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Question 1
Correct
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A 36-year-old woman, who has received a 3-unit blood transfusion following a hysterectomy, begins to feel unwell with a fever and chills 3 or 4 days later. There are no obvious signs of active bleeding. On examination, you note that she is mildly jaundiced.
Investigations:
Investigation Result Normal value
Pre-transfusion haemoglobin (Hb) 92 g/l 115–155 g/l
Post-transfusion Hb 98 g/l 115–155 g/l
Platelets 180 × 109/l 150–400 × 109/l
White cell count (WCC) 7.2 × 109/l 4–11 × 109/l
Sodium (Na+) 138 mmol/l 135–145 mmol/l
Potassium (K+) 4.9 mmol/l 3.5–5.0 mmol/l
Creatinine 130 μmol/l 50–120 µmol/l
Bilirubin 90 μmol/l 2–17 µmol/l
Alanine aminotransferase (ALT) 50 IU/l 5–30 IU/l
What is the most likely diagnosis for this 36-year-old woman who has received a 3-unit blood transfusion following a hysterectomy and is experiencing fever, chills, and mild jaundice without signs of active bleeding?Your Answer: Delayed transfusion haemolysis
Explanation:Delayed Transfusion Haemolysis: A Possible Cause for Minimal Increase in Haemoglobin
A patient who has received a 3-unit transfusion has only shown a minimal increase in haemoglobin levels, which is a cause for concern. The rise in bilirubin without a corresponding increase in liver enzymes suggests haemolysis from a delayed transfusion haemolysis reaction. This type of reaction occurs when a patient without certain red cell antigens is exposed to these antigens through blood transfusion, resulting in the development of new antibodies and haemolysis after 3-14 days. Symptoms include fevers, rigors, rash, and jaundice, which are less severe and more gradual in onset than acute haemolytic reactions.
Medical management of this patient will involve screening for a wider range of possible antigens and access to a blood bank with a sufficient number of available units for a clean transfusion. Serious complications of blood transfusion are rare due to screening techniques, leukocyte depletion, and improved collection and storage.
Other potential transfusion-related reactions and their approximate time-course include hyperacute (minutes to hours), acute (hours to days), and late (days or longer). Bacterial sepsis is a possible reaction that would occur most likely in the acute time course, but it does not explain the minimal increase in haemoglobin following a 3-unit transfusion. Further haemorrhage, cholestasis of pregnancy, and disseminated intravascular coagulation (DIC) are also unlikely causes.
In conclusion, delayed transfusion haemolysis is a possible cause for the minimal increase in haemoglobin levels in this patient. It is important to consider this reaction and manage it appropriately to prevent further complications.
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This question is part of the following fields:
- Haematology
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Question 2
Correct
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A 25-year-old female presents with severe anaemia and a skull x-ray shows a 'hair on end' appearance. Where is this appearance most commonly observed on a skull x-ray?
The 'hair on end' appearance is a radiological finding that can be seen on a skull x-ray. It is characterized by the presence of multiple small, spiculated bony projections that resemble the hair on a brush. This appearance is most frequently observed in conditions that cause bone marrow expansion, such as severe anaemia, thalassemia, and sickle cell disease. In these conditions, the bone marrow produces an increased number of red blood cells, leading to expansion of the bone marrow cavity and compression of the surrounding bone. This results in the characteristic 'hair on end' appearance on skull x-ray.Your Answer: Thalassaemia
Explanation:Skeletal Abnormalities in Thalassaemia Major
Patients with thalassaemia major often exhibit skeletal abnormalities, which can be observed in various parts of the body. One of the most notable changes is an expanded bone marrow space, which leads to the thinning of the bone cortex. This can be particularly striking in the skull, where it can cause a hair on end appearance. While this phenomenon can also occur in sickle cell disease, it is much less common.
In addition to changes in the skull, bone abnormalities can also be seen in the long bones, vertebrae, and pelvis. These changes can have a significant impact on a patient’s quality of life, as they can cause pain, deformities, and other complications. As such, it is important for healthcare providers to be aware of these skeletal abnormalities and to monitor patients for any signs of progression or deterioration. With proper management and treatment, many of these complications can be mitigated or prevented, allowing patients with thalassaemia major to lead healthy and fulfilling lives.
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This question is part of the following fields:
- Haematology
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Question 3
Correct
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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
Incorrect
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A patient on a medical ward received a transfusion 72 hours ago for symptomatic anaemia on a background of chronic renal disease and obstructive airways disease. He has a history of previous transfusions in the last six months. The patient, who is in his 60s, has now dropped his Hb by 20 g/L compared to his pretransfusion level and reports a dark coloured urine. The LDH and bilirubin are elevated. What is the most likely explanation for these findings?
Your Answer: Acute haemolytic transfusion reaction
Correct Answer: Delayed haemolytic transfusion reaction
Explanation:Delayed Haemolytic Transfusion Reaction
A delayed haemolytic transfusion reaction can occur 24 hours after a transfusion in patients who have been previously immunised through transfusions or pregnancy. Initially, the antibodies are not detectable, but they become apparent as a secondary immune response to the antigen exposure during the transfusion. In such cases, it is essential to carry out a haemoglobin level, blood film, LDH, direct antiglobulin test, renal profile, serum bilirubin, haptoglobin, and urinalysis for haemoglobinuria. Additionally, the group and antibody screen should be repeated.
It is unlikely that the patient is experiencing a transfusion-associated graft versus host disease or acute hepatitis as both would occur within a week or two. Furthermore, this is not an acute haemolysis that would be expected to occur during the transfusion. The rise in bilirubin and LDH levels indicates a haemolytic reaction. Therefore, it is crucial to monitor the patient’s condition and provide appropriate treatment.
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This question is part of the following fields:
- Haematology
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Question 5
Correct
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A 35-year-old woman is 10 weeks pregnant. She plans to undergo a Down's syndrome screening test around 15 weeks into her pregnancy.
What is included in the measurement of a Down's screening blood test?Your Answer: Alpha-fetoprotein
Explanation:AFP Measurement for Detecting Birth Defects and Chromosomal Abnormalities
When a woman is 15 weeks pregnant, a blood test called AFP measurement can be performed to determine if there is an increased risk of certain birth defects and chromosomal abnormalities. This test can detect open neural tube or abdominal wall defects, as well as Down’s syndrome and trisomy 18. In the past, if the results of the AFP measurement were abnormal, an ultrasound scan would be performed. However, it is possible that in the future, mid-trimester anomaly scanning may replace the use of AFP measurement altogether.
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This question is part of the following fields:
- Haematology
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Question 6
Correct
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A 50-year-old male has a history of severe rheumatoid arthritis for the past four years. Despite being compliant with therapy (NSAIDs and methotrexate), the disease remains poorly controlled. Recently, he has been experiencing extreme fatigue. Upon conducting an FBC, the following results were obtained:
- Haemoglobin 70 g/L (120-160)
- White cell count 1.5 ×109/L (4-11)
- Platelet count 40 ×109/L (150-400)
What could be the possible cause of his pancytopenia?Your Answer: Methotrexate
Explanation:Pancytopenia in a Patient with Erosive Rheumatoid Arthritis
This patient is showing signs of pancytopenia, a condition where there is a decrease in all three blood cell types (red blood cells, white blood cells, and platelets). Given her history of erosive rheumatoid arthritis for the past three years, it is likely that she has been on immunosuppressive therapy, which can lead to this type of blood disorder.
Immunosuppressive drugs such as methotrexate, sulfasalazine, penicillamine, and gold can all have an impact on blood cell production and lead to pancytopenia. It is important to monitor patients on these medications for any signs of blood disorders and adjust treatment accordingly. Early detection and management can prevent further complications and improve patient outcomes.
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This question is part of the following fields:
- Haematology
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Question 7
Incorrect
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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: No aspirin, flight stockings, ankle exercises, aisle seat
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.
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This question is part of the following fields:
- Haematology
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Question 8
Correct
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The most common error in transfusion according to the SHOT (serious hazards of transfusion) analysis?
Your Answer: Wrong identification or mislabelling of patient or sample
Explanation:Common Causes of Transfusion Errors
Mislabelling of samples, requests, or misidentifying recipients are the most frequent causes of transfusion errors. This was confirmed by the SHOT study, which examined transfusion errors and near-misses in a nationwide audit in the United Kingdom. Although other errors, such as cross-match errors, incorrect storage, and transfusion reactions due to undetected antibodies, do occur, they are infrequent.
In summary, the SHOT study found that the most common causes of transfusion errors are related to labelling and identification. Therefore, it is crucial to implement strict protocols and procedures to ensure that samples and requests are correctly labelled and recipients are accurately identified to prevent these errors from occurring. While other errors may occur, they are rare and can be mitigated through proper training and adherence to established guidelines.
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This question is part of the following fields:
- Haematology
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Question 9
Correct
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Which autoantibody is correctly matched with its associated disease in the following list?
Your Answer: Pernicious anaemia and anti-intrinsic factor antibodies
Explanation:Autoimmune Disorders and Associated Antibodies
Autoimmune disorders occur when the immune system mistakenly attacks healthy cells and tissues in the body. These disorders are often associated with the presence of specific antibodies that can help diagnose and monitor the disease. Here are some examples:
Pernicious Anaemia and Anti-Intrinsic Factor Antibodies
Pernicious anaemia is a type of anaemia caused by a deficiency in vitamin B12. It is associated with the presence of anti-intrinsic factor antibodies, which bind to intrinsic factor and prevent the absorption of vitamin B12 in the gut.Primary Biliary Cholangitis and Anti-Jo-1 Antibodies
Primary biliary cholangitis is an autoimmune disorder that affects the liver. It is associated with the presence of anti-mitochondrial antibodies, but not anti-Jo-1 antibodies, which are associated with other autoimmune disorders like polymyositis and dermatomyositis.Myasthenia Gravis and Voltage-Gated Calcium Channel Antibodies
Myasthenia gravis is a neuromuscular disorder that causes muscle weakness and fatigue. It is associated with the presence of anti-acetylcholine receptor antibodies, but not anti-striated muscle antibodies, which are found in other autoimmune disorders.Granulomatosis with Polyangiitis (GPA) and Anti-Myeloperoxidase (p-ANCA) Antibody
GPA is a type of vasculitis that affects small and medium-sized blood vessels. It is associated with the presence of cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), but not p-ANCA, which are found in other types of vasculitis.Hashimoto’s Thyroiditis and Thyroid-Stimulating Antibodies
Hashimoto’s thyroiditis is an autoimmune disorder that affects the thyroid gland. It is associated with the presence of anti-thyroglobulin and anti-thyroperoxidase antibodies, which attack the thyroid gland and cause inflammation. -
This question is part of the following fields:
- Haematology
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Question 10
Correct
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A 43-year-old woman was diagnosed with acute myeloid leukaemia (AML) with 71% of bone marrow blasts. She declined bone marrow transplant and was started on appropriate chemotherapy. After 2 months, a repeat bone marrow revealed 8% of blasts. Peripheral blood was blast-free and blood tests revealed:
Investigation Result Normal value
Haemoglobin 106 g/l 115–155 g/l
White cell count (WCC) 8.1 × 109/l 4–11 × 109/l
Neutrophils 5.2 × 109/l 2.5–7.58 × 109/l
Lymphocytes 1.8 × 109/l 1.5–3.5 × 109/l
Platelets 131 × 109/l 150–400 × 109/l
What is her clinical status?Your Answer: Partial remission
Explanation:Partial remission occurs when a patient meets all the criteria for complete remission except for having more than 5% bone marrow blasts. To be diagnosed with partial remission, the blast cells can be between 5% and 25% and must have decreased by at least 50% from their levels before treatment.
Complete remission is achieved when a patient meets specific criteria, including having a neutrophil count of over 1.0 × 109/l and a platelet count of over 100 × 109/l, not requiring red cell transfusions, having normal cellular components on bone marrow biopsy, having less than 5% blasts in the bone marrow without Auer rods present, and having no signs of leukemia anywhere else in the body.
Complete remission with incomplete recovery is when a patient meets all the criteria for complete remission except for continuing to have neutropenia or thrombocytopenia.
Resistant disease occurs when a patient fails to achieve complete or partial remission and still has leukemia cells in their peripheral blood or bone marrow seven days after completing initial therapy.
A morphologic leukemia-free state is when a patient has less than 5% bone marrow blasts without blasts with Auer rods present and no extramedullary disease, but they do not meet the criteria for neutrophils, platelets, and blood transfusions.
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This question is part of the following fields:
- Haematology
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Question 11
Incorrect
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A 6-year-old child of African descent presents with a 2-day history of increasing abdominal pain. It is not associated with nausea or vomiting, and the bowels are opening regularly. The child’s mother tells you that he has had a few episodes of similar symptoms in the past, but none as severe.
On examination, the child has generalised abdominal tenderness, and splenomegaly is noted. After further investigation, the child is diagnosed with sickle-cell anaemia.
Which organ is most responsible for the removal of sickled red blood cells from the circulatory system?Your Answer: Liver
Correct Answer: Spleen
Explanation:The Role of Organs in Immune Surveillance and Blood Production
The human body has several organs that play a crucial role in immune surveillance and blood production. The spleen, for instance, is responsible for removing abnormal and aged red blood cells from circulation and monitoring the blood for immune purposes. However, in sickle-cell anaemia patients, the spleen can become non-functional due to continuous hypoxic and thrombotic insults, leading to a process called autosplenectomy.
Lymph nodes, on the other hand, are involved in immunological surveillance of the lymph. They can swell in response to severe bacterial infections in specific body parts, such as the axillary lymph nodes in the case of a hand infection.
The thymus is responsible for programming pre-T cells to differentiate into T cells, which are responsible for the cellular immune response against pathogenic viruses and fungi and the destruction of malignant cells. It is most active during neonatal and pre-adolescent life.
The bone marrow is responsible for erythropoiesis, the production of red blood cells. In sickle-cell anaemia patients, erythropoiesis in the bone marrow is stimulated.
Finally, the liver can become a site of extramedullary erythropoiesis, which means it can produce red blood cells outside of the bone marrow.
Overall, these organs work together to maintain a healthy immune system and blood production in the body.
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This question is part of the following fields:
- Haematology
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Question 12
Incorrect
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A 50-year-old patient presents to the general practitioner with a complaint of darkening of urine, particularly noticeable in the morning. The patient has no family history of bleeding disorders and was recently hospitalized for deep venous thrombosis in the right leg. Upon examination, the patient's heart and lungs appear normal. Blood tests reveal anemia, elevated levels of lactate dehydrogenase (LDH), high bilirubin levels, and a high reticulocyte count. What is the most likely cause of this patient's condition?
Your Answer: Immunoglobulin M (IgM) antibody against red blood cells
Correct Answer: Phosphatidylinositol glycan A defect in red blood cells
Explanation:Understanding Different Causes of Haemolytic Anaemia
Haemolytic anaemia is a condition where red blood cells are destroyed faster than they can be produced, leading to a shortage of oxygen-carrying cells in the body. There are various causes of haemolytic anaemia, including phosphatidylinositol glycan A defect, vitamin B12 deficiency, glucose-6-phosphate dehydrogenase deficiency, loss of spectrin in the red blood cell membrane, and immunoglobulin M (IgM) antibody against red blood cells.
Phosphatidylinositol glycan A defect, also known as nocturnal haemoglobinuria, is an acquired condition caused by a mutation in the gene encoding for phosphatidylinositol glycan A. This leads to an increased susceptibility of red blood cells to complement proteins in an acidotic environment, resulting in haemolysis. Patients typically present with haematuria in the morning, and treatment involves managing symptoms and using medication such as eculizumab.
Vitamin B12 deficiency causes megaloblastic anaemia and is not related to haemolysis. Glucose-6-phosphate dehydrogenase deficiency is an inherited X-linked recessive condition that results in red blood cell breakdown. Loss of spectrin in the red blood cell membrane is seen in hereditary spherocytosis, where red blood cells become spherical and are trapped in the spleen, leading to haemolysis. IgM antibody against red blood cells causes autoimmune haemolytic anaemia, where the antibody binds to the I antigen on the membrane of red blood cells, leading to haemolysis at low temperatures.
Understanding the different causes of haemolytic anaemia is crucial for proper diagnosis and treatment.
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This question is part of the following fields:
- Haematology
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Question 13
Incorrect
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A 65-year-old man comes to the clinic with complaints of haematuria. He has a history of chronic atrial fibrillation and is currently taking warfarin. His blood tests reveal a Hb level of 112g/L and an INR of 9, but he is stable hemodynamically. The consulting physician recommends reversing the effects of warfarin. What blood product/s would be the most appropriate choice for this patient?
Your Answer: Recombinant factor VII
Correct Answer: Prothrombin concentrate ('Octaplex')
Explanation:Treatment Options for Warfarin Reversal
Prothrombin concentrates are the preferred treatment for reversing the effects of warfarin in cases of active bleeding and a significantly elevated INR. While packed cells are important for managing severe bleeding, they are not the recommended treatment for warfarin reversal. Cryoprecipitate, recombinant factor VII, and platelets are also not indicated for reversing the effects of warfarin. It is important to choose the appropriate treatment option based on the patient’s individual needs and medical history. Proper management of warfarin reversal can help prevent further complications and improve patient outcomes.
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This question is part of the following fields:
- Haematology
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Question 14
Correct
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A 35-year-old woman visits her GP complaining of fatigue that has lasted for 2 months. She has been consuming approximately 20 units of alcohol per week for the past decade. Her blood test reveals the following results:
Investigation Result Normal value
Haemoglobin (Hb) 98 g/l 115–155 g/l
Mean corpuscular volume (MCV) 126 fl 82–100 fl
What is the most probable cause of her anaemia based on these blood results?Your Answer: Alcohol excess
Explanation:Causes of Macrocytic and Microcytic Anaemia
Anaemia is a condition characterized by a decrease in the number of red blood cells or haemoglobin in the blood. Macrocytic anaemia is a type of anaemia where the red blood cells are larger than normal, while microcytic anaemia is a type where the red blood cells are smaller than normal. Here are some of the causes of macrocytic and microcytic anaemia:
Alcohol Excess: Alcohol toxicity can directly affect the bone marrow, leading to macrocytic anaemia. Additionally, alcoholism can cause poor nutrition and vitamin B12 deficiency, which can also lead to macrocytosis.
Congenital Sideroblastic Anaemia: This is a rare genetic disorder that produces ringed sideroblasts instead of normal erythrocytes, leading to microcytic anaemia.
Iron Deficiency: Iron deficiency is a common cause of anaemia, especially in women. However, it causes microcytic anaemia, not macrocytic anaemia.
Blood Loss from Menses: Chronic blood loss due to menorrhagia can result in microcytic iron deficiency anaemia. However, this is a physiological process and would not cause macrocytic anaemia.
Thalassemia: Thalassaemia is a genetic disorder that leads to abnormal or low haemoglobin, resulting in microcytic anaemia.
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This question is part of the following fields:
- Haematology
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Question 15
Incorrect
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What is the appropriate management for a 65-year-old woman with confusion, profuse sweating, bluish discoloration of toes and fingertips, and a petechial rash on the left side of her anterior abdominal wall, who has a history of dysuria and was prescribed antibiotics by her GP three days ago, and is now found to have disseminated intravascular coagulation (DIC) based on her blood results?
Your Answer: Prophylactic dose unfractionated heparin, human activated protein C, urine output monitoring, 500 ml 0.9% saline stat, serum lactate measurement, blood and urine cultures, empirical IV antibiotics and titration of oxygen to ≥94%
Correct Answer: Blood cross-match, urine output monitoring, 500 ml 0.9% saline stat, serum lactate measurement, blood and urine cultures, empirical IV antibiotics and titration of oxygen to ≥94%
Explanation:Managing DIC in a Patient with Septic Shock: Evaluating Treatment Options
When managing a patient with disseminated intravascular coagulation (DIC), it is important to consider the underlying condition causing the DIC. In the case of a patient with septic shock secondary to a urinary tract infection, the sepsis 6 protocol should be initiated alongside pre-emptive management for potential blood loss.
While a blood cross-match is sensible, emergency blood products such as platelets are unwarranted in the absence of acute bleeding. Activated protein C, previously recommended for DIC management, has been removed from guidelines due to increased bleeding risk without overall mortality benefit.
Anticoagulation with low molecular weight heparin is unnecessary at this time, especially when given with blood products, which are pro-coagulant. Tranexamic acid and platelet transfusions are only warranted in the presence of severe active bleeding.
Prophylactic dose unfractionated heparin may be a good management strategy in the presence of both thrombotic complications and increased bleeding risk, but should be given at a treatment dose if deemed necessary. Ultimately, managing the underlying septic shock is the best way to manage DIC in this patient.
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This question is part of the following fields:
- Haematology
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Question 16
Correct
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A 15-year-old girl presents to the clinic with her mother, reporting an increase in abdominal size and no menstrual periods for the past three months. Despite denying any sexual activity, you suspect she may be pregnant. What is the specific measurement of a urinary pregnancy test?
Your Answer: Beta-HCG
Explanation:The Role of Hormone Assays in Confirming Pregnancy
Beta-HCG is a hormone produced by the placenta during pregnancy. There are highly sensitive assays available to detect the presence of beta-HCG, which can confirm pregnancy. In fact, some manufacturers of pregnancy tests claim that their tests are more accurate than ultrasound dating in determining gestation during the early stages of pregnancy.
While alpha-fetoprotein may also be elevated in pregnancy, particularly in cases of neural tube defects, it is not the primary focus of pregnancy testing. Hormone assays for oestrogen, progesterone, or testosterone levels are not reliable methods for confirming pregnancy. Therefore, beta-HCG remains the most reliable hormone to test for when confirming pregnancy.
It is important to note that while hormone assays can confirm pregnancy, they cannot determine the viability of the pregnancy or the presence of any complications. Ultrasound imaging and other diagnostic tests may be necessary to assess the health of the pregnancy and the developing fetus.
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This question is part of the following fields:
- Haematology
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Question 17
Correct
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A 72-year-old man experiences sudden paralysis on the right side of his body. He has been a smoker for 30 years, consuming 15 cigarettes a day. His vital signs are as follows: temperature 37.2°C, pulse 80/minute, respiratory rate 18/minute, and blood pressure 150/95 mm Hg. An angiogram of the brain shows blockage in a branch of the middle cerebral artery. Laboratory results indicate a haemoglobin A1c level of 80 mmol/mol (9.5%). Which component of blood lipids is the most significant factor in contributing to his condition?
Your Answer: Oxidised low-density lipoprotein (LDL)
Explanation:The patient had a stroke likely caused by cerebral atherosclerosis or embolic disease from the heart due to ischaemic heart disease from atherosclerosis. LDL brings cholesterol to arterial walls, and when there is increased LDL or hypertension, smoking, and diabetes, there is more degradation of LDL to oxidised LDL which is taken up into arterial walls via scavenger receptors in macrophages to help form atheromas. Chylomicrons transport exogenous products and are formed in intestinal epithelial cells. HDL particles remove cholesterol from the circulation and transport it back to the liver for excretion or re-utilisation. Lipoprotein lipase hydrolyses triglycerides in lipoproteins and promotes cellular uptake of chylomicron remnants, lipoproteins, and free fatty acids. VLDL transports endogenous triglycerides, phospholipids, and cholesterol and cholesteryl esters.
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This question is part of the following fields:
- Haematology
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Question 18
Correct
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What are the criteria that must be met for blood used in an exchange transfusion for a neonate?
Your Answer: Plasma reduced whole blood in CPD less than 5-days-old and irradiated
Explanation:Requirements for Exchange Transfusion
Exchange transfusion is a medical procedure that necessitates the use of blood that has been processed to meet specific criteria. The blood used must be plasma-reduced whole blood, irradiated, and less than five days old. These requirements are necessary to ensure that the blood is free from any contaminants that could cause adverse reactions in the patient. Additionally, the Rh group of the blood used must either be Rh negative or identical to the neonate to prevent haemolytic transfusion reactions. These precautions are taken to ensure the safety and well-being of the patient undergoing the exchange transfusion. Proper blood selection is crucial in ensuring the success of the procedure and minimizing the risk of complications.
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This question is part of the following fields:
- Haematology
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Question 19
Incorrect
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A 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: Monoclonal gammopathy of unknown significance (MGUS)
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.
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This question is part of the following fields:
- Haematology
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Question 20
Correct
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A 29-year-old female patient presents to the clinic with a complaint of missed periods for the past four months despite negative pregnancy tests. She is also distressed about the loss of her libido and has noticed milk leakage with minimal nipple stimulation during intercourse. On physical examination, her blood pressure is 122/70 mmHg, pulse is 70 and regular, and general physical examination is unremarkable. Which blood test is most likely to show elevated levels?
Your Answer: Prolactin
Explanation:Symptoms and Diagnosis of Hyperprolactinaemia
Hyperprolactinaemia is a condition characterized by elevated levels of prolactin in the body. This condition is typically associated with symptoms such as milk production, decreased libido, and absence of menstruation. However, visual disturbances are not always present, as many cases of hyperprolactinaemia are related to a microprolactinoma.
When diagnosing hyperprolactinaemia, it is important to assess thyroid status as this condition is often associated with hypothyroidism. Thyroxine levels are usually low in individuals with hyperprolactinaemia. Additionally, beta-HCG levels are elevated in pregnancy, so it is important to rule out pregnancy as a potential cause of elevated prolactin levels.
In summary, hyperprolactinaemia is a condition that can present with a variety of symptoms, but is typically characterized by elevated prolactin levels. Diagnosis involves assessing thyroid status and ruling out pregnancy as a potential cause.
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This question is part of the following fields:
- Haematology
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Question 21
Correct
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A 28-year-old woman presents to her general practitioner after suffering from several miscarriages and is afraid her husband will leave her. The patient gave the history of bruising even with minor injuries and several spontaneous miscarriages. On examination, the patient is noted to have a rash in a butterfly distribution on the nose and cheeks. Tests reveal 1+ proteinuria only.
What is the most likely cause of her main concern?Your Answer: Antiphospholipid syndrome (APLS)
Explanation:Understanding Antiphospholipid Syndrome (APLS) and its Link to Recurrent Spontaneous Abortions
When a young woman experiences multiple spontaneous abortions, it may indicate an underlying disorder. One possible cause is antiphospholipid syndrome (APLS), a hypercoagulable state with autoantibodies against phospholipid components. This disorder can lead to recurrent spontaneous abortions during the first 20 weeks of pregnancy, and approximately 9% of APLS patients also have renal abnormalities.
Other potential causes of recurrent spontaneous abortions include poorly controlled diabetes, nephritic syndrome, dermatomyositis, and anatomic defects like a bicornuate uterus. However, the examination and test results in this case suggest a systemic etiology, making APLS a strong possibility.
Diagnosing systemic lupus erythematosus (SLE), which can also cause nephritic or nephrotic syndrome, requires meeting at least 4 out of 11 criteria established by the American Rheumatism Association (ARA).
Understanding these potential causes and their links to recurrent spontaneous abortions can help healthcare providers identify and treat underlying disorders in women of reproductive age.
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This question is part of the following fields:
- Haematology
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Question 22
Incorrect
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A 35-year-old 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: Buerger's disease
Correct 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.
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This question is part of the following fields:
- Haematology
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Question 23
Correct
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A 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: bcl-2
Explanation:Follicular lymphoma is a prevalent type of non-Hodgkin’s lymphoma that results from a chromosomal translocation between chromosome 14 and chromosome 18. This translocation causes the bcl-2 protein, which is anti-apoptotic, to be moved to the IgH promoter region, leading to overproduction of bcl-2. Consequently, abnormal B cells undergo clonal proliferation and are protected from apoptosis. Follicular lymphoma affects both genders equally, and its incidence increases with age. The disease typically presents with painless adenopathy that progresses over time. Systemic symptoms, such as fevers, night sweats, and weight loss, may occur later in the disease progression and can be associated with anaemia, thrombocytopenia, and lymphocytosis. Diagnosis requires a lymph node biopsy to demonstrate the expansion of follicles filling the node and chromosomal analysis of cells from bone marrow aspiration to detect t(14:18). Chemotherapy is the primary treatment, and rituximab, a monoclonal antibody against the CD20 protein found on B cells, is often used in combination with other agents. In Burkitt’s lymphoma, c-myc overexpression is caused by a translocation between chromosomes 8 and 14, while chronic myeloid leukaemia results from a translocation between chromosomes 9 and 22, forming the Philadelphia chromosome. Ewing’s sarcoma is caused by a translocation between chromosomes 11 and 12, leading to the formation of the fusion protein Ewsr1-fli1, which causes aberrant transcription of genes regulating cell growth and development. In a small subset of follicular lymphoma patients, translocations involving the bcl-6 gene and protein are found, which may increase the risk of transformation to a more aggressive form.
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This question is part of the following fields:
- Haematology
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Question 24
Incorrect
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A 48-year-old man was admitted with unconsciousness.
On examination, his left plantar response was extensor, and deep tendon jerks were increased on the left side. A computerised tomography (CT) scan of the brain revealed a right-middle cerebral artery territory infarct. He was not known to have diabetes or hypertension. He was not receiving any drugs, either.
His blood count revealed:
Investigation Result Normal value
Haemoglobin 110 g/l 135–175 g/l
White cell count (WCC) 331 × 109/l 4–11 × 109/l
Neutrophil count 145 × 109/l 2.5–7.58 × 109/l
Metamyelocyte 3000/mm3
Platelet 490 × 109/l 150–400 × 109/l
Peripheral smear Many band forms, myelocytes, basophils
What is the next appropriate therapy?Your Answer: Aspirin
Correct Answer: Leukapheresis
Explanation:Leukapheresis and Other Treatment Options for Chronic Myeloid Leukaemia with High White Blood Cell Count and Ischaemic Stroke
Chronic myeloid leukaemia can cause an extremely high white blood cell count, leading to hyperviscosity of the blood and an increased risk of ischaemic events such as stroke. While anticoagulation medications are important, they do not address the underlying issue of the high cell count. Leukapheresis is a procedure that can reduce the white cell volume by 30-60%, making it a crucial emergency treatment option. Other treatments, such as hydroxyurea and imatinib, can also be used to control disease burden. Imatinib is a tyrosine kinase inhibitor that is effective in treating chronic myeloid leukaemia with the Philadelphia chromosome translocation. Aspirin and heparin have limited roles in this scenario. While aspirin is recommended for long-term therapy after an ischaemic stroke, it does not address the hypercoagulable state caused by the high white blood cell count. Heparin is not used in the treatment of ischaemic strokes. Overall, leukapheresis should be the first step in emergency management for chronic myeloid leukaemia with a high white blood cell count and ischaemic stroke.
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This question is part of the following fields:
- Haematology
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Question 25
Correct
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A 70-year-old man is referred to the medical team on call. He has been feeling fatigued for two months and is now experiencing shortness of breath with minimal exertion. He has also had several episodes of syncope with postural hypotension. The GP conducted a blood count and the results showed:
- Haemoglobin 64 g/L (120-160)
- MCV 62 fL (80-96)
- WCC 11.6 ×109L (4-11)
- Platelets 170 ×109L (150-400)
- MCH 22 pg (28-32)
What is the most appropriate next step?Your Answer: Transfuse packed red cells
Explanation:Microcytic Hypochromic Anaemia and the Importance of Blood Transfusion
This patient is presenting with a microcytic hypochromic anaemia, which is commonly caused by iron deficiency due to occult gastrointestinal (GI) blood loss in a Caucasian population. To determine the cause of the anaemia, a full history and examination should be conducted to look for clues of GI blood loss. Given the microcytic hypochromic picture, it is likely that blood loss has been ongoing for some time.
Although there is no evidence of haemodynamic compromise or congestive cardiac failure (CCF), the patient is experiencing breathlessness on minimal exertion. This justifies an upfront transfusion to prevent the patient from going into obvious cardiorespiratory failure. At a Hb of 64 g/L in a 72-year-old, the benefits of transfusion outweigh the risks.
While haematinics such as ferritin, vitamin B12, and folate are important investigations, the most crucial management step is organising a blood transfusion. This will help to address the immediate issue of anaemia and prevent further complications.
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This question is part of the following fields:
- Haematology
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Question 26
Correct
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A 19-year-old Afro-Caribbean male with sickle cell disease complains of right upper abdominal pain and exhibits tenderness upon palpation. What diagnostic test should be performed to confirm the diagnosis?
Your Answer: Abdominal ultrasound scan
Explanation:Pigment Gallstones and High Haem Turnover
In cases of chronic haemolysis, such as sickle cell disease or thalassaemia, the presence of unconjugated bilirubin in bile can lead to the formation of pigment gallstones. These stones are black in color and are caused by the precipitation of calcium bilirubinate from solution. The high concentration of unconjugated bilirubin in bile is a result of the increased turnover of haemoglobin. This can cause pain and discomfort for the patient. It is important to manage the underlying condition causing the high haem turnover to prevent the formation of pigment gallstones.
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This question is part of the following fields:
- Haematology
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Question 27
Incorrect
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A 75-year-old woman comes in with a swollen and hot right calf. She had undergone a right knee arthroscopy and had been immobile for a while. She only takes paracetamol and codeine for osteoarthritis as needed. An above-knee deep vein thrombosis (DVT) is detected on ultrasound, which is her first DVT. What is the recommended treatment?
Your Answer: Immediate thrombolysis
Correct Answer: Three months of apixaban
Explanation:Management of Deep Vein Thrombosis and Pulmonary Embolism
Apixaban and rivaroxaban are the preferred medications for treating deep vein thrombosis (DVT), except for patients with renal impairment or antiphospholipid syndrome. Low molecular weight heparin (LMWH) and warfarin are alternative options for those who cannot take apixaban or rivaroxaban. Thrombolysis is used to manage pulmonary embolism (PE) in patients with haemodynamic instability.
The duration of anticoagulation treatment depends on the type of DVT. For provoked DVTs, which have an identifiable cause, treatment is recommended for at least three months. After this period, the risks and benefits of continuing anticoagulation treatment must be assessed to determine further treatment. For unprovoked DVTs, which have no identifiable cause, treatment is recommended for at least six months. After this period, a risk and benefit assessment is required to determine further treatment.
NICE has provided a helpful visual summary to assist in the management of DVT and PE.
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This question is part of the following fields:
- Haematology
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Question 28
Incorrect
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Which statement about rhesus antibodies in pregnancy is correct?
Your Answer: D positive women are less likely than D negative women to form antibodies to other red cell antigens (such as Kell and Duffy)
Correct Answer: Following delivery, the degree of fetomaternal haemorrhage should be calculated on a blood sample from a D negative mother
Explanation:Important Points to Remember about Fetomaternal Haemorrhage
Following the delivery of a baby, it is crucial to determine the degree of fetomaternal haemorrhage (FMH) in a D negative mother. This is done by analyzing a blood sample to adjust the dose of anti-D in the mother if she has delivered a D positive child. It is important to note that D positive and D negative women have the same likelihood of developing antibodies to other red cell antigens. Therefore, all pregnant women should undergo a blood group and antibody screen in their first trimester or at the time of presentation, whichever comes first. The fetal Rh type is determined by the Rh typing of both the mother and father. Additionally, maternal antibody titres are indicative of the degree of haemolytic disease of the newborn (HDN). For more information on the management of women with red cell antibodies during pregnancy, refer to the Royal College of Obstetricians and Gynaecologists (RCOG) Green-top Guideline No. 65.
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This question is part of the following fields:
- Haematology
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Question 29
Correct
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A 30-year-old donor experiences a sudden fainting spell during blood donation. What should be the immediate course of action to manage this adverse event?
Your Answer: Temporarily cease the donation, consider fluid replacement and elevate the donor's legs
Explanation:Donor safety is important in blood donation. Fainting is a common adverse event and should be treated by stopping the donation and reviving the donor. Elevation of the legs and monitoring vitals is necessary. Donors should be counseled on pre-donation expectations and encouraged to drink fluids after recovery. Hemoglobin checks are no longer required.
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This question is part of the following fields:
- Haematology
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Question 30
Incorrect
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A 50-year-old woman presents with headaches and nosebleeds and is found to have a raised platelet count. She is diagnosed with essential thrombocytosis by the haematologist.
Which of the following might be used to treat essential thrombocytosis?Your Answer: Interferon β
Correct Answer: Hydroxyurea
Explanation:Common Medications and Their Uses
Thrombocytosis and Hydroxyurea
Thrombocytosis is a condition characterized by an elevated platelet count, which can lead to bleeding or thrombosis. Primary or essential thrombocytosis is a myeloproliferative disorder that results in overproduction of platelets by the bone marrow. Hydroxyurea is the first-line treatment for essential thrombocytosis, as it inhibits an enzyme involved in DNA synthesis and reduces the rate of platelet production.Interferon Gamma for Immunomodulation
Interferon gamma is an immunomodulatory medication used to reduce the frequency of infections in patients with chronic granulomatous disease and severe malignant osteopetrosis. It is administered by subcutaneous injection.Cromoglycate for Inflammation
Sodium cromoglycate is a synthetic non-steroidal anti-inflammatory drug used in the treatment of asthma, allergic rhinitis, and various food allergies.Interferon β for Multiple Sclerosis
Interferon β is a cytokine used in the treatment of relapsing-remitting multiple sclerosis. It is administered subcutaneously.Ranitidine for Acid Reduction
Ranitidine is a H2 (histamine) receptor blocker that inhibits the production of acid in the stomach. It can be used in the treatment of gastro-oesophageal reflux disease, peptic ulcer disease, and gastritis. -
This question is part of the following fields:
- Haematology
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