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  • Question 1 - Out of the following, which tumour cells are found in patients with Hodgkin...

    Correct

    • Out of the following, which tumour cells are found in patients with Hodgkin disease?

      Your Answer: Reed-Sternberg cells

      Explanation:

      The diagnosis of Hodgkin disease requires the identification of Reed-Sternberg cells in a characteristic cellular background. The normal cell of origin for the Reed-Sternberg cells remains unclear, with the predominance of evidence indicating a B or T lymphocyte.

    • This question is part of the following fields:

      • Haematology & Oncology
      5.5
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  • Question 2 - A 32-year-old male who is a known case of sickle cell disease presents...

    Incorrect

    • A 32-year-old male who is a known case of sickle cell disease presents to the Accident and Emergency (A&E) department with fever, tachypnoea, and rib pain. On examination, he has a low-grade fever of 37.9°C, oxygen saturation of 95% on air, and bilateral vesicular breath sounds on chest auscultation. CXR shows opacification in the right middle zone. Which of these statements most accurately describes the initial management of this patient?

      Your Answer: Empirical antibiotic therapy is not indicated

      Correct Answer: Incentive spirometry is indicated

      Explanation:

      This is a typical picture of acute chest syndrome (ACS). According to the British Committee for Standards in Haematology (BCSH), ACS is defined as ‘an acute illness characterised by fever and/or respiratory symptoms, accompanied by a new pulmonary infiltrate on chest X-ray’. ACS occurs in sequestration crisis, which is one of the four main types of crises occurring in sickle cell disease.

      The fundamentals of initial management are as follows:
      1. Oxygen therapy to maintain saturation >95%
      2. Intravenous fluids to ensure euvolemia
      3. Adequate pain relief
      4. Incentive spirometry in all patients presenting with rib or chest pain
      5. Antibiotics with cover for atypical organisms
      6. Bronchodilators if asthma co-exists with acute chest syndrome, or if there is an evidence of acute bronchospasm on auscultation
      7. Early consultation with the critical care team and haematology department

      A senior haematologist then makes a decision as to whether a simple or exchange transfusion is necessary in order to achieve a target Hb of 10.0–11.0g/dL in either instance.

      Sickle Cell Crises:
      Sickle cell anaemia is characterised by periods of good health with intervening crises:
      1. Sequestration crisis: acute chest syndrome (i.e. fever, dyspnoea, chest/rib pain, low pO2, and pulmonary infiltrates)

      2. Thrombotic (painful or vaso-occlusive) crisis: precipitated by infection, dehydration, and deoxygenation

      3. Aplastic crisis: sudden fall in haemoglobin without marked reticulocytosis, usually occurring secondary to parvovirus infection

      4. Haemolytic crisis: fall in haemoglobin secondary to haemolysis, rare type of sickle cell crises

    • This question is part of the following fields:

      • Haematology & Oncology
      34.3
      Seconds
  • Question 3 - A 50-year-old woman with lung cancer develops deep vein thrombosis (DVT). She is...

    Incorrect

    • A 50-year-old woman with lung cancer develops deep vein thrombosis (DVT). She is reviewed in the hospital clinic and started on low-molecular-weight heparin (LMWH). Which of the following should be the most appropriate treatment plan?

      Your Answer: Switch to warfarin, continue for 6 months

      Correct Answer: Continue on LMWH for 6 months

      Explanation:

      Patients with active cancer are at a continuous risk of having venous thromboembolism (VTE), such as DVT. Therefore, a six-month course of an anticoagulant such as LMWH is recommended. LMWH is the drug of choice since its side effects can be reversed and it can be stopped easily in case of a cancer-related bleed, for example, massive haemoptysis in a patient with lung cancer.

    • This question is part of the following fields:

      • Haematology & Oncology
      44.9
      Seconds
  • Question 4 - A 49-year-old female is admitted to the hospital with shortness of breath and...

    Correct

    • A 49-year-old female is admitted to the hospital with shortness of breath and pleuritic chest pain. She also complains of loss of appetite for the past four months. Her admission CXR shows right-sided pleural effusion. An underlying malignancy is suspected and a series of tumour markers are requested, the results of which are: CA 19-9: 36 IU/mL (<40), CA 125: 654 IU/ml (<30), CA 15-3: 9 IU/ml (<40). What is the most likely underlying diagnosis?

      Your Answer: Ovarian fibroma

      Explanation:

      The patient has Meigs syndrome. Meigs syndrome is defined as a triad of benign ovarian tumour with ascites and pleural effusion that resolves after resection of the tumour. Ovarian fibromas constitute the majority of the benign tumours seen in Meigs syndrome.

      Tumour markers can be divided into:
      1. Monoclonal antibodies
      CA 125: Ovarian cancer, primary peritoneal cancer
      CA 19-9: Pancreatic cancer
      CA 15-3: Breast cancer

      2. Tumour specific antigens
      Prostate specific antigen (PSA): Prostatic carcinoma
      Alpha-feto protein (AFP): Hepatocellular carcinoma, teratoma
      Carcinoembryonic antigen (CEA): Colorectal cancer
      S-100: Melanoma, schwannomas
      Bombesin: Small cell lung carcinoma, gastric cancer

      3. Enzymes
      Alkaline phosphatase (ALP)
      Neuron specific enolase (NSE)

      4. Hormones
      Calcitonin
      Antidiuretic hormone (ADH)
      Human chorionic gonadotropin (hCG)

    • This question is part of the following fields:

      • Haematology & Oncology
      24.6
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  • Question 5 - A 23-year-old man is being investigated for excessive bleeding following a tooth extraction....

    Correct

    • A 23-year-old man is being investigated for excessive bleeding following a tooth extraction. His coagulation profile shows: Plts: 173 x 10^9/L, PT: 12.9 secs, APTT: 84 secs. Which clotting factor is he most likely deficient in?

      Your Answer: Factor VIII

      Explanation:

      The patient is most likely a case of haemophilia A which is the genetic deficiency of clotting factor VIII in blood.

      Haemophilia is an X-linked recessive disorder of coagulation. Up to 30% of patients have no family history of the condition. Haemophilia A is more common than haemophilia B and accounts for 90% of the cases. In haemophilia B (Christmas disease), there is a deficiency of clotting factor IX.

      Characteristic features of haemophilia include hemarthrosis, haematomas, and prolonged bleeding following trauma or surgery. Coagulation profile of a haemophiliac person shows prolonged bleeding time, activated partial thromboplastin time (APTT), thrombin time (TT), but a normal prothrombin time (PT).

    • This question is part of the following fields:

      • Haematology & Oncology
      18.2
      Seconds
  • Question 6 - A 34-year-old man who is HIV positive has started treatment for Burkitt lymphoma....

    Correct

    • A 34-year-old man who is HIV positive has started treatment for Burkitt lymphoma. His chemotherapy regime includes cyclophosphamide, vincristine, methotrexate, and prednisolone. After one day of starting chemotherapy, he becomes confused and complains of muscle cramps in his legs. Which one of the following is most likely to have occurred?

      Your Answer: Tumour lysis syndrome

      Explanation:

      Tumour lysis syndrome (TLS), triggered by the introduction of chemotherapy, has resulted in the aforementioned symptoms in this patient.

      TLS is a potentially fatal condition occurring as a complication during the treatment of high-grade lymphomas and leukaemias. It occurs from the simultaneous breakdown (lysis) of the tumour cells and subsequent release of chemicals into the bloodstream. This leads to hyperkalaemia and hyperphosphatemia in the presence of hyponatraemia. TLS can occur in the absence of chemotherapy, but it is usually triggered by the introduction of combination chemotherapy. Awareness of the condition is critical for its prophylactic management.

      Rasburicase should be given prior to chemotherapy in order to reduce the risk of tumour lysis syndrome (TLS). Rasburicase is a recombinant version of urate oxidase which is an enzyme that metabolizes uric acid to allantoin. Allantoin is 5–10 times more soluble than uric acid, hence, renal excretion is more effective.

      Burkitt lymphoma is a high-grade B-cell neoplasm. There are two major forms:
      1. Endemic (African) form: typically involves maxilla or mandible.
      2. Sporadic form: abdominal (e.g. ileocaecal) tumours are the most common form. More common in patients with HIV.

      Burkitt lymphoma is associated with the c-myc gene translocation, usually t(8;14). The Epstein-Barr virus (EBV) is strongly implicated in development of the African form of Burkitt lymphoma and to a lesser extent, the sporadic form.

      Management of the lymphoma is with chemotherapy. This tends to produce a rapid response which may cause TLS.

    • This question is part of the following fields:

      • Haematology & Oncology
      64.9
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  • Question 7 - In chemotherapy, what is the rationale behind using combinations of chemotherapeutic agents rather...

    Incorrect

    • In chemotherapy, what is the rationale behind using combinations of chemotherapeutic agents rather than single agents?

      Your Answer: Metastases are less common in combination therapy

      Correct Answer: Combination therapy decreases the chances of drug resistance developing

      Explanation:

      There are two main reasons for using combinations of chemotherapeutic agents rather than single agents. First, different drugs exert their effects through different mechanisms, therefore, carefully combining them will increase the number of tumour cells killed in each cycle as well as decrease their chances of developing drug resistance. Second, there may be an even greater effect with drugs that are synergistic.

    • This question is part of the following fields:

      • Haematology & Oncology
      35.5
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  • Question 8 - Which of the following is the most useful marker of prognosis in multiple...

    Correct

    • Which of the following is the most useful marker of prognosis in multiple myeloma?

      Your Answer: B2-microglobulin

      Explanation:

      B2-microglobulin is a useful marker of prognosis in multiple myeloma (MM). Raised levels imply a poorer prognosis. Low levels of albumin are also associated with a poor prognosis.

    • This question is part of the following fields:

      • Haematology & Oncology
      18
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  • Question 9 - A 67-year-old man who has terminal lung cancer and is taking morphine slow...

    Incorrect

    • A 67-year-old man who has terminal lung cancer and is taking morphine slow release tablet (MST) 60mg bd as an analgesic, is reviewed. Recently, he has been unable to take medications orally and, thus, a decision has been made to set up a syringe driver. Out of the following, what dose of diamorphine should be prescribed for the syringe driver?

      Your Answer: 30mg

      Correct Answer: 40mg

      Explanation:

      The dose is calculated, using the conversion factor, as follows:

      (Conversion factor used to convert oral morphine to subcutaneous diamorphine = Divide the total daily dose of oral morphine by 3)
      Hence,
      60mg*2 = 120mg
      120mg/3 = 40mg

      The side effects of opioids can be transient or persistent, and these include constipation, nausea, and drowsiness. Therefore, all patients taking opioids should also be prescribed a laxative and an anti-emetic (if the nausea is persistent). Dose-adjustment may be necessary in cases of persistent drowsiness. Moreover, strong opioids can also provide quick relief from metastatic bone pain, as compared to NSAIDs, bisphosphonates, and radiotherapy.

    • This question is part of the following fields:

      • Haematology & Oncology
      29.8
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  • Question 10 - A 25-year-old female presents to the acute medical unit with several lumps in...

    Incorrect

    • A 25-year-old female presents to the acute medical unit with several lumps in her neck and under her arms, weight loss, vomiting, and low mood. She is investigated and is found to have several areas of suspicious lymphadenopathy including in the neck, both axillae, and mediastinum. She also has multiple lesions in her liver which are confirmed to be the manifestations of Hodgkin lymphoma after biopsy. Which stage of the disease is the patient currently at?

      Your Answer: III

      Correct Answer: IV

      Explanation:

      The patient is on stage IV according to the Ann Arbor staging system for Hodgkin lymphoma (HL). The disease has spread beyond the lymph nodes into the liver (involvement of extra lymphatic organ).

      HL is a malignant proliferation of lymphocytes characterised by the presence of distinctive giant cells known as Reed-Sternberg cells. It has a bimodal age distribution being most common in the third and seventh decades of life.

      Staging of HL is done according to the Ann Arbor staging system:
      Stage
      I: Single lymph node region (I) or one extra lymphatic site (IE)

      II: Two or more lymph node regions on same side of the diaphragm (II) or local extra lymphatic extension plus one or more lymph node regions on same side of the diaphragm (IIE)

      III: Lymph node regions on both sides of the diaphragm (III) which may be accompanied by local extra lymphatic extension (IIIE)

      IV: Diffuse involvement of one or more extra lymphatic organs or sites

      Suffix
      A: No B symptoms

      B: Presence of at least one of the following: unexplained weight loss >10% baseline during 6 months before staging; recurrent unexplained fever >38°C; recurrent night sweats

    • This question is part of the following fields:

      • Haematology & Oncology
      37.5
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  • Question 11 - A 45-year-old woman who is being treated for Hodgkin's lymphoma with ABVD chemotherapy...

    Incorrect

    • A 45-year-old woman who is being treated for Hodgkin's lymphoma with ABVD chemotherapy is reviewed on the haematology ward. Six days ago, she was admitted with a fever of 38.9°C and was immediately started on piperacillin + tazobactam (Tazocin). Her blood picture on arrival was as follows: Haemoglobin: 10.1 g/dL, Platelets: 311 x 10^9/L, White cell count: 0.8 x 10^9/L, Neutrophils: 0.35 x 10^9/L, Lymphocytes: 0.35 x 10^9/L. After 48 hours, she remained febrile and tachycardic. Tazocin was stopped and meropenem in combination with vancomycin was prescribed. She still remains unwell today with a temperature of 38.4°C, heart rate of 96 bpm, and blood pressure of 102/66 mmHg. Respiratory examination is consistently unremarkable and blood and urine cultures have failed to show any cause for the fever. Which of the following is the most appropriate next step of management?

      Your Answer: Add G-CSF

      Correct Answer: Add amphotericin B

      Explanation:

      This patient meets the diagnostic criteria for neutropenic sepsis, which is a relatively common complication of cancer therapy – usually chemotherapy occurring 7–14 days after. It is defined as a neutrophil count of <0.5 x 10^9/L in a patient undergoing anticancer treatment and who has either a temperature higher than 38°C or has other features consistent with clinically significant sepsis. Management approach is the same as mentioned in this case. However, if the patient still remains unwell, then an antifungal such as amphotericin B is started after risk-stratifying the patient and carrying out investigations (e.g. HRCT and Aspergillus PCR) to determine the likelihood of systemic fungal infection.

    • This question is part of the following fields:

      • Haematology & Oncology
      30.7
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  • Question 12 - Which of the following is not a recognised feature of methemoglobinemia? ...

    Correct

    • Which of the following is not a recognised feature of methemoglobinemia?

      Your Answer: Decreased pO2 but normal oxygen saturation

      Explanation:

      Normal pO2 but decreased oxygen saturation is characteristic of methemoglobinemia.

      Methemoglobinemia is a rare condition in which the haemoglobin iron is in oxidized or ferric state (Fe3+) and cannot reversibly bind oxygen. Normally, the conversion of ferrous form of iron (Fe2+) to its ferric form (Fe3+) is regulated by NADH methaemoglobin reductase, which results in the reduction of methaemoglobin to haemoglobin. Disruption in the enzyme leads to increased methaemoglobin in the blood. There is tissue hypoxia as Fe3+ cannot bind oxygen, and hence the oxygen-haemoglobin dissociation curve is shifted to the left.

      Methemoglobinemia can occur due to congenital or acquired causes. Congenital causes include haemoglobin variants such as HbM and HbH, and deficiency of NADH methaemoglobin reductase. Acquired causes are drugs (e.g. sulphonamides, nitrates, dapsone, sodium nitroprusside, and primaquine) and chemicals (such as aniline dyes).

      The features of methemoglobinemia are cyanosis, dyspnoea, anxiety, headache, severe acidosis, arrhythmias, seizures, and loss of consciousness. Patients have normal pO2 but oxygen saturation is decreased. Moreover, presence of chocolate-brown coloured arterial blood (colour does not change with addition of O2) and brown urine also point towards the diagnosis of methemoglobinemia.

    • This question is part of the following fields:

      • Haematology & Oncology
      23.9
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  • Question 13 - Which of the following is mostly associated with thymomas? ...

    Correct

    • Which of the following is mostly associated with thymomas?

      Your Answer: Red cell aplasia

      Explanation:

      Red cell aplasia is commonly associated with thymomas.

      Thymoma is the most common tumour of the anterior mediastinum and is usually detected between the sixth and seventh decades of life. It is associated with myasthenia gravis (30–40% of patients), red cell aplasia, and dermatomyositis. Compression of airway and cardiac tamponade are the common causes of death in thymoma.

    • This question is part of the following fields:

      • Haematology & Oncology
      8.4
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  • Question 14 - A 4-year-old boy is admitted to the hospital after developing hemarthrosis in his...

    Correct

    • A 4-year-old boy is admitted to the hospital after developing hemarthrosis in his right knee whilst playing in the garden. Following blood results are obtained: Plts: 220 x 10^9/L, PT: 11 secs, APTT: 76 secs, Factor VIIIc activity: Normal. What is the most likely diagnosis?

      Your Answer: Haemophilia B

      Explanation:

      A grossly elevated APTT may be caused by heparin therapy, haemophilia, or antiphospholipid syndrome. A normal factor VIIIc activity, however, points towards the diagnosis of haemophilia B, which is the deficiency of factor IX in the blood.

      Haemophilia is an X-linked recessive disorder of coagulation. Up to 30% of patients have no family history of the condition. Haemophilia A is due to the deficiency of clotting factor VIII and is more common than haemophilia B, accounting for 90% of the cases. In haemophilia B (Christmas disease), there is a deficiency of clotting factor IX.

      Characteristic features of haemophilia include hemarthrosis, haematomas, and prolonged bleeding following trauma or surgery. Coagulation profile of a haemophiliac person shows prolonged bleeding time, activated partial thromboplastin time (APTT), thrombin time (TT), but a normal prothrombin time (PT).

    • This question is part of the following fields:

      • Haematology & Oncology
      23
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  • Question 15 - A 54-year-old woman presents to the A&E department with a five-day history of...

    Incorrect

    • A 54-year-old woman presents to the A&E department with a five-day history of back pain which is located in the lower thoracic region and is worsened by coughing and sneezing. There has been no change in bowel habit or urinary symptoms. Her past medical history includes breast cancer and osteoarthritis. On examination, there is diffuse tenderness in the lower thoracic region. Perianal sensation is normal and lower limb reflexes are brisk. Which one of the following is the most appropriate management plan?

      Your Answer: Oral dexamethasone + urgent thoracic/lumbar spine x-ray

      Correct Answer: Oral dexamethasone + urgent MRI

      Explanation:

      The patient has spinal cord compression until proven otherwise. Urgent assessment is required.

      Spinal cord compression is an oncological emergency and affects up to 5% of cancer patients. Extradural compression accounts for the majority of cases, usually due to vertebral body metastases. One of the most common causes of spinal cord compression is osteoarthritis. It is also more commonly seen in patients with lung, breast, or prostate cancer.

      Clinical features include:
      1. Back pain: the earliest and most common symptom, may worsen on lying down or coughing
      2. Lower limb weakness
      3. Sensory changes: sensory loss and numbness
      4. Neurological signs: depending on the level of the lesion.
      Lesions above L1 usually result in upper motor neurone signs in the legs. Lesions below L1 usually cause lower motor neurone signs in the legs and perianal numbness. Tendon reflexes are increased below the level of the lesion and absent at the level of the lesion.

      Management options are:
      1. High-dose oral dexamethasone
      2. Urgent MRI for consideration of radiotherapy or surgery

    • This question is part of the following fields:

      • Haematology & Oncology
      48.8
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  • Question 16 - A 25-year-old female patient presents with massive haemorrhage. After grouping, her blood sample...

    Correct

    • A 25-year-old female patient presents with massive haemorrhage. After grouping, her blood sample comes out to be B RhD negative. You work in the hospital's blood bank and are asked to prepare two units each of red blood cells (RBCs) and fresh frozen plasma (FFP). You manage to obtain the RBCs but not the Group B FFP as it is unavailable. Therefore, out of the following, FFP from a donor of which blood group would be best to transfuse?

      Your Answer: AB RhD negative

      Explanation:

      Group AB donors are the universal donors of FFP. This is because they produce neither anti-A nor anti-B antigens in their plasma and are, therefore, compatible with all ABO groups.

      The aforementioned patient’s blood group is B meaning, thereby, she naturally produces anti-A antigens in her plasma and would need to receive plasma that does not have anti-B antigens in it. Hence, she can only receive FFP from donors of group B or AB. Moreover, as she is of childbearing age, she must receive RhD negative blood in order to avoid problems with future pregnancies if her foetus would be RhD positive.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 17 - Which of the following features is characteristic of immune thrombocytopenic purpura? ...

    Incorrect

    • Which of the following features is characteristic of immune thrombocytopenic purpura?

      Your Answer: Splenomegaly is found in 50% of cases

      Correct Answer: Autoimmune haemolytic anaemia is a recognised association

      Explanation:

      Immune thrombocytopenic purpura (ITP) refers to thrombocytopaenia occurring in the absence of toxic exposure or other diseases associated with low platelets and involves IgG-type antibodies. It is characterised by normal or increased marrow megakaryocytes, shortened platelet survival, and the absence of splenomegaly. Autoimmune haemolytic anaemia (AIHA) occurs commonly in association with ITP. Leukemic transformation, however, does not occur in ITP.

      In neonatal ITP, IgG antibodies are passively transferred across the placenta. The infant platelet count may be normal at birth but decreases within 12–24 hours. It is rarely severe enough to induce bleeding diathesis in the infant.

    • This question is part of the following fields:

      • Haematology & Oncology
      21.9
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  • Question 18 - What are the most common types of transformation seen in patients with polycythaemia...

    Incorrect

    • What are the most common types of transformation seen in patients with polycythaemia vera?

      Your Answer: Myelofibrosis + chronic myeloid leukaemia

      Correct Answer: Myelofibrosis + acute myeloid leukaemia

      Explanation:

      5–15% of the cases of polycythaemia vera progress to myelofibrosis or acute myeloid leukaemia (AML).

      Polycythaemia vera (PV), also known as polycythaemia rubra vera, is a myeloproliferative disorder caused by clonal proliferation of marrow stem cells leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets. It has peak incidence in the sixth decade of life, with typical features including hyperviscosity, pruritus, splenomegaly, haemorrhage (secondary to abnormal platelet function), and plethoric appearance.

      Some management options of PV include lose-dose aspirin, venesection (first-line treatment), hydroxyurea (slightly increased risk of secondary leukaemia), and radioactive phosphorus (P-32) therapy.

      In PV, thrombotic events are a significant cause of morbidity and mortality. 5–15% of the cases progress to myelofibrosis or AML. The risk of having AML is increased with chemotherapy treatment.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 19 - A 42-year-old male patient is admitted with recurrent pancreatitis. He also has a...

    Correct

    • A 42-year-old male patient is admitted with recurrent pancreatitis. He also has a history of parotitis. CT scan is carried out revealing no pancreatic mass, but evidence of widespread lymphadenopathy is seen. Dedicated liver imaging reveals a stricture in the common bile duct but no stones. What is the most likely diagnosis?

      Your Answer: IgG4 disease

      Explanation:

      IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition that can affect nearly any organ system: the pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, meninges, aorta, breast, prostate, thyroid, pericardium, and skin. The histopathological features are similar across organs, regardless of the site. IgG4-RD is analogous to sarcoidosis, in which diverse organ manifestations are linked by similar histopathological characteristics. Raised concentrations of IgG4 in tissue and serum can be helpful in diagnosing IgG4 disease, but neither is a specific diagnostic marker.

      Some IgG4-RDs are:
      1. Autoimmune pancreatitis
      2. Riedel’s Thyroiditis
      3. Mediastinal and Retroperitoneal Fibrosis
      4. Periaortitis/periarteritis/Inflammatory aortic aneurysm
      5. Kuttner Tumour (submandibular glands)
      6. IgG4-related Mikulicz disease (lacrimal, parotid, and submandibular glands)

    • This question is part of the following fields:

      • Haematology & Oncology
      32.9
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  • Question 20 - A 4-year-old girl is diagnosed with acute lymphoblastic leukaemia after presenting with lethargy...

    Incorrect

    • A 4-year-old girl is diagnosed with acute lymphoblastic leukaemia after presenting with lethargy and easy bruising. Which of the following is a marker of a bad prognosis in this disease?

      Your Answer: Pre-B phenotype

      Correct Answer: Philadelphia chromosome positive

      Explanation:

      Philadelphia translocation, t(9;22), is a marker of poor prognosis in acute lymphoblastic leukaemia (ALL).

      ALL is the malignancy of lymphoid progenitor cells affecting B or T cell lineage. This results in the arrest of lymphoid cell maturation and proliferation of immature blast cells (lymphoblasts), leading to bone marrow and tissue infiltration.

      ALL is the most common type of childhood cancers. Its peak incidence is between two to three years of age.

      Acute B lymphoblastic leukaemia (B-ALL) is the most common type of ALL, involving overproduction of B-cell lymphoblasts. It is manifested by low initial WCC and is associated with a good prognosis.

      Poor prognostic factors for ALL include:
      1. Pre-B cell or T-cell ALL (T-ALL)
      2. Philadelphia translocation, t(9;22)
      3. Age <2 years or >10 years
      4. Male sex
      5. CNS involvement
      6. High initial WBC (e.g. >100 x 10^9/L)
      7. non-Caucasian

    • This question is part of the following fields:

      • Haematology & Oncology
      21.6
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  • Question 21 - Which one of the following is true of gamma delta T cells? ...

    Incorrect

    • Which one of the following is true of gamma delta T cells?

      Your Answer: They are the mature form of alpha beta T cells

      Correct Answer: They play a role in the skin and gut

      Explanation:

      Gamma delta T cells are of low abundance in the body, are found in the gut mucosa, skin, lungs and uterus, and are involved in the initiation and propagation of immune responses. Their ligands are not known in detail, but the gamma delta T cell receptors recognise intact proteins rather than MHC-presented peptides. Like alpha beta T cells, they develop in the thymus.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 22 - A 52-year-old man is referred to the oncology clinic by his GP. He...

    Correct

    • A 52-year-old man is referred to the oncology clinic by his GP. He recently suffered a fracture of his right humerus with minimal trauma. The results of the blood tests, taken on his arrival, prompted the referral: Hb: 8.9 g/dL, WCC: 9.5 x 10^9/L, Plts: 140 x 10^9/L, MCV: 86 fL, ESR: 60mm/1st hour, Na+: 149 mmol/L, K+: 3.6 mmol/L, Urea: 15 mmol/L, Creatinine: 160 mmol/L, Calcium (corrected): 2.89 mmol/L, Albumin: 28g/L, Total protein: 89 g/L. X-ray of right humerus reported a possible pathological fracture. Which of the following investigations would help best in confirming the most likely diagnosis?

      Your Answer: Protein electrophoresis

      Explanation:

      The most likely diagnosis with anaemia, raised erythrocyte sedimentation rate (ESR), hypercalcaemia, renal impairment, and raised total protein with low albumin is multiple myeloma (MM). Protein electrophoresis will confirm the presence of monoclonal band of paraprotein. Of note, a radioisotope bone scan is not a good test for picking up the lytic lesions of MM.

      Diagnosis of MM is based on the confirmation of (a) one major criterion and one minor criterion or (b) three minor criteria in an individual who has signs or symptoms of multiple myeloma.

      Major criteria:
      1. >30% plasma cells on bone marrow biopsy
      2. Monoclonal band of paraprotein on electrophoresis: >35g/L for IgG, 20g/L for IgA, or >1g of light chains excreted in the urine per day

      Minor criteria:
      1. 10–30% plasma cells on bone marrow biopsy
      2. Abnormal monoclonal band but levels less than listed above
      3. Lytic bone lesions observed radiographically
      4. Immunosuppression

    • This question is part of the following fields:

      • Haematology & Oncology
      39.8
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  • Question 23 - A 25-year-old woman noticed an episode of passing blood instead of urine in...

    Correct

    • A 25-year-old woman noticed an episode of passing blood instead of urine in the morning. She looks anaemic, but rest of the examination is normal. Her GP has arranged for a urological examination, which has come out to be normal as well. What is the most likely diagnosis?

      Your Answer: Paroxysmal nocturnal haemoglobinuria

      Explanation:

      The patient has paroxysmal nocturnal haemoglobinuria (PNH). The classic sign of the disease is red discolouration of the urine due to the presence of haemoglobin and hemosiderin from the breakdown of red blood cells. As the urine is more concentrated in the morning, this is when the colour is most pronounced.

      PNH is an acquired clonal disorder of haematopoietic stem cells, characterised by variable combinations of intravascular haemolysis, thrombosis, and bone marrow failure. Diagnosis is made by flow cytometric evaluation of blood, which confirms the CD55 and CD59 deficiencies and deficiency of expression of other GPI-linked proteins. This test is replacing older complement-based assays such as the Ham test and sucrose lysis test.

    • This question is part of the following fields:

      • Haematology & Oncology
      19.7
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  • Question 24 - A 53-year-old woman presents with a painless lump in the left breast associated...

    Correct

    • A 53-year-old woman presents with a painless lump in the left breast associated with nipple discharge. The skin over the lump has an orange peel skin appearance. According to the patient, the lump has increased in size, with time. Diagnosis of breast cancer is strongly suspected. Which of the following would be most useful in monitoring the prognosis of breast cancer, in this case?

      Your Answer: Lymph node metastases

      Explanation:

      The prognosis of breast cancer depends chiefly on the extent of nodal metastases.

      The breast cancer TNM staging system is the most common way that doctors use to stage breast cancer. TNM stands for Tumour, Node, Metastasis. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero), which is non-invasive ductal carcinoma in situ (DCIS), and stages I through IV (1 through 4), which are used for invasive breast cancer.

      Staging can be clinical or pathological. Clinical staging is based on the results of tests done before surgery, which may include physical examinations, mammogram, ultrasound, and MRI scans. Pathologic staging is based on what is found during surgery to remove breast tissue and lymph nodes. In general, pathological staging provides the most information to determine a patient’s prognosis.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 25 - A 24-year-old smoker with testicular cancer presents with exertional dyspnoea, wheezing, and persistent...

    Incorrect

    • A 24-year-old smoker with testicular cancer presents with exertional dyspnoea, wheezing, and persistent non-productive cough. He completed a course of chemotherapy comprising of cisplatin, bleomycin, and etoposide three months ago. On examination, there are fine bilateral basal crackles. Which of the following is the most likely diagnosis?

      Your Answer: Cryptogenic fibrosing alveolitis

      Correct Answer: Bleomycin toxicity

      Explanation:

      The cytotoxic drug bleomycin can cause bleomycin-induced pneumonitis (BIP). It usually occurs during chemotherapy but can also occur up to six months post-therapy.

    • This question is part of the following fields:

      • Haematology & Oncology
      51.7
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  • Question 26 - A 52-year-old woman is being reviewed in the oncology clinic following debulking surgery...

    Correct

    • A 52-year-old woman is being reviewed in the oncology clinic following debulking surgery one month ago. She is a known case of primary peritoneal cancer with two liver metastases and has now come in for review prior to adjuvant chemotherapy. During her chemotherapy, which of the following tumour markers would be the most appropriate to monitor her disease progression?

      Your Answer: CA 125

      Explanation:

      Tumour marker CA 125 is mostly associated with primary peritoneal cancer and ovarian cancer. It can be used to monitor response to chemotherapy, alongside regular CT scans.

      Tumour markers can be divided into:
      1. Monoclonal antibodies
      CA 125: Ovarian cancer, primary peritoneal cancer
      CA 19-9: Pancreatic cancer
      CA 15-3: Breast cancer

      2. Tumour specific antigens
      Prostate specific antigen (PSA): Prostatic carcinoma
      Alpha-feto protein (AFP): Hepatocellular carcinoma, teratoma
      Carcinoembryonic antigen (CEA): Colorectal cancer
      S-100: Melanoma, schwannomas
      Bombesin: Small cell lung carcinoma, gastric cancer

      3. Enzymes
      Alkaline phosphatase (ALP)
      Neuron specific enolase (NSE)

      4. Hormones
      Calcitonin
      Antidiuretic hormone (ADH)
      Human chorionic gonadotropin (hCG)

    • This question is part of the following fields:

      • Haematology & Oncology
      15.1
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  • Question 27 - A 11-year-old boy is referred to you following his seventh course of antibiotics...

    Incorrect

    • A 11-year-old boy is referred to you following his seventh course of antibiotics in the last six years for lower respiratory tract infections. He also has a history of eczema for which he is currently on a topical steroid cream. His full blood count (FBC) report shows: Hb: 13.9 g/dL, Plts: 65 x 10^9/L, WCC: 12.3 x 10^9/L. Which of the following genes should you expect an abnormality in?

      Your Answer: CFTR

      Correct Answer: WASP

      Explanation:

      The combination of frequent infections, eczema, and thrombocytopaenia are characteristic of Wiskott-Aldrich syndrome, which is due to an abnormality in the WASP gene. It is an X-linked recessive disorder that causes primary immunodeficiency owing to a combined B- and T-cell dysfunction.

      The other listed options are:
      1. PKD1: polycystic kidney disease
      2. CFTR: cystic fibrosis
      3. HFE1: haemochromatosis
      4. RET: multiple endocrine neoplasia, Hirschsprung’s disease

    • This question is part of the following fields:

      • Haematology & Oncology
      44.7
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  • Question 28 - According to the Ann Arbor staging system for Hodgkin lymphoma, which one of...

    Correct

    • According to the Ann Arbor staging system for Hodgkin lymphoma, which one of the following would be staged as IIIB?

      Your Answer: Nodes on both sides of diaphragm with night sweats

      Explanation:

      Involvement of lymph nodes on both sides of the diaphragm accompanied by night sweats would be staged as IIIB according to the Ann Arbor staging system for Hodgkin lymphoma (HL).

      HL is a malignant proliferation of lymphocytes characterised by the presence of distinctive giant cells known as Reed-Sternberg cells. It has a bimodal age distribution being most common in the third and seventh decades of life.

      Staging of HL is done according to the Ann Arbor staging system:
      Stage
      I: Single lymph node region (I) or one extra lymphatic site (IE)

      II: Two or more lymph node regions on same side of the diaphragm (II) or local extra lymphatic extension plus one or more lymph node regions on same side of the diaphragm (IIE)

      III: Lymph node regions on both sides of the diaphragm (III) which may be accompanied by local extra lymphatic extension (IIIE)

      IV: Diffuse involvement of one or more extra lymphatic organs or sites

      Suffix
      A: No B symptoms

      B: Presence of at least one of the following: unexplained weight loss >10% baseline during 6 months before staging; recurrent unexplained fever >38°C; recurrent night sweats—poor prognosis.

    • This question is part of the following fields:

      • Haematology & Oncology
      18.7
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  • Question 29 - A 76-year-old lady with known carcinoma of the caecum, was admitted to the...

    Incorrect

    • A 76-year-old lady with known carcinoma of the caecum, was admitted to the hospital for right hemicolectomy. She had a history of osteoarthritis for which she was taking nonsteroidal anti-inflammatory agents (NSAIDs), intermittently. Two years ago, she had a fibroma removed from her right breast as well. The patient was a non-smoker and drank approximately 8 units of alcohol per week. Investigations carried out pre-operatively showed: Hb: 10.8 g/dL, MCV: 75 fL , WCC: 8.4 x10^9/L, Plts: 402 x10^9/L. The surgery remained uncomplicated, and she was given two units of packed red blood cells postoperatively. Three days later, she has now become jaundiced and complains of fatigue. Her blood count now shows: Hb: 7.2 g/dL, MCV: 110 fL, WCC: 9.5 x10^9/L, Plts: 395 x10^9/L. Which of the following is the best investigation to confirm the diagnosis?

      Your Answer: Reticulocyte count

      Correct Answer: Direct Coombs test

      Explanation:

      The direct Coombs test will specifically confirm immune-mediated haemolysis occurring post-transfusion in the aforementioned case.

      There are two types of Coombs test used in immunohematology and immunology:

      1. Direct Coombs test—It confirms autoimmune haemolytic anaemia by detecting antibodies or complement proteins attached to the surface of red blood cells.

      2. Indirect Coombs test—It is used in prenatal testing of pregnant women and in testing prior to a blood transfusion. It detects antibodies floating freely in the blood, against foreign red blood cells.

    • This question is part of the following fields:

      • Haematology & Oncology
      52.1
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  • Question 30 - A 62-year-old man presents with haematuria. Cystoscopy is carried out which reveals transitional...

    Correct

    • A 62-year-old man presents with haematuria. Cystoscopy is carried out which reveals transitional cell carcinoma of the bladder. Occupational exposure to which of the following is a recognised risk factor for bladder cancer?

      Your Answer: Aniline dye

      Explanation:

      The risk factors for bladder cancer are:

      1. Smoking
      2. Exposure to aniline dyes in the printing and textile industry
      3. Exposure to rubber manufacturing
      4. Cyclophosphamides
      5. Schistosomiasis.

    • This question is part of the following fields:

      • Haematology & Oncology
      25.3
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Haematology & Oncology (16/30) 53%
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