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Question 1
Correct
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A middle-aged patient is newly diagnosed with a bleeding disorder after complaining of heavy periods. She presents with a prolonged APTT and bleeding time, but normal indices. The patient reports a family history of bleeding disorders on her mother's side. She denies any prior history of bleeding or bruising after minor surgical procedures. What is the most likely bleeding disorder in this patient?
Your Answer: von Willebrand’s disease
Explanation:The diagnosis in this scenario is von Willebrand’s disease, which is the most common hereditary bleeding disorder caused by a defective von Willebrand factor. This protein plays a crucial role in haemostasis by assisting in platelet adhesion and stabilising coagulation factor VIII. A deficiency in von Willebrand factor prolongs bleeding time and APTT, but does not affect platelet counts or PT. It is more pronounced in women and may present with menorrhagia. Treatment involves administration of recombinant von Willebrand factor. Haemophilia A, Bernard-Soulier syndrome, Glanzmann’s thrombasthenia, and vitamin K deficiency are other bleeding disorders with different causes and blood test results.
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This question is part of the following fields:
- Haematology
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Question 2
Correct
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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: Hydroxyurea
Explanation:Common Medications and Their Uses
Thrombocytosis and Hydroxyurea
Thrombocytosis is a condition characterized by an elevated platelet count, which can lead to bleeding or thrombosis. Primary or essential thrombocytosis is a myeloproliferative disorder that results in overproduction of platelets by the bone marrow. Hydroxyurea is the first-line treatment for essential thrombocytosis, as it inhibits an enzyme involved in DNA synthesis and reduces the rate of platelet production.Interferon Gamma for Immunomodulation
Interferon gamma is an immunomodulatory medication used to reduce the frequency of infections in patients with chronic granulomatous disease and severe malignant osteopetrosis. It is administered by subcutaneous injection.Cromoglycate for Inflammation
Sodium cromoglycate is a synthetic non-steroidal anti-inflammatory drug used in the treatment of asthma, allergic rhinitis, and various food allergies.Interferon β for Multiple Sclerosis
Interferon β is a cytokine used in the treatment of relapsing-remitting multiple sclerosis. It is administered subcutaneously.Ranitidine for Acid Reduction
Ranitidine is a H2 (histamine) receptor blocker that inhibits the production of acid in the stomach. It can be used in the treatment of gastro-oesophageal reflux disease, peptic ulcer disease, and gastritis. -
This question is part of the following fields:
- Haematology
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Question 3
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 4
Correct
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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: 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.
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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 68-year-old man who has been diagnosed with severe Gram-positive sepsis is admitted to the Intensive Care Unit (ICU). However, he is starting to deteriorate despite being on the Sepsis Six Bundle. He is pyrexial and appears very unwell. On examination:
Investigation Results Normal value
Respiratory rate (RR) 30 breaths/minute 12–18 breaths/minute
Heart rate (HR) 120 bpm 60–100 bpm
Blood pressure (BP) 88/40 mmHg < 120/80 mmHg
You noted some bleeding along the cannulation site and on his gums. The coagulation profile showed prolonged prothrombin time, a decrease in fibrinogen level and marked elevation of D-dimer. He has some purpuric rash on his extremities.
Which of the following is the most likely cause of the above condition?Your Answer: Disseminated intravascular coagulopathy (DIC)
Explanation:Comparison of DIC, von Willebrand’s Disease, Liver Failure, Haemophilia, and Heparin Administration
Disseminated intravascular coagulopathy (DIC) is a serious complication of severe sepsis that can lead to multiorgan failure and widespread bleeding. It is characterized by high prothrombin time and the use of fibrinogen for widespread clot formation, resulting in high levels of D-dimer due to intense fibrinolytic activity. DIC is a paradoxical state in which the patient is prone to clotting but also to bleeding.
Von Willebrand’s disease is an inherited disorder of coagulation that is usually autosomal dominant. There is insufficient information to suggest that the patient in this case has von Willebrand’s disease.
Liver failure could result in excessive bleeding due to disruption of liver synthetic function, but there is no other information to support liver failure in this case. Signs of hepatic encephalopathy or jaundice would also be expected.
Haemophilia is an X-linked recessive disorder of coagulation that is characterized by prolonged activated partial thromboplastin time (APTT) and normal prothrombin time.
There is no information to suggest that heparin has been administered, and the bleeding time and platelet count would be normal.
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This question is part of the following fields:
- Haematology
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Question 6
Correct
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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: 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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This question is part of the following fields:
- Haematology
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Question 7
Incorrect
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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: Side effect of NSAIDs
Correct 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 8
Correct
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A 58-year-old male presents with macrocytic anaemia and a megaloblastic bone marrow. What is the most probable cause of his macrocytosis?
Your Answer: Folate deficiency
Explanation:Megaloblastic Bone Marrow and Its Causes
A megaloblastic bone marrow is a condition that occurs due to a deficiency in vitamin B12 or folate, as well as some cytotoxic drugs. This condition is characterized by the presence of large, immature red blood cells in the bone marrow. However, other causes of macrocytosis, which is the presence of abnormally large red blood cells in the bloodstream, do not result in a megaloblastic bone marrow appearance. It is important to identify the underlying cause of macrocytosis to determine the appropriate treatment.
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This question is part of the following fields:
- Haematology
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Question 9
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: Six months warfarin with concurrent heparin use for at least five days and until INR >2 for at least 24 hours
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 10
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: Multiple myeloma
Correct Answer: Smouldering myeloma
Explanation:Smouldering myeloma is a stage between monoclonal gammopathy of unknown significance (MGUS) and myeloma. To diagnose this condition, the patient must have a monoclonal protein in the serum of at least 30 g/l and monoclonal plasma cells of at least 10% in bone marrow or tissue biopsy, but no evidence of end-organ damage. Patients with smouldering myeloma should be closely monitored as they are at high risk of developing symptomatic myeloma.
Multiple myeloma is a malignant neoplasm where there is clonal proliferation of plasma cells in the bone marrow, leading to the secretion of a monoclonal antibody and light immunoglobulin chains that cause organ damage. Patients with multiple myeloma present with various symptoms, including lethargy, bone pain, pathological fractures, renal impairment, amyloidosis, and pancytopenia due to marrow infiltration. To diagnose multiple myeloma, the patient must have a monoclonal antibody in serum and/or urine, clonal plasma cells of at least 10% on bone marrow biopsy, and evidence of end-organ damage.
MGUS is a condition where low levels of paraprotein are detected in the blood, but they are not causing clinically significant symptoms or end-organ damage. To diagnose MGUS, the patient must have a monoclonal protein in the serum of less than or equal to 30 g/l, monoclonal plasma cells of less than or equal to 10% in bone marrow or tissue biopsy, and no evidence of end-organ damage.
Non-secretory myeloma is a rare variant of multiple myeloma where the bone marrow findings and end-organ damage are similar to myeloma, but there is no detectable monoclonal protein in the serum or urine. This makes it difficult to diagnose.
Plasma cell leukemia is a rare and aggressive form of multiple myeloma characterized by high levels of plasma cells circulating in the peripheral blood. It can occur as a primary condition or a secondary leukaemic transformation of multiple myeloma.
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This question is part of the following fields:
- Haematology
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Question 11
Correct
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A 70-year-old man presents with melaena and an INR of 8. He is currently taking warfarin for atrial fibrillation, as well as antihypertensive medication and cholesterol-lowering agents. He recently received antibiotics from his GP for a cough. Which medication is the likely culprit for his elevated INR?
Your Answer: Erythromycin
Explanation:Medications that Interfere with Warfarin and Increase INR
Certain medications can affect the duration of warfarin’s effects in the body by interfering with the cytochrome P450 enzyme system in the liver. This can cause the INR to increase or decrease rapidly, making patients who are on a stable warfarin regimen vulnerable. To remember the drugs that inhibit cytochrome P450 and increase the effects of warfarin, the mnemonic O-DEVICES can be helpful.
Omeprazole, disulfiram, erythromycin, valproate, isoniazide, cimetidine and ciprofloxacin, ethanol (acutely), and sulphonamides are the drugs that can interfere with warfarin’s effects. These drugs can increase the INR, which can lead to bleeding complications. Therefore, it is important for healthcare providers to monitor patients who are taking warfarin and any of these medications closely to ensure that their INR remains within the therapeutic range. Patients should also inform their healthcare providers of any new medications they are taking to avoid potential interactions with warfarin.
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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 plasma donor on a continuous flow separator machine experiences light headedness, muscle cramps, and circumoral paraesthesia. What should be the next course of action in managing this patient?
Your Answer: Investigate for sepsis due to infection
Correct Answer: Investigate and treat citrate toxicity
Explanation:Citrate Toxicity and Hypocalcaemia in Apheresis Patients
This patient is experiencing symptoms of citrate toxicity, which has led to hypocalcaemia. While it is possible for haemorrhage to occur at the site of venepuncture or venous access, this is typically easy to identify through clinical examination. Sepsis is an uncommon occurrence if proper aseptic precautions have been taken, and the symptoms described here are not indicative of an infection. Immediate treatment is necessary, and this can be achieved by slowing or stopping the apheresis process. Treatment options include the administration of oral or intravenous calcium replacement.
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This question is part of the following fields:
- Haematology
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Question 13
Correct
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The risk of contracting a viral infection through a blood transfusion can vary greatly. What is the estimated risk of hepatitis B transmission in the United Kingdom, for instance?
Your Answer: 1 per 1 million donations
Explanation:Infective Risks of Blood Transfusion
Blood transfusions carry the risk of transmitting viral infections such as hepatitis B, hepatitis C, and HIV. The likelihood of infection varies depending on the source of the donation and the type of testing used. In the UK, the risk of contracting hepatitis B from a blood transfusion is approximately 1 in 1.3 million donations. The risks for HIV and hepatitis C are even lower, at 1 in 6.5 million and 1 in 28 million donations, respectively. It is important for healthcare professionals to have a comprehensive of these risks when obtaining consent from patients for blood transfusions. Adequate knowledge and communication can help patients make informed decisions about their healthcare.
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This question is part of the following fields:
- Haematology
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Question 14
Correct
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A 14-year-old girl presents to the clinic with her parents. She is worried about not having started her periods yet, while many of her peers have. Her medical history includes a lack of sense of smell, which she has had since childhood. On examination, she has normal height, early breast development, and minimal secondary sexual hair. Her BMI is 22. What blood test would be most helpful in determining the underlying cause of her amenorrhea?
Your Answer: FSH
Explanation:Kallmann’s Syndrome and its Differential Diagnosis
Anosmia and primary amenorrhoea are two symptoms that may indicate the presence of Kallmann’s syndrome. This condition is characterized by the underdevelopment of the olfactory bulb, which leads to a loss of the sense of smell, and the failure to produce gonadotrophin releasing hormone. As a result, low levels of follicle-stimulating hormone and luteinising hormone may cause a partial or complete failure to enter puberty in women.
Congenital adrenal hyperplasia, on the other hand, may cause electrolyte imbalances, but it is typically associated with abnormal female virilization. Prolactinoma, a type of pituitary tumor, is usually linked to secondary amenorrhoea. Meanwhile, thyrotoxicosis, a condition characterized by an overactive thyroid gland, may cause menstrual cessation, but it is less likely to be the cause of primary amenorrhoea, especially in the absence of hyperthyroidism symptoms.
In summary, Kallmann’s syndrome should be considered as a possible diagnosis in patients presenting with anosmia and primary amenorrhoea. However, other conditions such as congenital adrenal hyperplasia, prolactinoma, and thyrotoxicosis should also be ruled out through proper evaluation and testing.
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This question is part of the following fields:
- Haematology
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Question 15
Incorrect
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A 67-year-old man was admitted with pain in the left groin. He had fallen from his chair, after which the pain started. He was unable to walk and his left leg was externally rotated. X-rays revealed a neck of femur fracture. He lives alone in a flat and is a vegetarian. His past medical history includes shortness of breath, body itching and occasional swelling of legs. His blood tests revealed:
Investigation Result Normal range
Haemoglobin 120 g/l 135–175 g/l
Calcium 3.5 mmol/l 2.20–2.60 mmol/l
Phosphate 1.52 mmol/l 0.70–1.40 mmol/l
Erythrocyte sedimentation rate (ESR) 184 mm/h 0–15 mm/h
What is the most likely diagnosis?Your Answer: Vitamin D deficiency
Correct Answer: Multiple myeloma
Explanation:Diagnosis of Multiple Myeloma in a Patient with Pathological Fracture
A man has sustained a pathological fracture after a minor trauma, which is likely due to lytic bone lesions. He also presents with anemia, raised calcium, and ESR, all of which are consistent with a diagnosis of multiple myeloma. This is further supported by his age group for presentation.
Other possible diagnoses, such as osteoporosis, vitamin D deficiency, acute leukemia, and malignancy with metastasis, are less likely based on the absence of specific symptoms and laboratory findings. For example, in osteoporosis, vitamin D and phosphate levels are normal, and ESR and hemoglobin levels are not affected. In vitamin D deficiency, calcium and phosphate levels are usually normal or low-normal, and ESR is not raised. Acute leukemia typically presents with systemic symptoms and normal serum calcium levels. Malignancy with metastasis is possible but less likely without preceding symptoms suggestive of an underlying solid tumor malignancy.
In summary, the patient’s clinical presentation and laboratory findings suggest a diagnosis of multiple myeloma.
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This question is part of the following fields:
- Haematology
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Question 16
Incorrect
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An 80-year-old man comes to the clinic with painless, symmetrical swellings in his neck. He reports no other symptoms. Laboratory tests show a haemoglobin level of 100 g/l and a white cell count of 23 × 109/l. A blood film reveals smear cells, with more than 60% of the cells being small mature lymphocytes. What is the probable diagnosis?
Your Answer: Hodgkin’s lymphoma
Correct Answer: Chronic lymphocytic leukaemia
Explanation:Differentiating Leukaemia and Lymphoma: Understanding CLL and Other Types
Leukaemia and lymphoma are two types of blood cancers that can present with similar symptoms. However, each type has distinct characteristics that can help differentiate them. Among the different types of leukaemia and lymphoma, B-cell chronic lymphocytic leukaemia (B-CLL) is the most common leukaemia in adults. It is characterized by peripheral blood lymphocytosis and uncontrolled proliferation of B cell lymphocytes in the bone marrow, lymph nodes, and splenomegaly. Patients with CLL are often asymptomatic, and the condition is often picked up incidentally.
In contrast, acute lymphoblastic leukaemia is a common leukaemia of children aged 2–5 years and is very rare in adults. Multiple myeloma, on the other hand, is the uncontrolled proliferation of plasma cells and presents with bone pain, hypercalcaemia, renal failure, and neutropenia. Chronic myeloid leukaemia tends to present with more systemic, B symptoms in a slightly younger age group, and a classic symptom is massive hepatosplenomegaly.
While lymphoma is a possibility in this age group, CLL is the most likely diagnosis as it is more common in this age group and in the western world. Further investigation would be used to confirm the diagnosis. Understanding the characteristics of each type of leukaemia and lymphoma can aid in accurate diagnosis and appropriate treatment.
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This question is part of the following fields:
- Haematology
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Question 17
Incorrect
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What is the correct information regarding the storage requirements and lifespan of blood products?
Your Answer: Packed red cells are stored at 4°C for up to 25 days
Correct Answer: Fresh frozen plasma is stored at −25°C for up to 36 months
Explanation:Storage Guidelines for Blood Products
Blood products such as fresh frozen plasma, red cells, and platelets have specific storage guidelines to ensure their safety and efficacy. Fresh frozen plasma can be stored for up to 36 months at a temperature of −25°C. On the other hand, red cells are stored at a temperature of 4°C for a maximum of 35 days, while platelets are stored at a temperature of 22°C for up to 5 days on a platelet shaker/agitator.
These guidelines are important to follow to maintain the quality of blood products and prevent any adverse reactions in patients who receive them. It is crucial to store blood products at the appropriate temperature and for the recommended duration to ensure their effectiveness when used in transfusions. Healthcare professionals should be aware of these guidelines and ensure that they are followed to provide safe and effective blood transfusions to patients.
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This question is part of the following fields:
- Haematology
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Question 18
Incorrect
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A 35-year-old man presents with progressive weakness in his right upper limb and both lower limbs over the past four months. He has also developed digital infarcts affecting the second and third fingers on the right hand and the fifth finger on the left. On examination, his blood pressure is 160/140 mm Hg, all peripheral pulses are palpable, and there is an asymmetrical neuropathy. Laboratory investigations reveal a haemoglobin level of 120 g/L (130-170), a white cell count of 12.5 ×109/L (4-10), a platelet count of 430 ×109/L (150-450), and an ESR of 50 mm/hr (0-15). Urine examination shows proteinuria and 10-15 red blood cells per high power field without casts. What is the most likely diagnosis?
Your Answer: Systemic lupus erythematosus
Correct Answer: Polyarteritis nodosa
Explanation:Polyarteritis nodosa (PAN) is a systemic disease that affects small or medium-sized arteries in various organs, leading to a wide range of symptoms such as nerve damage, skin issues, joint and muscle pain, kidney problems, and heart issues. Laboratory findings include anemia, increased white blood cells and platelets, and elevated inflammatory markers. ANCA testing can help differentiate PAN from other vasculitis diseases.
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This question is part of the following fields:
- Haematology
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Question 19
Incorrect
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By how much does the haemoglobin concentration increase with the infusion of one unit of fresh blood?
Your Answer: 1 g/L
Correct Answer: 10 g/L
Explanation:The Effect of Fresh Blood on Haemoglobin Levels
When one unit of fresh blood is transfused, it increases the haemoglobin levels in the body by approximately 10 g/L. This is equivalent to the effect of one unit of red cell concentrate. Both fresh blood and red cell concentrate contain red blood cells, which are responsible for carrying oxygen throughout the body. Therefore, the increase in haemoglobin levels is due to the additional red blood cells that are introduced into the bloodstream. This information is important for medical professionals who need to monitor and manage the haemoglobin levels of their patients, particularly those who have undergone significant blood loss or have conditions that affect their red blood cell count.
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This question is part of the following fields:
- Haematology
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Question 20
Correct
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A patient on a medical ward received a transfusion 72 hours ago for symptomatic anaemia on a background of chronic renal disease and obstructive airways disease. He has a history of previous transfusions in the last six months. The patient, who is in his 60s, has now dropped his Hb by 20 g/L compared to his pretransfusion level and reports a dark coloured urine. The LDH and bilirubin are elevated. What is the most likely explanation for these findings?
Your Answer: Delayed haemolytic transfusion reaction
Explanation:Delayed Haemolytic Transfusion Reaction
A delayed haemolytic transfusion reaction can occur 24 hours after a transfusion in patients who have been previously immunised through transfusions or pregnancy. Initially, the antibodies are not detectable, but they become apparent as a secondary immune response to the antigen exposure during the transfusion. In such cases, it is essential to carry out a haemoglobin level, blood film, LDH, direct antiglobulin test, renal profile, serum bilirubin, haptoglobin, and urinalysis for haemoglobinuria. Additionally, the group and antibody screen should be repeated.
It is unlikely that the patient is experiencing a transfusion-associated graft versus host disease or acute hepatitis as both would occur within a week or two. Furthermore, this is not an acute haemolysis that would be expected to occur during the transfusion. The rise in bilirubin and LDH levels indicates a haemolytic reaction. Therefore, it is crucial to monitor the patient’s condition and provide appropriate treatment.
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This question is part of the following fields:
- Haematology
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Question 21
Incorrect
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A 68-year-old woman presents with lethargy and poor exercise tolerance. She also mentions that, over the last month, she has had excessive thirst and polyuria.
Initial investigations show:
Investigation Result Normal value
Haemoglobin (Hb) 78 g/l 115–155 g/l
Erythrocyte sedimentation rate (ESR) 109 mm/h 0–10mm in the first hour
Sodium (Na+) 134 mmol/l 135–145 mmol/l
Potassium (K+) 5.8 mmol/l 3.5–5.0 mmol/l
Urea 26.5 mmol/l 2.5–6.5 mmol/l
Creatinine 268 µmol/l 50–120 μmol/l
Corrected calcium (Ca2+) 3.02 mmol/l 2.20–2.60 mmol/l
Glucose 5.2 mmol/l 3.5–5.5 mmol/l
Which of the following tests is the most appropriate to confirm the underlying diagnosis?Your Answer: Serum PTH level
Correct Answer: Serum and urine electrophoresis
Explanation:Diagnostic Tests and Differential Diagnosis for a Patient with Multiple Derangements
The patient in question presents with several abnormalities in their blood tests, including anaemia, hypercalcaemia, electrolyte imbalances, and a significantly elevated ESR. These findings, along with the patient’s symptoms, suggest a diagnosis of malignancy, specifically multiple myeloma.
Multiple myeloma is characterized by the malignant proliferation of plasma cells, leading to bone marrow infiltration, pancytopenia, osteolytic lesions, hypercalcaemia, and renal failure. The ESR is typically elevated in this condition. To confirm a diagnosis of multiple myeloma, serum and urine electrophoresis can be performed to identify the presence of monoclonal antibodies and Bence Jones proteins, respectively. Bone marrow examination can also reveal an increased number of abnormal plasma cells.
Treatment for multiple myeloma typically involves a combination of chemotherapy and bisphosphonate therapy, with radiation therapy as an option as well. This condition is more common in men, particularly those in their sixth or seventh decade of life.
Other diagnostic tests that may be considered include an oral glucose tolerance test (to rule out diabetes as a cause of polydipsia and polyuria), a chest X-ray (to evaluate for a possible small cell carcinoma of the lung), and an abdominal CT scan (to assess the extent of disease and the presence of metastasis). A serum PTH level may also be useful in ruling out primary hyperparathyroidism as a cause of hypercalcaemia, although the patient’s symptoms and blood test results make malignancy a more likely diagnosis.
Diagnostic Tests and Differential Diagnosis for a Patient with Multiple Derangements
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This question is part of the following fields:
- Haematology
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Question 22
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 23
Incorrect
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A 70-year-old man presents with increasing shortness of breath on exertion. He also notes he has been bruising more easily of late. He is noted to be in sinus tachycardia but otherwise is haemodynamically stable. Examination reveals conjunctival pallor and hepatosplenomegaly. No definite lymphadenopathy is palpable. A full blood count is performed:
Investigation Result Normal value
Haemoglobin 69 g/l 135–175 g/l
White cell count (WCC) 0.7 × 109/l 4.0–11.0 × 109/l
Platelets 14 × 109/l 150–400 × 109/l
Blood film is reported as a leukoerythroblastic picture with teardrop-shaped erythrocytes. A bone marrow aspirate is attempted, but this is unsuccessful.
What is the likely diagnosis?Your Answer: Acute myelocytic leukaemia
Correct Answer: Myelofibrosis
Explanation:Understanding Myelofibrosis: A Comparison with Other Bone Marrow Disorders
Myelofibrosis is a rare disorder that primarily affects older patients. It is characterized by bone marrow failure, which can also be found in other diseases such as advanced prostate cancer, acute lymphoblastic leukemia, acute myelocytic leukemia, and chronic myeloid leukemia. However, myelofibrosis can be distinguished from these other disorders by specific diagnostic clues.
One of the key diagnostic features of myelofibrosis is the presence of a leukoerythroblastic picture with teardrop-shaped red blood cells, which is also seen in advanced prostate cancer. However, in myelofibrosis, a failed bone marrow aspirate, or dry tap, is frequent and a bone marrow trephine biopsy is needed for diagnosis. This is not the case in other bone marrow disorders.
Myelofibrosis is caused by the proliferation of megakaryocytes, which leads to intense bone marrow fibrosis, marrow failure, and secondary hepatosplenomegaly due to extramedullary hematopoiesis. Patients may present with systemic upset, symptoms of marrow failure, or abdominal discomfort from hepatosplenomegaly. Treatment is supportive, with bone marrow transplant reserved for younger patients. The median survival is 4-5 years, and transformation to acute myeloid leukemia is relatively common.
In contrast, acute lymphoblastic leukemia is a disease of childhood that presents with elevated white cell count and blasts on peripheral blood film. Acute myelocytic leukemia and chronic myeloid leukemia both present with raised white cell counts and blasts on blood film, but are more common in younger patients. Advanced prostate cancer may cause bone marrow failure if there is replacement of enough bone marrow by metastases, but patients would also complain of bone pain.
In summary, while bone marrow failure may be found in various diseases, specific diagnostic clues such as a leukoerythroblastic picture with teardrop-shaped red blood cells and a failed bone marrow aspirate can help distinguish myelofibrosis from other bone marrow disorders.
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This question is part of the following fields:
- Haematology
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Question 24
Correct
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A 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 25
Correct
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These results were obtained on a 65-year-old male:
Hb 110 g/L (120-170)
RBC 4.8 ×1012/L (4.2-5.8)
Hct 0.365 (0.37-0.49)
MCV 75 fL (82-98)
MCH 33.2 pg (28-33)
Platelets 310 ×109/L (140-450)
WBC 8.21 ×109/L (4.5-11)
Neutrophils 6.45 ×109/L (1.8-7.5)
Lymphocytes 1.23 ×109/L (1.0-4.5)
Monocytes 0.28 ×109/L (0-0.8)
Eosinophils 0.18 ×109/L (0.02-0.5)
Basophils 0.09 ×109/L (0-0.1)
Others 0.18 ×109/L -
What could be the possible reason for these FBC results in a 65-year-old male?Your Answer: Gastrointestinal blood loss
Explanation:Microcytic Anaemia in a 63-Year-Old Female
A Full Blood Count (FBC) analysis has revealed that a 63-year-old female is suffering from microcytic anaemia, which is characterized by low mean corpuscular volume (MCV) and low haemoglobin (Hb) levels. This type of anaemia is typically caused by iron deficiency, which is often the result of blood loss. However, in this case, menorrhagia can be ruled out as the patient is postmenopausal. Therefore, the most likely cause of the microcytic anaemia is peptic ulceration. It is important to note that pernicious anaemia or folate deficiency can cause macrocytosis, which is characterized by elevated MCV levels. Proper diagnosis and treatment are necessary to address the underlying cause of the microcytic 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 65-year-old man has been diagnosed as being vitamin B12-deficient with a B12 level of < 50 pmol/l (160–900 pmol/l) and a haemoglobin (Hb) level of 85 (115–155 g/l). It is not diet-related.
What is the most suitable course of action?Your Answer: Hydroxocobalamin 1 mg im three times a week for two weeks and then 1 mg im 3-monthly
Explanation:Treatment Options for Pernicious Anemia with Hydroxocobalamin
Pernicious anemia is a type of anemia caused by a deficiency in vitamin B12, often due to the presence of anti-intrinsic factor antibodies. Hydroxocobalamin is a form of vitamin B12 that can be used for supplementation in patients with pernicious anemia. Here are some treatment options with hydroxocobalamin:
1. Hydroxocobalamin 1 mg IM three times a week for two weeks, then 1 mg IM every three months: This is the standard dose for patients with pernicious anemia without neurological deficits.
2. Hydroxocobalamin 1 mg IM on alternate days indefinitely: This is used for patients with pernicious anemia and neurological involvement until symptom improvement reaches a plateau, then maintenance involves 1 mg IM every two months.
3. Hydroxocobalamin 1 mg IV three times a week for two weeks, then monthly: This is used for the treatment of cyanide poisoning, not for pernicious anemia.
4. Hydroxocobalamin 1 mg IM three times a week for two weeks, then oral 1 mg hydroxocobalamin: Oral supplementation is not appropriate for patients with pernicious anemia due to absorption issues.
5. Hydroxocobalamin 1 mg subcutaneously three times a week for two weeks, monthly for three months, then 3-monthly: Hydroxocobalamin is administered IM, not subcutaneously.
In conclusion, hydroxocobalamin is an effective treatment option for pernicious anemia, but the dosage and administration route should be carefully considered based on the patient’s individual needs.
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This question is part of the following fields:
- Haematology
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Question 27
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 28
Incorrect
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A 70-year-old man presents with fatigue, pallor and shortness of breath. He has been battling with an indolent colon carcinoma for the past 5 years. He also suffers from insulin-dependent diabetes, hypertension, coronary artery disease and rheumatoid arthritis. He has been feeling unwell for the past few weeks. He denies any history of melaena or haematochezia and has been amenorrhoeic for decades. A bedside stool guaiac test is negative for any blood in the stool. He is well nourished, reports taking daily supplements and is not a vegetarian. He reports that his haematocrit is 0.28 (0.35–0.55) and haemoglobin level 100 g/l (115–155 g/l).
What additional findings would you expect to observe in his full blood count?Your Answer: Reticulocytosis
Correct Answer: Increased ferritin
Explanation:Understanding Anaemia of Chronic Disease: Increased Ferritin and Decreased TIBC
Anaemia of chronic disease is a type of anaemia that is commonly seen in patients with chronic inflammatory conditions. It is characterised by a low haemoglobin level and low haematocrit, but unlike iron deficiency anaemia, it is associated with increased ferritin levels and decreased total iron-binding capacity (TIBC). This is because ferritin is a serum reactive protein that is elevated in response to the underlying inflammatory process.
Diagnosis of anaemia of chronic disease requires the presence of a chronic inflammatory condition and anaemia, which can be either normocytic or microcytic. It is important to note that a haemoglobin level of <80 g/l is very rarely associated with this type of anaemia. Treatment involves addressing the underlying disorder causing the anaemia and monitoring the haemoglobin level. Blood transfusion is only used in severe cases. It is important to differentiate anaemia of chronic disease from other types of anaemia. For example, it is characterised by a low reticulocyte count, and not reticulocytosis. Serum transferrin receptor is not affected in anaemia of chronic disease and would therefore be normal. Additionally, TIBC is reduced in anaemia of chronic disease, whereas it is increased in iron deficiency anaemia. Finally, anaemia of chronic disease is associated with either microcytosis or normocytosis, whereas macrocytosis is associated with other types of anaemia such as folate deficiency, vitamin B12 deficiency, alcohol excess, and myelodysplastic disease. In summary, understanding the unique features of anaemia of chronic disease, such as increased ferritin and decreased TIBC, can aid in its diagnosis and management.
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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 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 30
Incorrect
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What is true for a patient with blood type group O before receiving a blood transfusion?
Your Answer: The red cells have A/B antigen but no antibodies in the plasma
Correct Answer: The red cells have absent A/B antigen and plasma has anti A and anti B antibodies
Explanation:Blood Groups
Blood groups are determined by the presence or absence of certain antigens on the surface of red blood cells and the corresponding antibodies in the plasma. Blood Group O has no A or B antigens on the red cells and has both anti-A and anti-B antibodies in the plasma. Blood Group AB has both A and B antigens on the red cells but no antibodies in the plasma. Blood Group A has only A antigens on the red cells and anti-B antibodies in the plasma. Blood Group B has only B antigens on the red cells and anti-A antibodies in the plasma. It is important to know your blood group for medical purposes, such as blood transfusions, as incompatible blood types can cause serious health complications.
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This question is part of the following fields:
- Haematology
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