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  • Question 1 - A 54-year-old white woman without past medical history presents with pallor, shortness of...

    Incorrect

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

      Your Answer:

      Correct Answer: Anti-intrinsic factor antibodies

      Explanation:

      Causes and Symptoms of Vitamin B12 Deficiency

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

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

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

    • This question is part of the following fields:

      • Haematology
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  • Question 2 - 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:

      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 3 - A woman has some blood tests taken by her general practitioner (GP). The...

    Incorrect

    • A woman has some blood tests taken by her general practitioner (GP). The results show a Hb of 10.0, MCV of 69 and a ferritin of 9.
      Which is the most appropriate management plan for this patient?

      Your Answer:

      Correct Answer: Treat with ferrous sulphate 200 mg three times a day and repeat bloods in three months

      Explanation:

      Understanding Iron Deficiency Anaemia and Treatment Options

      Iron deficiency anaemia is a common condition that can present with symptoms such as lethargy, tiredness, and shortness of breath on exertion. It is often seen in women due to menstruation and blood loss associated with it, as well as in pregnant women. However, it is not a common finding in men and should be investigated further if present.

      Treatment for iron deficiency anaemia involves the use of ferrous sulfate, typically at a dose of 200 mg two to three times a day for at least three months. Blood tests should be repeated after this time to assess the effectiveness of therapy. Folic acid supplementation may also be necessary in cases of folate deficiency anaemia, which presents with a raised MCV.

      It is important to investigate persistent anaemia despite adequate iron supplementation, as it may indicate an underlying malignancy. Men with unexplained iron deficiency anaemia and a haemoglobin level of < 110 g/l should be referred urgently to the gastroenterology team for investigation of upper or lower gastrointestinal malignancy. Overall, understanding the causes and treatment options for iron deficiency anaemia can help improve patient outcomes and prevent complications.

    • This question is part of the following fields:

      • Haematology
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  • Question 4 - 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:

      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 5 - A 23-year-old female patient presents at the clinic for a check-up. She complains...

    Incorrect

    • A 23-year-old female patient presents at the clinic for a check-up. She complains of irregular, heavy menstrual periods, has a BMI of 30 kg/m2, and experiences acne and excessive facial hair growth. She is not taking any medication. Her sister has been diagnosed with polycystic ovarian syndrome (PCOS), and she suspects that she may have the same condition.

      During the physical examination, her blood pressure is 149/90 mmHg, and her pulse is regular at 78 bpm. She has excessive hair growth on her beard line and upper torso, and central obesity. However, the rest of her physical examination is normal.

      Which blood test is the most diagnostic for PCOS?

      Your Answer:

      Correct Answer: Total/free testosterone

      Explanation:

      Diagnosis and Associated Risks of Polycystic Ovary Syndrome (PCOS)

      Polycystic ovary syndrome (PCOS) is diagnosed when there is evidence of at least two out of three features, with other potential causes excluded. These features include oligoamenorrhoea, elevated levels of total/free testosterone (or clinical features suggestive of hyperandrogenism), or the presence of polycystic ovaries on ultrasound. While an extremely marked elevation in testosterone can suggest an androgen-secreting tumor, this is rare. Additionally, raised luteinising hormone (LH) with a normal follicle-stimulating hormone (FSH) can lead to an elevated LH/FSH ratio, but this is not diagnostic.

      PCOS is associated with an increased risk of impaired glucose tolerance, although this may take a number of years to become apparent. Furthermore, there may be a reduction in levels of oestriol, although this is not always a consistent finding. It is important to diagnose PCOS early on to manage the associated risks and prevent potential complications.

    • This question is part of the following fields:

      • Haematology
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  • Question 6 - 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:

      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 7 - Which statement about rhesus antibodies in pregnancy is correct? ...

    Incorrect

    • Which statement about rhesus antibodies in pregnancy is correct?

      Your Answer:

      Correct Answer: Following delivery, the degree of fetomaternal haemorrhage should be calculated on a blood sample from a D negative mother

      Explanation:

      Important Points to Remember about Fetomaternal Haemorrhage

      Following the delivery of a baby, it is crucial to determine the degree of fetomaternal haemorrhage (FMH) in a D negative mother. This is done by analyzing a blood sample to adjust the dose of anti-D in the mother if she has delivered a D positive child. It is important to note that D positive and D negative women have the same likelihood of developing antibodies to other red cell antigens. Therefore, all pregnant women should undergo a blood group and antibody screen in their first trimester or at the time of presentation, whichever comes first. The fetal Rh type is determined by the Rh typing of both the mother and father. Additionally, maternal antibody titres are indicative of the degree of haemolytic disease of the newborn (HDN). For more information on the management of women with red cell antibodies during pregnancy, refer to the Royal College of Obstetricians and Gynaecologists (RCOG) Green-top Guideline No. 65.

    • This question is part of the following fields:

      • Haematology
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  • Question 8 - A 35-year-old man visits the clinic seeking guidance. He has planned a long-distance...

    Incorrect

    • A 35-year-old man visits the clinic seeking guidance. He has planned a long-distance trip to Australia and is worried about the risk of deep vein thrombosis (DVT). He has no significant medical history and is in good health.
      What is the best advice to offer him?

      Your Answer:

      Correct Answer: No aspirin, ankle exercises, aisle seat and no alcohol

      Explanation:

      Venous Thromboembolism Prophylaxis for Long-Haul Flights

      When it comes to preventing venous thromboembolism (VTE) during long-haul flights, the approach varies depending on the patient’s risk level. For low-risk patients without history of VTE, cardiac disease, major illness, or recent surgery, NICE recommends avoiding long periods of immobility by taking an aisle seat, performing ankle exercises, and wearing loose-fitting clothing. It’s also important to stay hydrated and avoid alcohol.

      Moderate to high-risk patients, on the other hand, may benefit from compression stockings, especially if they have a history of VTE, cardiac disease, varicose veins, or are pregnant or postpartum. High-risk patients with thrombophilia, cancer, or recent surgery requiring general anesthesia should also consider compression stockings.

      While some sources recommend a single dose of aspirin for VTE prophylaxis during long-haul flights, current NICE guidelines do not recommend this approach. Instead, patients should focus on staying mobile, wearing compression stockings if necessary, and staying hydrated.

    • This question is part of the following fields:

      • Haematology
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  • Question 9 - A 14-year-old girl presents to the clinic with her parents. She is worried...

    Incorrect

    • 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:

      Correct 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 10 - A 36-year-old Afro-Caribbean woman presents to the Emergency Department complaining of shortness of...

    Incorrect

    • 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:

      Correct 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 11 - A 5-year-old girl presents with purpura following a recent upper respiratory infection. Upon...

    Incorrect

    • A 5-year-old girl presents with purpura following a recent upper respiratory infection. Upon examination, her platelet count is found to be 20 ×109/L (normal range: 150-400) and a bone marrow examination reveals megakaryocyte hyperplasia. What is the correct statement regarding her condition?

      Your Answer:

      Correct Answer: A spontaneous remission is likely

      Explanation:

      Treatment and Prognosis of Acute Temporary Thrombocytopenic Purpura

      Acute temporary thrombocytopenic purpura is a condition that often occurs after a viral infection. Fortunately, 85% of children with this condition will recover within a year. Platelet transfusions are not helpful unless there is active bleeding or surgery is necessary. Instead, treatment typically involves immune suppression with medications like prednisolone or intravenous immune globulin infusions. The clotting time remains normal because the coagulation factors are not affected. However, detecting antiplatelet antibodies can be challenging with many assays. While splenectomy may be an option in some cases, it is not recommended early in the disease as it may resolve on its own within a year. Overall, with proper treatment and monitoring, most children with acute temporary thrombocytopenic purpura can expect a positive outcome.

    • This question is part of the following fields:

      • Haematology
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  • Question 12 - A 68-year-old man who has been diagnosed with severe Gram-positive sepsis is admitted...

    Incorrect

    • 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:

      Correct 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 13 - 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:

      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 14 - A 10-year-old girl injures her leg while playing soccer at the park. Her...

    Incorrect

    • A 10-year-old girl injures her leg while playing soccer at the park. Her mother takes her to the emergency doctor at their GP clinic later that day as the bleeding has not stopped. The cut is not very big and is on the girl's calf. They immediately applied pressure to the wound and then a bandage, however on examination the wound is still bleeding a little bit. The girl's mother knows that there is a bleeding disorder in her family.
      What is the most suitable course of action for this patient, given the probable diagnosis?

      Your Answer:

      Correct Answer: Give desmopressin and tranexamic acid

      Explanation:

      Treatment Options for Bleeding Disorders: Haemophilia A and Von Willebrand Disease

      Haemophilia A, a genetic bleeding disorder affecting men, is characterized by a propensity to bleed with minor injuries. Diagnosis is made through a prolonged APTT on a background of normal PT and bleeding time. Treatment for minor bleeds includes desmopressin and tranexamic acid, while major bleeds require infusion with recombinant factor 8. Fresh-frozen plasma and platelets are used in major trauma as replacement therapy, while heparin is an anticoagulant and should be avoided. Von Willebrand factor is given once the diagnosis of Von Willebrand disease is confirmed. Children with severe haemophilia A should receive prophylactic infusion of factor 8 at least once a week until physical maturity, and those undergoing elective surgery or pregnant women will require prophylactic treatment.

    • This question is part of the following fields:

      • Haematology
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  • Question 15 - A patient on the ward receiving a blood transfusion after knee replacement surgery...

    Incorrect

    • A patient on the ward receiving a blood transfusion after knee replacement surgery is noted to have rigors.

      On examination, their temperature is 39°C (baseline 36.5°C). They also complain of abdominal pain and their blood pressure is 90/55 mmHg (baseline 126/84 mmHg).

      What is the next step in managing this patient?

      Your Answer:

      Correct Answer: Stop the transfusion immediately and commence IV fluids

      Explanation:

      Management of Acute Haemolytic Transfusion Reaction

      When a patient experiences a temperature rise of more than 2°C, abdominal pain, and hypotension after a blood transfusion, an acute haemolytic transfusion reaction should be suspected. In such cases, the transfusion must be stopped immediately, and the set should be taken down. Saline infusion should be initiated to maintain the patient’s blood pressure.

      The blood bank should be notified of the suspected reaction, and a sample may need to be collected for further investigation. However, the priority is to manage the patient’s symptoms and prevent further complications. If the reaction is severe, the transfusion should not be continued.

      In summary, prompt recognition and management of acute haemolytic transfusion reactions are crucial to prevent serious complications. Healthcare providers should be vigilant in monitoring patients who receive blood transfusions and act quickly if any adverse reactions occur.

    • This question is part of the following fields:

      • Haematology
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  • Question 16 - What is the diagnostic tool for beta thalassaemia? ...

    Incorrect

    • What is the diagnostic tool for beta thalassaemia?

      Your Answer:

      Correct 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.

    • This question is part of the following fields:

      • Haematology
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  • Question 17 - A 35-year-old woman with type 1 diabetes and Addison's disease presents with a...

    Incorrect

    • 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:

      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.

    • This question is part of the following fields:

      • Haematology
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  • Question 18 - In a 71-year-old man who is asymptomatic and attending clinic for an annual...

    Incorrect

    • In a 71-year-old man who is asymptomatic and attending clinic for an annual review, a full blood count result shows a mild lymphocytosis of 15 × 109/l with a few smear cells. What is the most crucial investigation to determine a diagnosis of chronic lymphocytic leukaemia (CLL)?

      Your Answer:

      Correct Answer: Peripheral blood flow cytometry

      Explanation:

      Diagnosis and Staging of Chronic Lymphocytic Leukemia

      Chronic lymphocytic leukemia (CLL) can be diagnosed through flow cytometry, which shows a specific pattern of monoclonal B cell proliferation. This pattern includes CD19/5 coexpressing, CD23 positive, and light chain restricted B cell population. However, smear cells, which are fragile lymphocytes that are smeared on the glass slide, can also be present in other lymphoproliferative disorders and benign lymphocytosis. Therefore, they do not necessarily indicate CLL.

      While CT scan and LDH are not essential for diagnosis, they are necessary for staging CLL. These investigations help determine the extent of the disease and the organs affected. Additionally, cervical lymphadenopathy, which is the enlargement of lymph nodes in the neck, may be present in CLL. However, it can also be seen in other causes of lymphadenopathy, such as viral infections or adenopathy secondary to local dental infection.

      In summary, flow cytometry is a crucial tool in diagnosing CLL, while CT scan and LDH are necessary for staging. Smear cells may be present but do not necessarily indicate CLL, and cervical lymphadenopathy can be seen in various conditions.

    • This question is part of the following fields:

      • Haematology
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  • Question 19 - A 35-year-old woman comes to the clinic for follow-up three months after undergoing...

    Incorrect

    • 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:

      Correct 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.

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      • Haematology
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  • Question 20 - A 50-year-old woman presents with headaches and nosebleeds and is found to have...

    Incorrect

    • 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:

      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.

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      • Haematology
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  • Question 21 - In the monophyletic model of haematopoiesis, which mature cell in the peripheral circulation...

    Incorrect

    • In the monophyletic model of haematopoiesis, which mature cell in the peripheral circulation is a derivative of the CMP? This model suggests that the haematopoietic stem cell (HSC) can give rise to either the common lymphoid progenitor (CLP) or the common myeloid progenitor (CMP).

      Your Answer:

      Correct Answer: Monocyte

      Explanation:

      Different Types of Blood Cells and their Progenitor Cells

      Blood cells are formed from different types of progenitor cells. The common myeloid progenitor cell gives rise to myeloblasts, pro-erythroblasts, megakaryoblasts, and monoblasts. Myeloblasts produce granulocytes, while pro-erythroblasts produce red blood cells. Megakaryoblasts give rise to megakaryocytes and platelets, and monoblasts produce monocytes that can become tissue-specific macrophages. B cells, NK cells, and T cells are derivatives of the common lymphoid progenitor cell. Plasma cells, which are antibody-secreting cells, are derived from B cells. Understanding the different types of blood cells and their progenitor cells is important for studying blood disorders and developing treatments.

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      • Haematology
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  • Question 22 - A 25-year-old patient presented with red rashes on their feet. Upon examination, they...

    Incorrect

    • A 25-year-old patient presented with red rashes on their feet. Upon examination, they were found to be pale with purpuric spots on their lower legs. Their temperature was 38.3 °C and they also complained of nausea. On the second day of admission, their fever increased and they became disoriented. New bleeding spots started appearing on their face. Blood reports revealed low hemoglobin, high white cell count, low platelets, and high creatinine levels. A peripheral blood smear showed helmet cells and anisocytosis. The CSF study was normal. What test should be done next for this patient?

      Your Answer:

      Correct Answer: Urinary β-human chorionic gonadotrophin (hCG)

      Explanation:

      The patient is presenting with thrombotic thrombocytopenic purpura (TTP), which is characterized by low platelet count due to clotting and platelet sequestration in small vessels. TTP is associated with haemolytic anaemia, thrombocytopenic purpura, fever, and neurological and renal abnormalities. The patient’s risk factors for TTP include being female, obese, pregnant, and of Afro-Caribbean origin. To determine the appropriate management, a urinary β-hCG test should be performed to establish pregnancy status. The first-line treatment for TTP is plasma exchange with fresh frozen plasma. Blood cultures should also be performed to check for underlying septicaemia. Antiplatelet antibody titres can be raised in idiopathic thrombocytopenic purpura (ITP), but ITP does not cause renal failure. A bone marrow study is appropriate to rule out leukaemia. Illicit drug use should also be considered as a cause of disseminated intravascular coagulation (DIC).

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      • Haematology
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  • Question 23 - 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:

      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 24 - A 17-year-old male patient comes in with a history of fevers, night sweats,...

    Incorrect

    • 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:

      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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      • Haematology
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  • Question 25 - A 29-year-old female patient presents to the clinic with a complaint of missed...

    Incorrect

    • 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:

      Correct 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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      • Haematology
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  • Question 26 - A 67-year-old man presents to his General Practitioner with a 5-month history of...

    Incorrect

    • A 67-year-old man presents to his General Practitioner with a 5-month history of night sweats and weight loss. He has no history of cough, shortness of breath or foreign travel. He denies any back or joint pain. He is normally fit and well, except for high blood pressure for which he takes enalapril. He continues to drink ten units of alcohol per week.
      On examination, he is noted to have cervical and groin lymphadenopathy, with an enlarged spleen. The remainder of the examination and observations are normal.
      Which of the following is most likely to be used in the management of this patient?

      Your Answer:

      Correct Answer: Rituximab

      Explanation:

      The patient in question is displaying symptoms of non-Hodgkin’s lymphoma, including night sweats, weight loss, lymphadenopathy, and splenomegaly. While other symptoms may include pruritus, fever, and shortness of breath, the most common treatment for progressive non-Hodgkin’s lymphoma is a combination chemotherapy called R-CHOP, which includes rituximab. Rituximab is an anti-CD20 monoclonal antibody used for non-Hodgkin’s lymphoma and rheumatoid arthritis. The other options, including infliximab, lenalidomide, radiotherapy, and rifampicin, are used for different conditions such as Crohn’s disease, multiple myeloma, Hodgkin’s lymphoma, and tuberculosis, respectively.

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      • Haematology
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  • Question 27 - A 35-year-old man comes to you seeking advice. He had a splenectomy ten...

    Incorrect

    • A 35-year-old man comes to you seeking advice. He had a splenectomy ten years ago after a cycling accident and has been in good health since. However, a friend recently told him that he should be receiving treatment for his splenectomy. He is currently not taking any medication.

      What would you recommend to him?

      Your Answer:

      Correct Answer: Pneumococcal vaccination

      Explanation:

      Asplenic Patients and the Importance of Vaccination

      Asplenic patients are individuals who have had their spleen removed, leaving them at risk of overwhelming bacterial infections, particularly from pneumococcus and meningococcus. To prevent such infections, it is recommended that these patients receive the Pneumovax vaccine two weeks before surgery or immediately after emergency surgery. This vaccine should be repeated every five years. Additionally, influenzae vaccination is also recommended to prevent super added bacterial infections.

      While oral penicillin is recommended for children, its long-term use in adults is a topic of debate. However, current guidance suggests that splenectomized patients should receive both antibiotic prophylaxis and appropriate immunization. It is crucial to take these preventative measures to protect asplenic patients from potentially life-threatening infections.

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      • Haematology
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  • Question 28 - A 72-year-old man is receiving a 2 units of blood transfusion for anaemia...

    Incorrect

    • A 72-year-old man is receiving a 2 units of blood transfusion for anaemia of unknown cause – haemoglobin (Hb) 65 g/l (normal 135–175 g/l). During the third hour of the blood transfusion he spikes a temperature of 38.1°C (normal 36.1–37.2°C). Otherwise the patient is asymptomatic and his other observations are normal.
      Given the likely diagnosis, what should you do?

      Your Answer:

      Correct Answer: Temporarily stop transfusion, repeat clerical checks. Then treat with paracetamol and repeat observations more regularly (every 15 minutes)

      Explanation:

      Treatment Options for Non-Haemolytic Febrile Transfusion Reaction

      Non-haemolytic febrile transfusion reaction is a common acute reaction to plasma proteins during blood transfusions. If a patient experiences this reaction, the transfusion should be temporarily stopped, and clerical checks should be repeated. The patient should be treated with paracetamol, and observations should be repeated more regularly (every 15 minutes).

      If the patient’s temperature is less than 38.5 degrees, and they are asymptomatic with normal observations, the transfusion can be continued with more frequent observations and paracetamol. However, if the patient experiences transfusion-associated circulatory overload, furosemide is a suitable treatment option.

      Adrenaline is not needed unless there are signs of anaphylaxis, and antihistamines are only suitable for urticaria during blood transfusions. Therefore, it is essential to identify the specific type of transfusion reaction and provide appropriate treatment accordingly.

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      • Haematology
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  • Question 29 - Which autoantibody is correctly matched with its associated disease in the following list?...

    Incorrect

    • Which autoantibody is correctly matched with its associated disease in the following list?

      Your Answer:

      Correct Answer: Pernicious anaemia and anti-intrinsic factor antibodies

      Explanation:

      Autoimmune Disorders and Associated Antibodies

      Autoimmune disorders occur when the immune system mistakenly attacks healthy cells and tissues in the body. These disorders are often associated with the presence of specific antibodies that can help diagnose and monitor the disease. Here are some examples:

      Pernicious Anaemia and Anti-Intrinsic Factor Antibodies
      Pernicious anaemia is a type of anaemia caused by a deficiency in vitamin B12. It is associated with the presence of anti-intrinsic factor antibodies, which bind to intrinsic factor and prevent the absorption of vitamin B12 in the gut.

      Primary Biliary Cholangitis and Anti-Jo-1 Antibodies
      Primary biliary cholangitis is an autoimmune disorder that affects the liver. It is associated with the presence of anti-mitochondrial antibodies, but not anti-Jo-1 antibodies, which are associated with other autoimmune disorders like polymyositis and dermatomyositis.

      Myasthenia Gravis and Voltage-Gated Calcium Channel Antibodies
      Myasthenia gravis is a neuromuscular disorder that causes muscle weakness and fatigue. It is associated with the presence of anti-acetylcholine receptor antibodies, but not anti-striated muscle antibodies, which are found in other autoimmune disorders.

      Granulomatosis with Polyangiitis (GPA) and Anti-Myeloperoxidase (p-ANCA) Antibody
      GPA is a type of vasculitis that affects small and medium-sized blood vessels. It is associated with the presence of cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), but not p-ANCA, which are found in other types of vasculitis.

      Hashimoto’s Thyroiditis and Thyroid-Stimulating Antibodies
      Hashimoto’s thyroiditis is an autoimmune disorder that affects the thyroid gland. It is associated with the presence of anti-thyroglobulin and anti-thyroperoxidase antibodies, which attack the thyroid gland and cause inflammation.

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      • Haematology
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  • Question 30 - A 19-year-old Afro-Caribbean male with sickle cell disease complains of right upper abdominal...

    Incorrect

    • 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:

      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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      • Haematology
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