.
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This question is part of the following fields:
- Haematology
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Question 21
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 22
Incorrect
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A 35-year-old man presents with progressive weakness in his right upper limb and both lower limbs over the past four months. He has also developed digital infarcts affecting the second and third fingers on the right hand and the fifth finger on the left. On examination, his blood pressure is 160/140 mm Hg, all peripheral pulses are palpable, and there is an asymmetrical neuropathy. Laboratory investigations reveal a haemoglobin level of 120 g/L (130-170), a white cell count of 12.5 ×109/L (4-10), a platelet count of 430 ×109/L (150-450), and an ESR of 50 mm/hr (0-15). Urine examination shows proteinuria and 10-15 red blood cells per high power field without casts. What is the most likely diagnosis?
Your Answer: Granulomatosis with polyangiitis
Correct Answer: Polyarteritis nodosa
Explanation:Polyarteritis nodosa (PAN) is a systemic disease that affects small or medium-sized arteries in various organs, leading to a wide range of symptoms such as nerve damage, skin issues, joint and muscle pain, kidney problems, and heart issues. Laboratory findings include anemia, increased white blood cells and platelets, and elevated inflammatory markers. ANCA testing can help differentiate PAN from other vasculitis diseases.
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This question is part of the following fields:
- Haematology
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Question 23
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A patient on a medical ward received a transfusion 72 hours ago for symptomatic anaemia on a background of chronic renal disease and obstructive airways disease. He has a history of previous transfusions in the last six months. The patient, who is in his 60s, has now dropped his Hb by 20 g/L compared to his pretransfusion level and reports a dark coloured urine. The LDH and bilirubin are elevated. What is the most likely explanation for these findings?
Your Answer: Delayed haemolytic transfusion reaction
Explanation:Delayed Haemolytic Transfusion Reaction
A delayed haemolytic transfusion reaction can occur 24 hours after a transfusion in patients who have been previously immunised through transfusions or pregnancy. Initially, the antibodies are not detectable, but they become apparent as a secondary immune response to the antigen exposure during the transfusion. In such cases, it is essential to carry out a haemoglobin level, blood film, LDH, direct antiglobulin test, renal profile, serum bilirubin, haptoglobin, and urinalysis for haemoglobinuria. Additionally, the group and antibody screen should be repeated.
It is unlikely that the patient is experiencing a transfusion-associated graft versus host disease or acute hepatitis as both would occur within a week or two. Furthermore, this is not an acute haemolysis that would be expected to occur during the transfusion. The rise in bilirubin and LDH levels indicates a haemolytic reaction. Therefore, it is crucial to monitor the patient’s condition and provide appropriate treatment.
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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 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: 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 25
Incorrect
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A middle-aged woman presents with increasing fatigue and daytime exhaustion that is starting to affect her work as a receptionist. She has been referred to the gynaecology clinic for evaluation of menorrhagia. What results would you anticipate on her complete blood count (CBC)?
Your Answer: Haemoglobin - low, MCV - normal
Correct Answer: Haemoglobin - low, MCV - reduced
Explanation:Interpreting Blood Results for Anaemia: Understanding the Relationship between Haemoglobin and MCV
When interpreting blood results for anaemia, it is important to understand the relationship between haemoglobin and mean corpuscular volume (MCV). A low haemoglobin and reduced MCV may indicate iron deficiency anaemia secondary to menorrhagia, which is a common cause of microcytosis. Treatment for this would involve managing the underlying menorrhagia and supplementing with iron. On the other hand, a low haemoglobin and raised MCV may indicate macrocytic anaemia, commonly associated with vitamin B12 or folate deficiency. It is important to note that a normal haemoglobin with a reduced MCV or a normal haemoglobin and MCV is unlikely in cases of significant symptoms and abnormal bleeding. Understanding these relationships can aid in the diagnosis and management of anaemia.
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This question is part of the following fields:
- Haematology
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Question 26
Incorrect
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Which statement about rhesus antibodies in pregnancy is correct?
Your Answer: D positive women are less likely than D negative women to form antibodies to other red cell antigens (such as Kell and Duffy)
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 27
Incorrect
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The risk of contracting a viral infection through a blood transfusion can vary greatly. What is the estimated risk of hepatitis B transmission in the United Kingdom, for instance?
Your Answer: 1 per 10 million donations
Correct Answer: 1 per 1 million donations
Explanation:Infective Risks of Blood Transfusion
Blood transfusions carry the risk of transmitting viral infections such as hepatitis B, hepatitis C, and HIV. The likelihood of infection varies depending on the source of the donation and the type of testing used. In the UK, the risk of contracting hepatitis B from a blood transfusion is approximately 1 in 1.3 million donations. The risks for HIV and hepatitis C are even lower, at 1 in 6.5 million and 1 in 28 million donations, respectively. It is important for healthcare professionals to have a comprehensive of these risks when obtaining consent from patients for blood transfusions. Adequate knowledge and communication can help patients make informed decisions about their healthcare.
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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 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: 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 29
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A 32-year-old woman comes to Haematology complaining of fatigue, anaemia and splenomegaly. She is diagnosed with a genetic disorder that causes abnormal blood cell shape due to a dysfunctional membrane protein. As a result, these cells are broken down by the spleen, leading to haemolytic anaemia and splenomegaly. What is the most probable abnormality observed in a blood film of individuals with this condition?
Your Answer: Sphere-shaped red blood cells
Explanation:Understanding Abnormalities in Red Blood Cells: Hereditary Spherocytosis and Other Conditions
Hereditary spherocytosis is an inherited condition that causes red blood cells to take on a sphere shape instead of their normal biconcave disc shape. This abnormality leads to increased rupture of red blood cells in capillaries and increased degradation by the spleen, resulting in hypersplenism, splenomegaly, and haemolytic anaemia. Patients with hereditary spherocytosis often present with jaundice, splenomegaly, anaemia, and fatigue.
Schistocytes, irregular and jagged fragments of red blood cells, are not typically seen in hereditary spherocytosis. They are the result of mechanical destruction of red blood cells in conditions such as haemolytic anaemia.
Acanthocytes or spur cells, which have a spiked, irregular surface due to deposition of lipids and/or proteins on the membrane, are not typically seen in hereditary spherocytosis. They are seen in several conditions, including cirrhosis, anorexia nervosa, and pancreatitis.
Microcytic red blood cells, which are smaller than normal red blood cells but have a normal shape, are typically seen in iron deficiency anaemia, thalassaemia, and anaemia of chronic disease.
Teardrop-shaped red blood cells are seen in conditions where there is an abnormality of bone marrow function, such as myelofibrosis. This is different from hereditary spherocytosis, which is a primary disorder of abnormal red blood cell shape.
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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 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: Blood culture
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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