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  • Question 1 - A 14-year-old boy with thalassaemia major, receiving regular blood transfusions, has been added...

    Incorrect

    • A 14-year-old boy with thalassaemia major, receiving regular blood transfusions, has been added to the transplant waiting list for chronic heart failure. What is the probable reason for his heart failure?

      Your Answer: Transfusion-related bacterial endocarditis

      Correct Answer: Transfusion haemosiderosis

      Explanation:

      Complications of Blood Transfusions: Understanding the Risks

      Blood transfusions are a common medical intervention used to treat a variety of conditions, from severe bleeding to anaemia. While they can be life-saving, they also carry certain risks and potential complications. Here are some of the most common complications associated with blood transfusions:

      Transfusion haemosiderosis: Repeated blood transfusions can lead to the accumulation of iron in the body’s organs, particularly the heart and endocrine system. This can cause irreversible heart failure if left untreated.

      High-output cardiac failure: While anaemia on its own may not be enough to cause heart failure, it can exacerbate the condition in those with reduced left ventricular systolic dysfunction.

      Acute haemolytic transfusion reaction: This occurs when there is a mismatch between the major histocompatibility antigens on blood cells, such as the ABO system. It can cause severe intravascular haemolysis, disseminated intravascular coagulation, renal failure, and shock, and has a high mortality rate if not recognized and treated quickly.

      Pulmonary oedema: While rare in patients with normal left ventricular systolic function, blood transfusions can cause fluid overload and pulmonary oedema, which can exacerbate chronic heart failure.

      Transfusion-related bacterial endocarditis: While rare, bacterial infections can occur from blood transfusions. Platelet pools, which are stored at room temperature, have a slightly higher risk of bacterial contamination that can cause fulminant sepsis.

      Understanding the potential complications of blood transfusions is important for both patients and healthcare providers. By recognizing and addressing these risks, we can ensure that blood transfusions remain a safe and effective treatment option for those who need them.

    • This question is part of the following fields:

      • Haematology
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  • Question 2 - 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 3 - A 32-year-old woman comes to Haematology complaining of fatigue, anaemia and splenomegaly. She...

    Correct

    • 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 4 - What is true for a patient with blood type group O before receiving...

    Correct

    • What is true for a patient with blood type group O before receiving a blood transfusion?

      Your Answer: The red cells have absent A/B antigen and plasma has anti A and anti B antibodies

      Explanation:

      Blood Groups

      Blood groups are determined by the presence or absence of certain antigens on the surface of red blood cells and the corresponding antibodies in the plasma. Blood Group O has no A or B antigens on the red cells and has both anti-A and anti-B antibodies in the plasma. Blood Group AB has both A and B antigens on the red cells but no antibodies in the plasma. Blood Group A has only A antigens on the red cells and anti-B antibodies in the plasma. Blood Group B has only B antigens on the red cells and anti-A antibodies in the plasma. It is important to know your blood group for medical purposes, such as blood transfusions, as incompatible blood types can cause serious health complications.

    • This question is part of the following fields:

      • Haematology
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  • Question 5 - A 35-year-old woman is 10 weeks pregnant. She plans to undergo a Down's...

    Correct

    • A 35-year-old woman is 10 weeks pregnant. She plans to undergo a Down's syndrome screening test around 15 weeks into her pregnancy.

      What is included in the measurement of a Down's screening blood test?

      Your Answer: Alpha-fetoprotein

      Explanation:

      AFP Measurement for Detecting Birth Defects and Chromosomal Abnormalities

      When a woman is 15 weeks pregnant, a blood test called AFP measurement can be performed to determine if there is an increased risk of certain birth defects and chromosomal abnormalities. This test can detect open neural tube or abdominal wall defects, as well as Down’s syndrome and trisomy 18. In the past, if the results of the AFP measurement were abnormal, an ultrasound scan would be performed. However, it is possible that in the future, mid-trimester anomaly scanning may replace the use of AFP measurement altogether.

    • This question is part of the following fields:

      • Haematology
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  • Question 6 - A 75-year-old woman who is in hospital for pneumonia begins to deteriorate on...

    Incorrect

    • A 75-year-old woman who is in hospital for pneumonia begins to deteriorate on her third day of intravenous antibiotics. She develops purple bruises on her skin and on the inside of her mouth, and tells you that she feels short of breath and fatigued. Her platelet count has dropped from 165 × 109/l to 43 × 109/l over the last two days. She also complains of blurred vision in the last few hours.
      Which of the following will form part of her initial management?

      Your Answer: Platelet transfusions

      Correct Answer: Plasma exchange

      Explanation:

      Treatment Options for Thrombotic Thrombocytopenic Purpura

      Thrombotic thrombocytopenic purpura (TTP) is a medical emergency that requires prompt treatment. The most common initial management for TTP is plasma exchange, which aims to remove the antibodies that block the ADAMTS13 enzyme and replace the ADAMTS13 enzymes in the blood. Intravenous methylprednisone and rituximab may also be used in conjunction with plasma exchange.

      Aspirin should only be considered when the platelet count is above 50 × 109/l, and even then, it is not an essential part of initial management and will depend on the patient’s comorbidities. Cryoprecipitate is not recommended for TTP treatment, as it is indicated for disseminated intravascular coagulation or fibrinogen deficiency.

      Factor VIII infusion is used for haemophilia A, a C-linked-recessive disorder that presents with excessive bleeding and anaemia, and is less likely to be associated with thrombocytopenia and TTP. Platelet transfusions are relatively contraindicated in TTP and should only be considered in cases of catastrophic bleeding or urgent surgery that cannot wait until after plasma exchange. Platelet transfusions increase the risk of arterial thrombosis, which can lead to myocardial infarction and stroke.

      In summary, plasma exchange is the most common initial management for TTP, and other treatment options should be carefully considered based on the patient’s individual circumstances. Early diagnosis and prompt treatment are crucial for a successful outcome.

    • This question is part of the following fields:

      • Haematology
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  • Question 7 - A plasma donor on a continuous flow separator machine experiences light headedness, muscle...

    Incorrect

    • 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: Reassure patient that symptoms will subside with oral hydration

      Correct 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 8 - A 14-year-old girl presents with a history of easy bruising and excessive bleeding...

    Incorrect

    • A 14-year-old girl presents with a history of easy bruising and excessive bleeding after a dental procedure. She is diagnosed with von Willebrand's disease and is scheduled for additional dental extractions. The physician prescribes DDAVP. What is the mechanism of action of DDAVP in treating von Willebrand's disease?

      Your Answer: Acts as a substitute carrier for factor VIII

      Correct Answer: Stimulates release of von Willebrand's factor from endothelial cells

      Explanation:

      DDAVP for Increasing von Willebrand Factor

      DDAVP is a medication that can be administered to increase the amount of von Willebrand factor in the body, which is necessary for surgical or dental procedures. This medication can increase plasma von Willebrand factor and factor VIII concentrations by two to five times. The mechanism of action involves the induction of cyclic adenosine monophosphate (cAMP)-mediated vWF secretion through a direct effect on endothelial cells. Overall, DDAVP is a useful tool for increasing von Willebrand factor levels in the body, allowing for safer and more successful surgical and dental procedures.

    • This question is part of the following fields:

      • Haematology
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  • Question 9 - A 70-year-old man presents with increasing shortness of breath on exertion. He also...

    Incorrect

    • A 70-year-old man presents with increasing shortness of breath on exertion. He also notes he has been bruising more easily of late. He is noted to be in sinus tachycardia but otherwise is haemodynamically stable. Examination reveals conjunctival pallor and hepatosplenomegaly. No definite lymphadenopathy is palpable. A full blood count is performed:
      Investigation Result Normal value
      Haemoglobin 69 g/l 135–175 g/l
      White cell count (WCC) 0.7 × 109/l 4.0–11.0 × 109/l
      Platelets 14 × 109/l 150–400 × 109/l
      Blood film is reported as a leukoerythroblastic picture with teardrop-shaped erythrocytes. A bone marrow aspirate is attempted, but this is unsuccessful.
      What is the likely diagnosis?

      Your Answer: Acute myelocytic leukaemia

      Correct Answer: Myelofibrosis

      Explanation:

      Understanding Myelofibrosis: A Comparison with Other Bone Marrow Disorders

      Myelofibrosis is a rare disorder that primarily affects older patients. It is characterized by bone marrow failure, which can also be found in other diseases such as advanced prostate cancer, acute lymphoblastic leukemia, acute myelocytic leukemia, and chronic myeloid leukemia. However, myelofibrosis can be distinguished from these other disorders by specific diagnostic clues.

      One of the key diagnostic features of myelofibrosis is the presence of a leukoerythroblastic picture with teardrop-shaped red blood cells, which is also seen in advanced prostate cancer. However, in myelofibrosis, a failed bone marrow aspirate, or dry tap, is frequent and a bone marrow trephine biopsy is needed for diagnosis. This is not the case in other bone marrow disorders.

      Myelofibrosis is caused by the proliferation of megakaryocytes, which leads to intense bone marrow fibrosis, marrow failure, and secondary hepatosplenomegaly due to extramedullary hematopoiesis. Patients may present with systemic upset, symptoms of marrow failure, or abdominal discomfort from hepatosplenomegaly. Treatment is supportive, with bone marrow transplant reserved for younger patients. The median survival is 4-5 years, and transformation to acute myeloid leukemia is relatively common.

      In contrast, acute lymphoblastic leukemia is a disease of childhood that presents with elevated white cell count and blasts on peripheral blood film. Acute myelocytic leukemia and chronic myeloid leukemia both present with raised white cell counts and blasts on blood film, but are more common in younger patients. Advanced prostate cancer may cause bone marrow failure if there is replacement of enough bone marrow by metastases, but patients would also complain of bone pain.

      In summary, while bone marrow failure may be found in various diseases, specific diagnostic clues such as a leukoerythroblastic picture with teardrop-shaped red blood cells and a failed bone marrow aspirate can help distinguish myelofibrosis from other bone marrow disorders.

    • This question is part of the following fields:

      • Haematology
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  • Question 10 - 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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