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Question 1
Incorrect
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A 45-year-old woman is referred by her general practitioner due to complaints of headache, fatigue and weakness. Recently, she had a prolonged menstrual bleeding that only stopped after the application of compression for a long time. She also suffered from a urinary tract infection the previous month. She has no family history of a bleeding disorder.
On examination, she has pallor, hepatosplenomegaly and lymphadenopathy. Investigations reveal that she has a low haemoglobin level, a low white blood cell count and a low platelet count; numerous blast cells are visible on peripheral blood film. Bone marrow biopsy reveals 30% of blast cells. The blood film is shown below.
Which one of the following is the most likely diagnosis?Your Answer: Chronic myeloid leukaemia (CML)
Correct Answer: Acute myeloid leukaemia (AML)
Explanation:Leukaemia is a type of cancer that affects the blood and bone marrow. There are several types of leukaemia, including acute myeloid leukaemia (AML), acute lymphoblastic leukaemia (ALL), chronic lymphocytic leukaemia (CLL), chronic myeloid leukaemia (CML), and hairy cell leukaemia. AML is characterized by the rapid proliferation of immature myeloid cells called blasts, which can cause anaemia, thrombocytopenia, bleeding problems, and an increased risk of infections. ALL is caused by a clonal proliferation of lymphoid precursors, which can lead to pancytopenia and symptoms such as fever and abdominal pain. CLL is the most common type of leukaemia and is caused by the clonal proliferation of monoclonal B lymphocytes. CML is the rarest form of leukaemia and is caused by a chromosomal translocation involving chromosomes 9 and 22. Hairy cell leukaemia is characterized by the presence of abnormal white cells with hair-like cytoplasmic projections. Treatment for leukaemia typically involves chemotherapy and sometimes a bone marrow transplant, depending on the type of disease present. Prognosis varies depending on the type of leukaemia and the age of the patient.
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This question is part of the following fields:
- Haematology/Oncology
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Question 2
Correct
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A 30-year-old woman comes to her General Practitioner complaining of fatigue and sudden shortness of breath. An urgent full blood count is performed and the patient is diagnosed with acute lymphoblastic leukemia (ALL).
What is the most probable first-line treatment option for this patient's leukemia?Your Answer: Chemotherapy
Explanation:Treatment Options for Acute Leukaemia: Chemotherapy, Stem-Cell Transplant, Blood Transfusion, Intravenous Immunoglobulins, and Radiotherapy
Acute leukaemias, such as acute lymphoblastic leukaemia (ALL), are characterized by an increase in primitive undifferentiated blast cells in the bone marrow and blood, leading to marrow failure. The traditional treatment for ALL involves four components: induction, consolidation, maintenance, and central nervous system (CNS) prophylaxis. The first-line therapy for ALL is combination chemotherapy, which aims to eradicate the blast cells. Once remission is induced, maintenance chemotherapy is given to eliminate the disease that cannot be detected under the microscope.
Stem-cell transplants can be used to treat ALL once remission is induced using chemotherapy. This treatment can be allogeneic (the patient receives stem cells from a matched or partially mismatched related or unrelated donor) or autologous (the patient receives their own stem cells). The goal of a stem-cell transplant is to restore the body’s ability to produce normal blood cells. While it can be a curative treatment for patients with this disease, it is not used as a first-line treatment.
Blood transfusions are not a treatment for acute leukaemia, but they may be necessary to treat anaemia or platelet deficiency. Patients with leukaemia are at risk of graft-versus-host disease, so they are typically given irradiated blood components. Intravenous immunoglobulins are not used to treat acute leukaemia but may be used prophylactically against infection in patients with hypogammaglobulinaemia due to cancer treatment.
Radiotherapy is not a first-line treatment for acute leukaemia, but it may be used to treat the brain or spinal cord if the disease has spread there. Total body radiotherapy may also be used before a stem-cell transplant to suppress the immune system and reduce the risk of transplant rejection.
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This question is part of the following fields:
- Haematology/Oncology
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Question 3
Correct
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A 26-year-old female patient comes to the clinic after discovering a lump in her right breast. She is uncertain about how long it has been there and reports no pain or other symptoms. She has no significant medical history. During the physical examination, a smooth, rubbery, mobile mass of approximately 2 cm in diameter is palpated. The patient is immediately referred for imaging, which reveals a small, lobulated lesion measuring about 2.5cm in width, highly suggestive of a fibroadenoma. What is the next step in managing this patient?
Your Answer: Reassurance and monitoring
Explanation:A young patient has a small fibroadenoma <3 cm, which is highly suggestive on imaging. There is no increase in the risk of breast cancer, so a core-needle biopsy is not necessary. Watchful waiting is appropriate, and cryoablation may be used for larger fibroadenomas. Fine-needle aspiration is only necessary for larger lumps or in older patients. Understanding Breast Fibroadenoma Breast fibroadenoma is a type of breast mass that develops from a whole lobule. It is characterized by a mobile, firm, and smooth lump in the breast, which is often referred to as a breast mouse. Fibroadenoma accounts for about 12% of all breast masses and is more common in women under the age of 30. Fortunately, fibroadenomas are usually benign and do not increase the risk of developing breast cancer. In fact, over a two-year period, up to 30% of fibroadenomas may even get smaller on their own. However, if the lump is larger than 3 cm, surgical excision is typically recommended. In summary, breast fibroadenoma is a common type of breast mass that is usually benign and does not increase the risk of breast cancer. While it may cause concern for some women, it is important to remember that most fibroadenomas do not require treatment and may even resolve on their own.
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This question is part of the following fields:
- Haematology/Oncology
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Question 4
Incorrect
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A 49-year-old homeless man has been admitted to the emergency department after collapsing with sudden onset left side hemiparesis and facial droop. A CT scan has revealed a large intracerebral haemorrhage, which is likely to have occurred spontaneously. The patient's medical and family history is unknown, but you suspect that coagulopathy may be the cause of the spontaneous bleed. The clotting profile and specific clotting factor levels are as follows:
- PT prolonged
- APTT prolonged
- Factor VIII high
- Factor II low
- Factor V low
- Factor VII low
- Factor IX low
- Von Willebrand factor high
What is the most probable reason for the coagulopathy?Your Answer: Disseminated intravascular coagulation
Correct Answer: Liver failure
Explanation:It is highly probable that the individual is suffering from liver failure as all clotting factors, except for factor VIII, are below normal levels. In cases of liver failure, both PT and APTT may be prolonged. Haemophilia A and B are unlikely as they result in deficiencies of specific clotting factors, whereas Von Willebrand disease may have low levels of Von Willebrand factor but the other factors remain unaffected. Disseminated intravascular coagulation, on the other hand, leads to depletion of all clotting factors.
Coagulopathy in Liver Disease: Paradoxical Supra-normal Factor VIII and Increased Thrombosis Risk
In liver failure, the levels of all clotting factors decrease except for factor VIII, which paradoxically increases. This is because factor VIII is synthesized not only in hepatic endothelial cells but also in endothelial cells throughout the body. Moreover, good hepatic function is required for the rapid clearance of activated factor VIII from the bloodstream, leading to further increases in circulating factor VIII. Despite conventional coagulation studies suggesting an increased risk of bleeding, patients with chronic liver disease are paradoxically at an increased risk of thrombosis formation. This is due to several factors, including reduced synthesis of natural anticoagulants such as protein C, protein S, and anti-thrombin, which are all essential for preventing thrombosis.
Reference
Tripodi et al. An imbalance of pro- vs anti-coagulation factors in plasma from patients with cirrhosis. Gastroenterology. 2009 Dec;137(6):2105-11. -
This question is part of the following fields:
- Haematology/Oncology
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Question 5
Correct
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A 14-year-old girl is referred to haematology due to heavy and prolonged periods that have not responded well to tranexamic acid and the combined oral contraceptive pill. Her blood tests reveal Hb of 10.3 g/dl, Plt of 239 * 109/l, WBC of 6.5 * 109/l, PT of 12.9 secs, and APTT of 37 secs. What is the probable diagnosis?
Your Answer: Von Willebrand's disease
Explanation:Understanding Von Willebrand’s Disease
Von Willebrand’s disease is a common inherited bleeding disorder that is usually passed down in an autosomal dominant manner. It behaves like a platelet disorder, with symptoms such as nosebleeds and heavy menstrual bleeding being common, while joint and muscle bleeding are rare. The disease is caused by a deficiency or abnormality in von Willebrand factor, a large glycoprotein that promotes platelet adhesion to damaged endothelium and acts as a carrier molecule for factor VIII.
There are three types of Von Willebrand’s disease. Type 1 is the most common and is characterized by a partial reduction in von Willebrand factor. Type 2 is further divided into four subtypes, each with a different abnormality in the von Willebrand factor. Type 3 is the most severe form and is caused by a total lack of von Willebrand factor, inherited in an autosomal recessive manner.
Diagnosis of Von Willebrand’s disease involves tests such as a prolonged bleeding time, APTT, factor VIII levels, and platelet aggregation with ristocetin. Management options include tranexamic acid for mild bleeding, desmopressin to raise levels of von Willebrand factor, and factor VIII concentrate. While there is no clear correlation between symptomatic presentation and type of Von Willebrand’s disease, common themes among patients include excessive mucocutaneous bleeding, bruising in the absence of trauma, and menorrhagia in females.
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This question is part of the following fields:
- Haematology/Oncology
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Question 6
Correct
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A 25-year-old man visited his GP after he discovered a solid lump in his left testis.
What is the most probable diagnosis?
Your Answer: Teratoma
Explanation:Common Scrotal Conditions: Understanding Testicular Lumps and Swellings
Testicular lumps and swellings can be a cause for concern, but not all of them are cancerous. In fact, most scrotal conditions are benign and can be easily treated. Here are some common scrotal conditions and their characteristics:
Teratoma: This solid testicular lump is suggestive of a tumour and typically presents in young soldiers aged 20-30 years.
Seminoma: This tumour usually presents in more senior soldiers aged 35-45 years.
Epididymal cyst: This benign, fluid-filled lump is usually found at the head of the epididymis and is separate from the testis.
Hydrocele: This painless, cystic, scrotal swelling lies below and anterior to the testes and will normally transilluminate.
Varicocele: This painless swelling of the testes on the left side (rarely on the right) is described as a bag of worms within the spermatic cord above the testis. It tends to occur in those aged 12+ years and is associated with infertility in approximately a quarter of men with abnormal semen parameters.
If you notice any changes in your scrotum, it is important to seek medical attention. Your healthcare provider can help determine the cause of the lump or swelling and recommend appropriate treatment.
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This question is part of the following fields:
- Haematology/Oncology
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Question 7
Correct
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A 72-year-old man presents to the Nephrology Clinic with an estimated glomerular filtration rate (eGFR) of 3 ml/min/1.73 m2. He is already undergoing renal replacement therapy. He also presents with worsening anaemia.
What is the most likely diagnosis?Your Answer: Erythropoietin deficiency
Explanation:Causes of Anaemia in End-Stage Renal Failure Patients
Anaemia is a common complication in patients with end-stage renal failure, primarily due to a decrease in the production of erythropoietin by the kidneys. Other causes of anaemia can include folate and vitamin B12 deficiency, uraemic inhibitors, and reduced half-life of circulating blood cells. The prevalence of anaemia in patients with chronic kidney disease (CKD) increases as eGFR levels decrease, with a prevalence of 12% in CKD patients. Folate deficiency and iron deficiency can also cause anaemia, but in this case, the anaemia is more likely related to poor kidney function. Deficiencies in granulocyte colony-stimulating factor and pyridoxine are less likely causes of anaemia in end-stage renal failure patients.
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This question is part of the following fields:
- Haematology/Oncology
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Question 8
Correct
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A 76-year-old male with a history of COPD and metastatic lung cancer is admitted with worsening shortness of breath. After consulting with family, it is determined that active treatment, including fluids and antibiotics, will be withdrawn as this admission is likely a terminal event. However, two days later, the patient becomes restless and agitated. What is the best course of action in managing this situation?
Your Answer: Subcutaneous midazolam
Explanation:Palliative Care Prescribing for Agitation and Confusion
When dealing with agitation and confusion in palliative care patients, it is important to identify and treat any underlying causes such as hypercalcaemia, infection, urinary retention, or medication. If these specific treatments fail, medication can be used to manage symptoms. Haloperidol is the first choice for treating agitation and confusion, with chlorpromazine and levomepromazine as alternative options. In the terminal phase of the illness, midazolam is the preferred medication for managing agitation or restlessness. Proper management of these symptoms can greatly improve the quality of life for palliative care patients.
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This question is part of the following fields:
- Haematology/Oncology
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Question 9
Incorrect
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Sophie, a 23-years old woman, visits her doctor complaining of frequent nosebleeds and unexplained bruising on her arms and legs. She mentions that her mother has also experienced similar issues but never sought medical attention. As a result, the doctor orders several blood tests to determine the most probable diagnosis.
What kind of results would be anticipated based on the likely diagnosis?Your Answer: Prolonged bleeding time, low platelet count, prolonged APTT
Correct Answer: Prolonged bleed time, normal platelet count, prolonged APTT
Explanation:Von Willebrand’s disease is a common genetic bleeding disorder that is inherited in an autosomal dominant manner. It behaves like a platelet disorder because von Willebrand Factor (vWF) is necessary for platelet adhesion to the damaged endothelium. As a result, patients experience mucocutaneous bleeding after minor injuries, such as nosebleeds and bruising. Bleeding time is prolonged because they cannot adhere to form the platelet plug, but the platelet count itself is normal. APTT is also prolonged because vWF acts as a carrier molecule for factor VIII, which is measured by APTT. This is also observed in haemophilia A, but to a greater extent.
Understanding Von Willebrand’s Disease
Von Willebrand’s disease is a common inherited bleeding disorder that is usually passed down in an autosomal dominant manner. It behaves like a platelet disorder, with symptoms such as nosebleeds and heavy menstrual bleeding being common, while joint and muscle bleeding are rare. The disease is caused by a deficiency or abnormality in von Willebrand factor, a large glycoprotein that promotes platelet adhesion to damaged endothelium and acts as a carrier molecule for factor VIII.
There are three types of Von Willebrand’s disease. Type 1 is the most common and is characterized by a partial reduction in von Willebrand factor. Type 2 is further divided into four subtypes, each with a different abnormality in the von Willebrand factor. Type 3 is the most severe form and is caused by a total lack of von Willebrand factor, inherited in an autosomal recessive manner.
Diagnosis of Von Willebrand’s disease involves tests such as a prolonged bleeding time, APTT, factor VIII levels, and platelet aggregation with ristocetin. Management options include tranexamic acid for mild bleeding, desmopressin to raise levels of von Willebrand factor, and factor VIII concentrate. While there is no clear correlation between symptomatic presentation and type of Von Willebrand’s disease, common themes among patients include excessive mucocutaneous bleeding, bruising in the absence of trauma, and menorrhagia in females.
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This question is part of the following fields:
- Haematology/Oncology
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Question 10
Incorrect
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A 49-year-old school teacher presents with abdominal fullness and weight loss over the last six months. On examination, he is pale.
Investigations reveal the following:
Investigation Result Normal range
Haemoglobin (Hb) 102 g/l 135–175 g/l
White cell count (WCC) 68.2 × 109/l 4.0–11.0 × 109/l
Neutrophil count 39.1 × 109/l 2.2–8.6 ×109/l
Lymphocyte count 4.8 × 109/l 0.8–3.5 × 109/l
Platelets (PLT) 505 × 109/l 150–400 × 109/l
Erythrocyte sedimentation rate (ESR) 56 mm/hour 1–20 mm/hour
His peripheral blood picture is shown here:
Picture courtesy: Dr R Paul, MCH, Kolkata
What is the next most definitive step in diagnosis?Your Answer: Immunophenotyping
Correct Answer: Genotype study
Explanation:Diagnostic Approaches for Chronic Myeloid Leukaemia
Chronic myeloid leukaemia (CML) is a type of blood cancer that can be diagnosed through various diagnostic approaches. A patient with elevated total leukocyte and neutrophil counts, mild anaemia, and an elevated platelet count, along with numerous myeloid line cells in different stages of differentiation, is likely to have CML. However, to confirm the diagnosis, a genotype study is necessary to demonstrate the cytogenetic hallmark of t(9:22). This can be done through molecular methods like fluorescence in situ hybridisation or cytogenetic analysis.
A bone marrow study can also be performed, which will show a greatly increased myeloid: erythroid ratio, but it will not help in confirming the diagnosis. Similarly, a low leukocyte alkaline phosphatase (LAP) score can differentiate from a leukemoid reaction but cannot confirm the diagnosis. Immunophenotyping can show cells of myeloid lineage but cannot provide a definitive diagnosis.
Iron kinetics studies are not necessary in this case as the increased total leukocyte count and peripheral smear picture suggest a chronic myeloproliferative state rather than iron deficiency. In conclusion, a genotype study is the most appropriate next step to confirm the diagnosis of CML.
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This question is part of the following fields:
- Haematology/Oncology
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Question 11
Incorrect
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An 85-year-old man has just undergone an emergency repair for a ruptured abdominal aortic aneurysm. Preoperatively he was taking aspirin and warfarin; intraoperatively he received unfractionated heparin prior to application of the aortic cross-clamp. His observation findings are heart rate 120 bpm, BP 100/60 mmHg, respiratory rate 23/min, oxygen saturation 98% on air, temperature 38ºC. His blood results on admission to the critical care unit are as follows:
Hb 110 g/L
Male: (135-180)
Female: (115 - 160)
Platelets 30 * 109/L
(150 - 400)
WBC 15 * 109/L
(4.0 - 11.0)
Fibrinogen 0.5 g/L
(2-4 g/L)
PT 20 seconds
(10-12 seconds)
APTT 60 seconds
(35-45 seconds)
FDP 60 ug/mL
(<10 ug/mL)
What is the most likely explanation for the blood abnormalities seen in this case?Your Answer: Heparin induced thrombocytopaenia
Correct Answer: Disseminated intravascular coagulation
Explanation:The most likely diagnosis for the patient’s deranged vital signs after a major operation and suspected sepsis is disseminated intravascular coagulation (DIC). This is characterized by low platelets, increased clotting time, and raised fibrin degradation products (FDPs). DIC is often caused by the release of tissue factor (TF) in response to cytokines, tumor necrosis factor, and endotoxin. Anastomotic leak, heparin-induced thrombocytopenia, warfarin use, and aspirin use are unlikely causes based on the patient’s history and blood results.
Understanding Disseminated Intravascular Coagulation (DIC) Diagnosis
Under normal conditions, coagulation and fibrinolysis work together to maintain homeostasis. However, in DIC, these processes become dysregulated, leading to widespread clotting and bleeding. One key factor in the development of DIC is the release of tissue factor (TF), a glycoprotein found on the surface of various cell types. Normally, TF is not in contact with the general circulation, but it is exposed after vascular damage or in response to certain cytokines. Once activated, TF triggers the extrinsic pathway of coagulation, which then triggers the intrinsic pathway. DIC can be caused by various factors, including sepsis, trauma, obstetric complications, and malignancy.
To diagnose DIC, a typical blood picture will show decreased platelets and fibrinogen, increased fibrinogen degradation products, and the presence of schistocytes due to microangiopathic hemolytic anemia. Additionally, both the prothrombin time and activated partial thromboplastin time are prolonged, while bleeding time and platelet count are often low. Understanding the diagnosis of DIC is crucial for prompt and effective treatment.
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This question is part of the following fields:
- Haematology/Oncology
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Question 12
Correct
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A 32-year-old patient who is a vegan and very active has been experiencing fatigue and difficulty breathing for the past two months. During examination, she appears to be anaemic.
What is the most probable vitamin deficiency causing her anaemia?
Your Answer: Vitamin B12
Explanation:Vegan Nutrition: Understanding the Availability of Vitamins
As a vegan, it is important to understand the availability of vitamins in your diet. One vitamin that vegans are at risk of deficiency in is vitamin B12, which is primarily found in animal products such as meat, fish, eggs, and milk. However, there are vegan-friendly sources of vitamin B12 such as fortified cereals and plant-based milks.
Contrary to popular belief, vitamin B6, vitamin A, vitamin B1, and vitamin C are all readily available in vegan-friendly foods. Vitamin B6 can be found in bread, whole grains, vegetables, peanuts, and potatoes. Vitamin A can be found in yellow, red, and green vegetables as well as yellow fruits. Vitamin B1 can be found in peas, fruit, whole grain bread, and fortified cereals. Vitamin C is predominantly found in fruits and vegetables such as oranges, peppers, strawberries, broccoli, potatoes, and Brussels sprouts.
By understanding the availability of vitamins in vegan-friendly foods, vegans can ensure they are meeting their nutritional needs and maintaining a healthy diet.
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This question is part of the following fields:
- Haematology/Oncology
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Question 13
Incorrect
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A 32-year-old woman presents to her General Practitioner with a 1-week history of epistaxis and bleeding gums. She recently recovered from influenza and has a history of hypothyroidism for which she takes levothyroxine. On examination, she has multiple bruises and petechiae over her lower limbs and trunk. Her investigations reveal a low platelet count and normal coagulation parameters. What is the most likely diagnosis?
Your Answer: Factor V Leiden
Correct Answer: Immune thrombocytopenic purpura (ITP)
Explanation:The patient is likely suffering from immune thrombocytopenic purpura (ITP), a condition where the immune system destroys platelets. This can be caused by autoantibodies targeting glycoprotein IIb/IIIa or Ib–V–IX complexes, and is often seen in patients with autoimmune diseases. Acute cases can occur in children following a viral infection or vaccination, and symptoms typically include bleeding from the skin and mucous membranes. Given the patient’s history of hypothyroidism, ITP is a more likely diagnosis than other conditions such as Factor V Leiden, thrombotic thrombocytopenic purpura (TTP), or von Willebrand’s disease (VWD). Disseminated intravascular coagulation (DIC) is also unlikely, as the patient’s coagulation profile is normal aside from the low platelet count.
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This question is part of the following fields:
- Haematology/Oncology
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Question 14
Incorrect
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A 65-year-old man with a history of depression and lumbar spinal stenosis presents with a swollen and painful left calf. He is evaluated in the DVT clinic and a raised D-dimer is detected. A Doppler scan reveals a proximal deep vein thrombosis. Despite being active and feeling well, the patient has not undergone any recent surgeries or been immobile for an extended period. As a result, he is initiated on a direct oral anticoagulant. What is the optimal duration of treatment?
Your Answer: Lifelong
Correct Answer: 6 months
Explanation:For provoked cases of venous thromboembolism, such as those following recent surgery, warfarin treatment is typically recommended for a duration of three months. However, for unprovoked cases, where the cause is unknown, a longer duration of six months is typically recommended.
NICE updated their guidelines on the investigation and management of venous thromboembolism (VTE) in 2020. The use of direct oral anticoagulants (DOACs) is recommended as first-line treatment for most people with VTE, including as interim anticoagulants before a definite diagnosis is made. Routine cancer screening is no longer recommended following a VTE diagnosis. The cornerstone of VTE management is anticoagulant therapy, with DOACs being the preferred choice. All patients should have anticoagulation for at least 3 months, with the length of anticoagulation being determined by whether the VTE was provoked or unprovoked.
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This question is part of the following fields:
- Haematology/Oncology
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Question 15
Correct
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A 32-year-old man presents to his General Practitioner having been feeling unwell for a few days with a persistent cough.
Investigations:
Investigation Result Normal value
Haemoglobin (Hb) 130 g/l 120–160 g/l
White cell count (WCC) 18 × 109/l 4.5–11.0 × 109/l
Neutrophils 12 × 109/l 2.0–7.5 × 109/l
Platelets (PLT) 450 × 109/l 150–400 × 109/l
A blood film shows atypical lymphocytes.
What is the most likely diagnosis?Your Answer: Acute bacterial infection
Explanation:Differential Diagnosis for a Patient with Abnormal Full Blood Count Results
When a patient presents with abnormal full blood count (FBC) results, it is important to consider a range of potential diagnoses. In this case, the patient has neutrophilia and atypical lymphocytes, indicating an acute bacterial infection. Other potential diagnoses, such as chronic myeloid leukaemia, tuberculosis, cytomegalovirus infection, and pregnancy, can be ruled out based on the absence of key symptoms and blood film findings. Clinical prediction scores can be used to aid in antibiotic stewardship. It is important to consider all potential diagnoses and conduct further testing as needed to ensure accurate diagnosis and appropriate treatment.
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This question is part of the following fields:
- Haematology/Oncology
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