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  • Question 1 - A 36-year-old woman, who has received a 3-unit blood transfusion following a hysterectomy,...

    Correct

    • 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 2 - A 35-year-old woman presents to the hospital with a painful and swollen right...

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    • A 35-year-old woman presents to the hospital with a painful and swollen right leg that has been bothering her for the past three days. She has a history of deep vein thrombosis in the same leg ten years ago after returning from her honeymoon in Australia. Additionally, she was treated for pleurisy three years ago by her general practitioner. She has been experiencing recurrent water infections for the past two years, with intermittent bouts of flank pain and dark urine in the morning. Her general practitioner has recently investigated this issue, but the ultrasound scan of her renal tract and intravenous pyelogram were normal. She works as a cashier in a building society and lives with her husband and two children. She does not have a recent history of travel, but she smokes 10 cigarettes daily and occasionally drinks alcohol. On examination, her right calf is swollen, red, and tender, but she appears otherwise well. Her blood tests show low hemoglobin, white cell count, and platelets, as well as elevated bilirubin and alkaline phosphatase. What is the most likely diagnosis?

      Your Answer: Paroxysmal nocturnal haemoglobinuria

      Explanation:

      Paroxysmal Nocturnal Haemoglobinuria: A Clonal Defect of Red Cells

      Paroxysmal nocturnal haemoglobinuria (PNH) is a condition where red blood cells have an increased susceptibility to lysis by complement due to an acquired clonal defect. This disorder typically presents in young adults and is often associated with other stem cell disorders, such as aplastic anaemia. The classic symptom of PNH is the intermittent passage of bloody urine, which tends to occur more frequently at night for unknown reasons. Diagnosis is often made through investigation of anaemia, pancytopenia, or recurrent thrombotic episodes, which are likely caused by complement-induced platelet aggregation. Flow cytometry can confirm the diagnosis by demonstrating a lack of erythrocyte membrane proteins CD59 and decay accelerating factor (DAF).

      Overall, PNH is a rare but serious condition that can lead to significant complications if left untreated. Early diagnosis and management are crucial for improving outcomes and preventing further damage to the body.

    • This question is part of the following fields:

      • Haematology
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  • Question 3 - A worried mother brings her 7-year-old son to the Emergency Department as she...

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    • A worried mother brings her 7-year-old son to the Emergency Department as she is concerned about his left knee. The child injured his knee while playing outside, and the knee is now significantly swollen and he is experiencing a lot of pain. The mother mentions that the child bruises easily. The mother herself does not have any such issues, but her sister had similar problems when she was young.
      What is the most probable pathophysiologic mechanism responsible for this boy's symptoms?

      Your Answer: Deficiency of a clotting factor in the intrinsic pathway of coagulation

      Explanation:

      Pathophysiological Mechanisms of Various Medical Conditions

      Haemophilia: Deficiency of a Clotting Factor in the Intrinsic Pathway of Coagulation
      Haemophilia is an X-linked recessive condition that affects the intrinsic pathway of coagulation. It is caused by a mutation in factor VIII or IX, leading to deficient coagulation. Patients present with excessive bleeding, such as spontaneous bruising, prolonged bleeding following a dental procedure or minor injury, bleeding into the joints (haemarthrosis), and epistaxis. Treatment involves correcting the deficiency with concentrated factor VIII or IX.

      Von Willebrand’s Disease: Deficiency of a Protein Found in Endothelial Cells and Released by Endothelial Damage
      Von Willebrand’s disease is an autosomal dominant, inherited bleeding disorder caused by a deficiency of the von Willebrand factor. This protein is found in the endothelial cells lining the vessels and is released following endothelial damage. It promotes adhesion of platelets to the area of damage and stabilizes factor VIII, both actions promoting haemostasis. Symptoms include easy bruising and prolonged bleeding following minimal trauma.

      Ewing’s Sarcoma: Translocation Between Chromosomes 11 and 22
      Ewing’s sarcoma is a malignant bone tumour seen in children and young adults. It is caused by a translocation between chromosomes 11 and 22.

      Leukaemia: Invasion of Bone Marrow by Leukaemic Cells
      Leukaemia is a type of cancer that affects the blood and bone marrow. It is caused by the invasion of bone marrow by leukaemic cells, leading to pancytopenia, a condition in which there is a deficiency of all three types of blood cells: red blood cells, white blood cells, and platelets. Symptoms include fatigue, weakness, shortness of breath, and increased susceptibility to infections. Treatment involves chemotherapy, radiation therapy, and bone marrow transplantation.

    • This question is part of the following fields:

      • Haematology
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  • Question 4 - A patient on the ward receiving a blood transfusion after knee replacement surgery...

    Correct

    • 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 5 - A 67-year-old man was admitted with pain in the left groin. He had...

    Correct

    • 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 6 - Which autoantibody is correctly matched with its associated disease in the following list?...

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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 7 - A 35-year-old woman comes to the clinic for follow-up three months after undergoing...

    Correct

    • A 35-year-old woman comes to the clinic for follow-up three months after undergoing evacuation of a hydatidiform mole. She reports feeling well and has not experienced any significant issues since the procedure.

      What blood test is the most effective in monitoring for the recurrence of trophoblastic disease?

      Your Answer: Beta-HCG

      Explanation:

      Hydatidiform Mole and Trophoblastic Disease

      A hydatidiform mole is a type of abnormal pregnancy that only generates placental tissue. However, approximately 10% of cases of hydatidiform mole can transform into malignant trophoblastic disease. To assess if there is any retained tissue or recurrence/malignant transformation, the best way is to measure the levels of HCG, which is primarily produced by the placenta. On the other hand, alpha-fetoprotein, CEA, and CA-125 are tumour markers associated with hepatocellular carcinoma, colonic carcinoma, and ovarian carcinoma, respectively. It is important to note that progesterone levels are not useful in determining the prognosis of trophoblastic disease. the characteristics and markers of hydatidiform mole and trophoblastic disease is crucial in the diagnosis and management of these conditions.

    • This question is part of the following fields:

      • Haematology
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  • Question 8 - A 50-year-old female patient visits the clinic with complaints of abdominal discomfort and...

    Correct

    • 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 9 - The most common error in transfusion according to the SHOT (serious hazards of...

    Correct

    • 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 10 - A 14-year-old girl with known sickle-cell disease presents to the Emergency Department with...

    Incorrect

    • A 14-year-old girl with known sickle-cell disease presents to the Emergency Department with severe abdominal pain.
      On examination, she is found to have a tachycardia of 130 bpm, with generalised abdominal tenderness and 3 cm splenomegaly. Blood tests reveal marked anaemia, and a diagnosis of splenic sequestration crisis is considered.
      Which blood vessel in the spleen is most responsible for monitoring the quality of red blood cells and removing aged ones from circulation?

      Your Answer: Trabecular artery

      Correct Answer: Splenic sinusoid

      Explanation:

      The Anatomy of the Spleen: Splenic Sinusoids, Trabecular Veins, Arteries, and Sheathed Capillaries

      The spleen is an important organ in the immune system, responsible for filtering blood and removing old or damaged red blood cells. Its unique anatomy allows it to perform this function effectively.

      One key component of the spleen is the splenic sinusoid. These sinusoids are lined with elongated, cuboidal endothelial cells that are closely associated with macrophages. The gaps between the endothelial cells and incomplete basement membrane allow for the passage of red blood cells, with younger and more deformable cells passing through easily while older or abnormal cells are more readily destroyed by the macrophages.

      The trabecular veins receive blood from the splenic sinusoids, while the trabecular arteries are branches of the afferent splenic artery. These arteries pass deep into the spleen along connective tissue trabeculae and branch into central arteries that pass through the white pulp of the spleen.

      The central arteries then lead to sheathed capillaries, which are branches of the central arteries. These capillaries open directly into the red pulp of the spleen, allowing for further filtration and removal of old or damaged red blood cells.

      Overall, the anatomy of the spleen is complex and specialized, allowing it to perform its important functions in the immune system.

    • This question is part of the following fields:

      • Haematology
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  • Question 11 - A 68-year-old woman presents with lethargy and poor exercise tolerance. She also mentions...

    Correct

    • A 68-year-old woman presents with lethargy and poor exercise tolerance. She also mentions that, over the last month, she has had excessive thirst and polyuria.
      Initial investigations show:
      Investigation Result Normal value
      Haemoglobin (Hb) 78 g/l 115–155 g/l
      Erythrocyte sedimentation rate (ESR) 109 mm/h 0–10mm in the first hour
      Sodium (Na+) 134 mmol/l 135–145 mmol/l
      Potassium (K+) 5.8 mmol/l 3.5–5.0 mmol/l
      Urea 26.5 mmol/l 2.5–6.5 mmol/l
      Creatinine 268 µmol/l 50–120 μmol/l
      Corrected calcium (Ca2+) 3.02 mmol/l 2.20–2.60 mmol/l
      Glucose 5.2 mmol/l 3.5–5.5 mmol/l
      Which of the following tests is the most appropriate to confirm the underlying diagnosis?

      Your Answer: Serum and urine electrophoresis

      Explanation:

      Diagnostic Tests and Differential Diagnosis for a Patient with Multiple Derangements

      The patient in question presents with several abnormalities in their blood tests, including anaemia, hypercalcaemia, electrolyte imbalances, and a significantly elevated ESR. These findings, along with the patient’s symptoms, suggest a diagnosis of malignancy, specifically multiple myeloma.

      Multiple myeloma is characterized by the malignant proliferation of plasma cells, leading to bone marrow infiltration, pancytopenia, osteolytic lesions, hypercalcaemia, and renal failure. The ESR is typically elevated in this condition. To confirm a diagnosis of multiple myeloma, serum and urine electrophoresis can be performed to identify the presence of monoclonal antibodies and Bence Jones proteins, respectively. Bone marrow examination can also reveal an increased number of abnormal plasma cells.

      Treatment for multiple myeloma typically involves a combination of chemotherapy and bisphosphonate therapy, with radiation therapy as an option as well. This condition is more common in men, particularly those in their sixth or seventh decade of life.

      Other diagnostic tests that may be considered include an oral glucose tolerance test (to rule out diabetes as a cause of polydipsia and polyuria), a chest X-ray (to evaluate for a possible small cell carcinoma of the lung), and an abdominal CT scan (to assess the extent of disease and the presence of metastasis). A serum PTH level may also be useful in ruling out primary hyperparathyroidism as a cause of hypercalcaemia, although the patient’s symptoms and blood test results make malignancy a more likely diagnosis.

      Diagnostic Tests and Differential Diagnosis for a Patient with Multiple Derangements

    • This question is part of the following fields:

      • Haematology
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  • Question 12 - A 6-year-old child of African descent presents with a 2-day history of increasing...

    Correct

    • 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 13 - A 5-year-old girl presents with purpura following a recent upper respiratory infection. Upon...

    Correct

    • 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: 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 14 - A 70-year-old man presents with fatigue, pallor and shortness of breath. He has...

    Incorrect

    • A 70-year-old man presents with fatigue, pallor and shortness of breath. He has been battling with an indolent colon carcinoma for the past 5 years. He also suffers from insulin-dependent diabetes, hypertension, coronary artery disease and rheumatoid arthritis. He has been feeling unwell for the past few weeks. He denies any history of melaena or haematochezia and has been amenorrhoeic for decades. A bedside stool guaiac test is negative for any blood in the stool. He is well nourished, reports taking daily supplements and is not a vegetarian. He reports that his haematocrit is 0.28 (0.35–0.55) and haemoglobin level 100 g/l (115–155 g/l).
      What additional findings would you expect to observe in his full blood count?

      Your Answer: Reticulocytosis

      Correct Answer: Increased ferritin

      Explanation:

      Understanding Anaemia of Chronic Disease: Increased Ferritin and Decreased TIBC

      Anaemia of chronic disease is a type of anaemia that is commonly seen in patients with chronic inflammatory conditions. It is characterised by a low haemoglobin level and low haematocrit, but unlike iron deficiency anaemia, it is associated with increased ferritin levels and decreased total iron-binding capacity (TIBC). This is because ferritin is a serum reactive protein that is elevated in response to the underlying inflammatory process.

      Diagnosis of anaemia of chronic disease requires the presence of a chronic inflammatory condition and anaemia, which can be either normocytic or microcytic. It is important to note that a haemoglobin level of <80 g/l is very rarely associated with this type of anaemia. Treatment involves addressing the underlying disorder causing the anaemia and monitoring the haemoglobin level. Blood transfusion is only used in severe cases. It is important to differentiate anaemia of chronic disease from other types of anaemia. For example, it is characterised by a low reticulocyte count, and not reticulocytosis. Serum transferrin receptor is not affected in anaemia of chronic disease and would therefore be normal. Additionally, TIBC is reduced in anaemia of chronic disease, whereas it is increased in iron deficiency anaemia. Finally, anaemia of chronic disease is associated with either microcytosis or normocytosis, whereas macrocytosis is associated with other types of anaemia such as folate deficiency, vitamin B12 deficiency, alcohol excess, and myelodysplastic disease. In summary, understanding the unique features of anaemia of chronic disease, such as increased ferritin and decreased TIBC, can aid in its diagnosis and management.

    • This question is part of the following fields:

      • Haematology
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  • Question 15 - By how much does the haemoglobin concentration increase with the infusion of one...

    Incorrect

    • By how much does the haemoglobin concentration increase with the infusion of one unit of fresh blood?

      Your Answer: 20 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 16 - As a part of a haematology rotation, a final-year medical student is asked...

    Correct

    • As a part of a haematology rotation, a final-year medical student is asked to give a PowerPoint presentation to the team about a 20-year-old patient who presented with sickle-cell crisis.
      With regard to sickle-cell disease, which of the following statements is correct?

      Your Answer: Aplastic crisis can be precipitated by parvovirus B19

      Explanation:

      Understanding Aplastic Crisis and Sickle-Cell Disease

      Aplastic crisis is a condition of transient bone marrow failure that can be precipitated by parvovirus B19, Epstein–Barr virus (EBV), or Streptococcus. In sickle-cell disease, aplastic crisis is usually caused by parvovirus B19 and is characterized by reticulocytopenia, symptomatic anemia, and the presence of parvovirus immunoglobulin M (IgM) antibodies. It is managed by monitoring and symptomatic relief with blood transfusion until normal erythrocyte function returns. Aplastic crisis is most common in individuals of Mediterranean descent.

      Sickle-cell disease is most common in individuals of Black Afro-Caribbean descent and, to a lesser extent, in individuals of Mediterranean or Middle Eastern descent. It occurs as a result of the production of an abnormal beta (β) chain in haemoglobin, caused by a mutation that changes adenine to thymine in the sixth codon of the β chain gene. This results in the formation of HbS, which circulates in the blood and forms polymers in the deoxygenated state, causing sickling of red blood cells. The resulting blood film shows elongated, thin, sickled red blood cells, target cells, and Howell–Jolly bodies.

      Splenomegaly is most usually seen in childhood, as most children with sickle-cell disease have a splenic infarction event in late childhood and develop hyposplenism. Spherocytes, on the other hand, are abnormal red blood cells with a spherical shape, seen on the blood film of spherocytosis, a form of haemolytic anaemia.

    • This question is part of the following fields:

      • Haematology
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  • Question 17 - A 67-year-old man presented with fever and body ache for 6 months. His...

    Incorrect

    • A 67-year-old man presented with fever and body ache for 6 months. His blood tests revealed a haemoglobin level of 110 g/l and erythrocyte sedimentation rate (ESR) of 121 mm in the first hour. Serum protein electrophoresis revealed an M band in the gamma globulin region with a total IgG level of 70 g/l. Bone marrow biopsy shows plasma cells in the marrow of 11%. A skeletal survey reveals no abnormalities. Other blood tests revealed:
      Test Parameter Normal range
      Calcium 2.60 mmol/l 2.20–2.60 mmol/l
      Creatinine 119 μmol/l 50–120 μmol/l
      Phosphate 1.30 mmol/l 0.70–1.40 mmol/l
      Potassium (K+) 4.6 mmol/l 3.5–5.0 mmol/l
      Lactate dehydrogenase 399 IU/l 100–190 IU/l
      His body weight was 80 kg.
      What is his condition better known as?

      Your Answer: Multiple myeloma

      Correct Answer: Smouldering myeloma

      Explanation:

      Smouldering myeloma is a stage between monoclonal gammopathy of unknown significance (MGUS) and myeloma. To diagnose this condition, the patient must have a monoclonal protein in the serum of at least 30 g/l and monoclonal plasma cells of at least 10% in bone marrow or tissue biopsy, but no evidence of end-organ damage. Patients with smouldering myeloma should be closely monitored as they are at high risk of developing symptomatic myeloma.

      Multiple myeloma is a malignant neoplasm where there is clonal proliferation of plasma cells in the bone marrow, leading to the secretion of a monoclonal antibody and light immunoglobulin chains that cause organ damage. Patients with multiple myeloma present with various symptoms, including lethargy, bone pain, pathological fractures, renal impairment, amyloidosis, and pancytopenia due to marrow infiltration. To diagnose multiple myeloma, the patient must have a monoclonal antibody in serum and/or urine, clonal plasma cells of at least 10% on bone marrow biopsy, and evidence of end-organ damage.

      MGUS is a condition where low levels of paraprotein are detected in the blood, but they are not causing clinically significant symptoms or end-organ damage. To diagnose MGUS, the patient must have a monoclonal protein in the serum of less than or equal to 30 g/l, monoclonal plasma cells of less than or equal to 10% in bone marrow or tissue biopsy, and no evidence of end-organ damage.

      Non-secretory myeloma is a rare variant of multiple myeloma where the bone marrow findings and end-organ damage are similar to myeloma, but there is no detectable monoclonal protein in the serum or urine. This makes it difficult to diagnose.

      Plasma cell leukemia is a rare and aggressive form of multiple myeloma characterized by high levels of plasma cells circulating in the peripheral blood. It can occur as a primary condition or a secondary leukaemic transformation of multiple myeloma.

    • This question is part of the following fields:

      • Haematology
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  • Question 18 - A patient on a medical ward received a transfusion 72 hours ago for...

    Correct

    • 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 19 - A plasma donor on a continuous flow separator machine experiences light headedness, muscle...

    Correct

    • 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 20 - A 54-year-old white woman without past medical history presents with pallor, shortness of...

    Correct

    • A 54-year-old white woman without past medical history presents with pallor, shortness of breath, palpitations and difficulty balancing.
      On examination, her vitals are heart rate 110 bpm at rest and 140 bpm on ambulation, blood pressure 100/60 mmHg, respiratory rate 18 breaths/minute, temperature 37 ° C and oxygen saturation 98% on room air. She is pale. Her lungs are clear to auscultation; her heart rate is regular without murmurs, rubs or gallops; her abdomen is soft and non-tender; she is moving all extremities equally, and a stool guaiac test is heme-negative. Her gait is wide and she has difficulty balancing. She has decreased sensation to fine touch in her feet. Her mini-mental status exam is normal.
      Blood work shows:
      Haematocrit: 0.19 (0.35–0.55)
      Mean cell volume: 110 fl (76–98 fl)
      White blood cell count: 5 × 109/l (4–11 × 109/l)
      Which one of the following findings would most likely lead to the correct diagnosis?

      Your Answer: Anti-intrinsic factor antibodies

      Explanation:

      Causes and Symptoms of Vitamin B12 Deficiency

      Vitamin B12 deficiency can lead to macrocytic anaemia and neurological symptoms. The most common cause of this deficiency is the presence of anti-intrinsic factor antibodies. Intrinsic factor is necessary for the absorption of dietary vitamin B12 in the terminal ileum. Without it, vitamin B12 cannot be absorbed, leading to deficiency and anaemia. Symptoms of vitamin B12 deficiency include fatigue, lethargy, dyspnoea on exertion, and neurological symptoms such as peripheral loss of vibration and proprioception, weakness, and paraesthesiae. If left untreated, it can lead to hepatosplenomegaly, heart failure, and demyelination of the spinal cord, causing ataxia.

      Diagnosis can be made with a vitamin B12 level test, which reveals anaemia, often pancytopenia, and a raised MCV. A blood film reveals hypersegmented neutrophils, megaloblasts, and oval macrocytes. Treatment involves replacement of vitamin B12.

      Other possible causes of vitamin B12 deficiency include intestinal tapeworm, which is rare, and gastrointestinal malignancy, which causes iron deficiency anaemia with a low MCV. Destruction of the anterior and lateral horns of the spinal cord describes anterolateral sclerosis (ALS), which is characterised by progressive muscle weakness and would not cause anaemia or loss of sensation. Enlargement of the ventricles on head CT indicates hydrocephalus, which could explain the wide-based gait but not the anaemia and other symptoms. A haemoglobin A1c of 12.2% is associated with diabetes, which could explain decreased peripheral sensation to fine touch but would not be associated with megaloblastic anaemia.

    • This question is part of the following fields:

      • Haematology
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