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  • Question 1 - A 34-year-old man who is HIV positive has started treatment for Burkitt lymphoma....

    Incorrect

    • 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: Haemorrhagic cystitis leading to acute renal failure

      Correct 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
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  • Question 2 - A 49-year-old female is admitted to the hospital with shortness of breath and...

    Incorrect

    • 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:

      Correct 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
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  • Question 3 - 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:

      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
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  • Question 4 - A 45-year-old male patient who was initially investigated for having an abdominal mass...

    Incorrect

    • A 45-year-old male patient who was initially investigated for having an abdominal mass is diagnosed as having Burkitt lymphoma. He is due to start chemotherapy today. Which one of the following should be given prior to his chemotherapy in order to reduce the risk of tumour lysis syndrome?

      Your Answer:

      Correct Answer: Rasburicase

      Explanation:

      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.

      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.

      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
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  • Question 5 - A 35-year-old woman is referred to the oncology clinic by a general surgeon....

    Incorrect

    • A 35-year-old woman is referred to the oncology clinic by a general surgeon. She has undergone mastectomy for carcinoma of the right breast. Out of the following, which factor is associated with a poor prognosis in patients with breast cancer?

      Your Answer:

      Correct Answer: Young age

      Explanation:

      Poor prognostic factors for breast cancer include:
      1. Young age (<40 years)
      2. Premenopausal at the time of diagnosis
      3. Increased tumour size
      4. High-grade tumour
      5. Oestrogen and progesterone receptor-negative tumour
      6. Positive lymph node status

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 6 - A 52-year-old woman is diagnosed as having acute myeloid leukaemia. What is the...

    Incorrect

    • A 52-year-old woman is diagnosed as having acute myeloid leukaemia. What is the single most important test in determining her prognosis?

      Your Answer:

      Correct Answer: Cytogenetics

      Explanation:

      All of the aforementioned options may be important however cytogenetics, for detecting chromosomal abnormalities, is the single most important test to determine her disease prognosis.

      Acute myeloid leukaemia (AML) is the acute expansion of the myeloid stem line, which may occur as a primary disease or follow the secondary transformation of a myeloproliferative disorder. It is more common over the age of 45 and is characterized by signs and symptoms largely related to bone marrow failure such as anaemia (pallor, lethargy), frequent infections due to neutropenia (although the total leucocyte count may be very high), thrombocytopaenia (bleeding), ostealgia, and splenomegaly.

      The disease has a poor prognosis if:
      1. Age of the patient >60 years
      2. >20% blasts seen after the first course of chemotherapy
      3. Chromosomal aberration with deletion of part of chromosome 5 or 7.

      Acute promyelocytic leukaemia (APL) is an aggressive form of AML.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 7 - A 58-year-old female patient is being investigated for breathlessness, cough, and severe weight...

    Incorrect

    • A 58-year-old female patient is being investigated for breathlessness, cough, and severe weight loss. On the medical ward round, her CXR is reviewed showing hilar lymphadenopathy and multiple peripheral lung metastases. Which of the following tumours is least likely to be the underlying cause of this lung appearance?

      Your Answer:

      Correct Answer: Brain

      Explanation:

      All of the aforementioned listed tumours, except brain tumours, can metastasise to lungs and produce the typical CXR picture consisting of hilar lymphadenopathy with either diffuse multinodular shadows resembling miliary disease or multiple large well-defined masses (canon balls). Occasionally, cavitation or calcification may also be seen.

      Most brain tumours, however, do not metastasise. Some, derived form neural elements, do so but in these cases, intraparenchymal metastases generally precede distant haematogenous spread.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 8 - A 32-year-old woman, with a history of infertility, presented with post-operative bleeding from...

    Incorrect

    • A 32-year-old woman, with a history of infertility, presented with post-operative bleeding from her abdominal wound. Her full blood count (FBC) and blood film showed hyperleukocytosis and the presence of promyelocytes, along with the following: Hb: 9.2g/dL, Plts: 932 x 10^9/L, INR: 1.4 (Coagulation profile). What should be the next step of management?

      Your Answer:

      Correct Answer: Give fresh frozen plasma

      Explanation:

      The patient has acute promyelocytic leukaemia (APML) with associated disseminated intravascular coagulation (DIC). Although
      the platelet count is high, platelet function is ineffective.

      Patients may present, as in this case, with severe bleeding, and the most appropriate emergency treatment would be administration of fresh frozen plasma (FFP).

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 9 - A 28-year-old woman with a history of recurrent pulmonary emboli (PE) has been...

    Incorrect

    • A 28-year-old woman with a history of recurrent pulmonary emboli (PE) has been identified as having factor V Leiden. How does this particular inherited thrombophilia increase her risk of venous thromboembolic events?

      Your Answer:

      Correct Answer: Activated factor V is inactivated much more slowly by activated protein C

      Explanation:

      In patients with factor V Leiden, inactivation of the active factor V (a clotting factor) by active protein C occurs 10x more slowly than normal. Therefore, this condition is also called activated protein C resistance.

      Factor V Leiden is the most commonly inherited thrombophilia, being present in around 5% of the UK’s population. It occurs due to gain-of-function mutation in the Factor V Leiden protein.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 10 - Which of the following is the main mechanism of vitamin B12 absorption? ...

    Incorrect

    • Which of the following is the main mechanism of vitamin B12 absorption?

      Your Answer:

      Correct Answer: Active absorption in the terminal ileum

      Explanation:

      Vitamin B12 is mostly used in the body for the development of red blood cells and maintenance of the nervous system. It is actively absorbed in the terminal ileum after binding to the intrinsic factor (IF) which is secreted from the parietal cells of stomach. Moreover, a small amount is also passively absorbed without being bound to IF.

      Vitamin B12 deficiency is characterised by sore tongue and mouth, mood disturbances, ataxia, and macrocytic anaemia. Its causes include poor diet, impaired absorption (due to disorders of terminal ileum), pernicious anaemia, and post-gastrectomy. While managing this condition, if the person is also deficient in folic acid, then it is important to treat the B12 deficiency first in order to avoid precipitating subacute combined degeneration of the cord.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 11 - A 60-year-old man with a history of chronic lymphocytic leukaemia is admitted to...

    Incorrect

    • A 60-year-old man with a history of chronic lymphocytic leukaemia is admitted to the acute medical unit for pneumonia. This is his fourth admission for pneumonia in the past six months. Which of the following factors is most likely to be responsible?

      Your Answer:

      Correct Answer: Hypogammaglobulinaemia

      Explanation:

      Hypogammaglobulinemia is a complication of chronic lymphocytic leukaemia (CLL) that leads to recurrent infections.

      CLL is a type of cancer caused by monoclonal proliferation of well-differentiated lymphocytes, typically B cells (99%). Onset of the disease is usually asymptomatic and later constitutes anorexia, weight loss, bleeding, and recurrent infections. Lymphadenopathy is more marked in CLL than in chronic myelogenous leukaemia (CML).

      Investigations to diagnose CLL include blood film and immunophenotyping. Smudge cells (also known as smear cells) seen on the blood film point towards CLL. Complications of the disease include hypogammaglobulinemia leading to recurrent infections, autoimmune haemolytic anaemia in 10–15% of the patients, and transformation to high-grade lymphoma (Richter’s transformation).

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 12 - A 28-year-old woman presents with easy bruising. She has no history of mucosal...

    Incorrect

    • A 28-year-old woman presents with easy bruising. She has no history of mucosal bleeding and is generally well apart from occasional diarrhoea. She has previously attended a psychiatric unit for self-harming behaviour and is now brought in by her mother having consumed a number of pills. Her mother has had recurrent venous thromboses, but there is no family history of a bleeding disorder. Her full blood count (FBC) is normal, but her coagulation profile shows: Activated partial thromboplastin time (APTT): 60 secs (28–38 secs), Prothrombin time (PT): no clot after 120 secs (10–14 secs), Fibrinogen: 3.6 g/L (2–4 g/L). What is the most likely explanation?

      Your Answer:

      Correct Answer: Warfarin overdose

      Explanation:

      Warfarin inhibits the vitamin K-dependent procoagulants II, VII, IX, and X as well as anticoagulant protein C and S. It is highly protein-bound and can be displaced by a wide variety of drugs. It has a half-life of 36–48 hours.

      Bleeding is the major side effect. Easy bruising, as seen in this case, is commonly seen in patients of warfarin overdose. Grossly prolonged PT and lesser increase in APTT may be seen in such cases.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 13 - A 53-year-old woman presents with a painless lump in the left breast associated...

    Incorrect

    • 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:

      Correct 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 14 - 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:

      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 15 - 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:

      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 16 - 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:

      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
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  • Question 17 - An 82-year-old man is reviewed in the haematology clinic. He has been referred...

    Incorrect

    • An 82-year-old man is reviewed in the haematology clinic. He has been referred due to weight loss, lethargy, and a significantly elevated IgM level. His recent blood results show: Hb: 13.8 g/dL, Plts: 127 x 10^9/L, ESR: 45 mm/hr, IgM: 2150 mg/dL (50-330 mg/dL). Given the probable diagnosis, which one of the following complications is he most likely to develop?

      Your Answer:

      Correct Answer: Hyperviscosity syndrome

      Explanation:

      The patient is most likely suffering from Waldenström’s macroglobulinemia in which IgM paraproteinemia is found. Hyperviscosity syndrome can occur in the patients accounting for 10–15% of the cases.

      Waldenström’s macroglobulinemia (also called lymphoplasmacytic lymphoma) is an uncommon type of non-Hodgkin lymphoma seen in older people. It is a lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein. Its features include monoclonal IgM paraproteinemia; hyperviscosity syndrome leading to bilateral central retinal vein occlusion (CRVO) and hence, visual disturbances; weight loss and lethargy; hepatosplenomegaly and lymphadenopathy; and cryoglobulinemia. It is not, however, associated with bone pain.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 18 - A 65-year-old man is referred to the oncology clinic with progressively worsening lower...

    Incorrect

    • A 65-year-old man is referred to the oncology clinic with progressively worsening lower back pain for the last three months and history of weight loss for the past eight months. MRI lumbar spine confirms the suspicion of bone metastasis. What is the most likely primary tumour?

      Your Answer:

      Correct Answer: Prostate carcinoma

      Explanation:

      Prostate cancer is the most common primary tumour that metastasises to the bone.

      Most common tumours causing bone metastasis (in descending order):
      1. Prostate (32%)
      2. Breast (22%)
      3. Kidneys (16%)
      4. Lungs
      5. Thyroid

      Most common sites of bone metastasis (in descending order):
      1. Spine
      2. Pelvis
      3. Ribs
      4. Skull
      5. Long bones

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 19 - A 46-year-old man presents with a swollen, red, and painful left calf. He...

    Incorrect

    • A 46-year-old man presents with a swollen, red, and painful left calf. He does not have a history of any recent surgery or a long-haul flight. He is generally fit and well and takes no regular medication other than propranolol for migraine prophylaxis. There is also no history of venous thromboembolism (VTE) in his family. The patient is referred to the deep vein thrombosis (DVT) clinic where he is diagnosed with a proximal DVT in his left calf. Following the diagnosis, he is started on low-molecular-weight heparin (LMWH) whilst awaiting review by the warfarin clinic. Other than commencing warfarin, what further action, if any, is required?

      Your Answer:

      Correct Answer: Investigate for underlying malignancy + check antiphospholipid antibodies

      Explanation:

      CXR, blood, and urine tests should be carried out initially to exclude an underlying malignancy. If these are normal, a CT scan of abdomen and pelvis should be arranged as the patient’s age is >40 years. Antiphospholipid antibodies should also be checked for the first unprovoked DVT/PE. There is no history, however, to support an inherited thrombophilia.

      The National Institute for Health and Care Excellence (NICE) published guidelines in 2012 for the investigation and management of DVT. If a patient is suspected of having DVT, a two-level DVT Wells score should be used:

      DVT likely: 2 points or more
      DVT unlikely: 1 point or less

      This system of points is based on the following clinical features:
      1. Active cancer (treatment ongoing, within six months, or palliative)—1
      2. Paralysis, paresis, or recent plaster immobilisation of the lower extremities—1
      3. Recently bedridden for three days or more, or major surgery within 12 weeks requiring general or regional anaesthesia—1
      4. Localised tenderness along the distribution of the deep venous system—1
      5. Entire leg swollen—1
      6. Calf swelling at least three cms larger than the asymptomatic side—1
      7. Pitting oedema confined to the symptomatic leg—1
      8. Collateral superficial veins (non-varicose)—1
      9. Previously documented DVT—1
      10. An alternative diagnosis is at least as likely as DVT—2

      If two points or more—DVT is ‘likely’
      If one point or less—DVT is ‘unlikely’

      Management

      1. LMWH or fondaparinux should be given initially after a DVT is diagnosed.
      2. A vitamin K antagonist such as warfarin should be given within 24 hours of the diagnosis.
      3. LMWH or fondaparinux should be continued for at least five days or until the international normalised ratio (INR) is 2.0 or above for at least 24 hours. LMWH or fondaparinux is given at the same time as warfarin until the INR is in the therapeutic range.
      4. Warfarin should be continued for at least three months. At three months, clinicians should assess the risks and benefits of extending the treatment.
      5. Consider extending warfarin beyond three months for patients with unprovoked proximal DVT if their risk of VTE recurrence is high and there is no additional risk of major bleeding. This essentially means that if there is no obvious cause or provoking factor (surgery, trauma, significant immobility, etc.), it may be implied that the patient has a tendency to thrombose and should be given treatment longer than the normal of three months. In practice, most clinicians give six months of warfarin for patients with an unprovoked DVT/PE.
      6. For patients with active cancer, LMWH should be used for six months.

      As both malignancy and thrombophilia are obvious risk factors for DVT, therefore, all patients with unprovoked DVT/PE who are not already known to have cancer should undergo the following investigations:
      1. Physical examination (guided by the patient’s full history)
      2. Chest X-ray
      3. Blood tests (full blood count, serum calcium, and liver function tests) and urinalysis
      4. Testing for antiphospholipid antibodies
      5. Testing for hereditary thrombophilia in patients who have had unprovoked DVT/PE and have a first-degree relative who has a history of DVT/PE.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 20 - Which of the following features is characteristic of acute intermittent porphyria? ...

    Incorrect

    • Which of the following features is characteristic of acute intermittent porphyria?

      Your Answer:

      Correct Answer: Increased urinary porphobilinogen between acute attacks

      Explanation:

      Urinary porphobilinogen is increased between attacks of acute intermittent porphyria (AIP) and even more so, between acute attacks.

      AIP is a rare autosomal dominant condition caused by a defect in porphobilinogen deaminase, an enzyme involved in the biosynthesis of haem. This results in the toxic accumulation of delta-aminolaevulinic acid and porphobilinogen.

      Abdominal and neuropsychiatric symptoms are characteristic of AIP especially in people between the ages of 20–40 years. The disease is more common in females than in males (5:1). Major signs and symptoms of AIP include abdominal pain, vomiting, motor neuropathy, hypertension, tachycardia, and depression.

      Diagnosis:
      1. Urine turns deep red on standing (classical picture of AIP)
      2. Raised urinary porphobilinogen (elevated between attacks and to a greater extent, between acute attacks)
      3. Raised serum levels of delta-aminolaevulinic acid and porphobilinogen
      4. Assay of red blood cells for porphobilinogen deaminase

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 21 - A 46-year-old nurse presents with a short history of epistaxis and bleeding gums....

    Incorrect

    • A 46-year-old nurse presents with a short history of epistaxis and bleeding gums. Her complete blood count, coagulation profile, and blood film are requested. The results are as follows: Hb: 8.6 g/dL, WCC: 2.3 x 10^9/L, Plts: 18 x 10^9/L, Coagulation profile: deranged, Blood film: bilobed large mononuclear cells. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Acute myeloid leukaemia

      Explanation:

      This is a picture of bone marrow failure secondary to acute myeloid leukaemia (AML). AML is the acute expansion of the myeloid stem line, which may occur as a primary disease or follow the secondary transformation of a myeloproliferative disorder. It is more common over the age of 45 and is characterized by signs and symptoms largely related to bone marrow failure such as anaemia (pallor, lethargy), frequent infections due to neutropenia (although the total leucocyte count may be very high), thrombocytopaenia (bleeding), ostealgia, and splenomegaly.

      The disease has poor prognosis if:
      1. Age of the patient >60 years
      2. >20% blasts seen after the first course of chemotherapy
      3. Chromosomal aberration with deletion of part of chromosome 5 or 7.

      Acute promyelocytic leukaemia (APL) is an aggressive form of AML.

      Other listed options are ruled out because:
      1. Von Willebrand disease: may present with epistaxis and bleeding gums in severe cases but rarely with abnormalities on blood results.

      2. Acute lymphoblastic leukaemia: mostly seen in children.

      3. Lymphoma: usually presents with rubbery enlargement of lymph nodes.

      4. Warfarin overdose: no bilobed large mononuclear cells seen on blood film.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 22 - A 65-year-old male patient is found to have an elevated serum paraprotein level...

    Incorrect

    • A 65-year-old male patient is found to have an elevated serum paraprotein level of 35g/L. Bone marrow aspirate reveals 32% monoclonal plasma cell infiltrate. He has no evidence of anaemia, renal impairment, hypercalcaemia, or lytic lesions. What should be the next step in management?

      Your Answer:

      Correct Answer: Observe and monitor

      Explanation:

      The patient is asymptomatic but matches the diagnostic criteria for multiple myeloma (MM). Therefore, the underlying diagnosis of this condition is smouldering multiple myeloma (SMM). SMM is an early precursor to MM. Its treatment is typically to watch and wait.

      MM is a neoplasm of the bone marrow plasma cells. Peak incidence is in patients aged 60–70 years.

      Clinical features of MM include:
      1. Ostealgia, osteoporosis, pathological fractures (typically vertebral), and osteolytic lesions
      2. Lethargy
      3. Infections
      4. Hypercalcaemia
      5. Renal failure
      6. Other features: amyloidosis e.g. macroglossia, carpal tunnel syndrome; neuropathy; hyperviscosity

      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

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      • Haematology & Oncology
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  • Question 23 - A 52-year-old woman who is a known case of breast cancer is being...

    Incorrect

    • A 52-year-old woman who is a known case of breast cancer is being reviewed six months after she had a mastectomy following the diagnosis. Which of the following tumour markers is the most useful in monitoring her disease?

      Your Answer:

      Correct Answer: CA 15-3

      Explanation:

      Tumour marker CA 15-3 is mostly associated with breast cancer.

      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
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  • Question 24 - A 52-year-old woman is being reviewed in the oncology clinic following debulking surgery...

    Incorrect

    • 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:

      Correct 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
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  • Question 25 - Which of the following is not a recognised feature of methemoglobinemia? ...

    Incorrect

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

      Your Answer:

      Correct 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.

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      • Haematology & Oncology
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  • Question 26 - Which one of the following features is least associated with Waldenström's macroglobulinemia? ...

    Incorrect

    • Which one of the following features is least associated with Waldenström's macroglobulinemia?

      Your Answer:

      Correct Answer: Bone pain

      Explanation:

      Waldenström’s macroglobulinemia (also called lymphoplasmacytic lymphoma) is an uncommon type of non-Hodgkin lymphoma seen in older people. It is a lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein. Its features include weight loss and lethargy; monoclonal IgM paraproteinemia; hyperviscosity syndrome leading to bilateral central retinal vein occlusion (CRVO) and hence, visual disturbances; hepatosplenomegaly and lymphadenopathy; and cryoglobulinemia. It is not, however, associated with bone pain.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 27 - Which one of the following features is most helpful in distinguishing beta thalassaemia...

    Incorrect

    • Which one of the following features is most helpful in distinguishing beta thalassaemia trait from iron deficiency anaemia?

      Your Answer:

      Correct Answer: Haemoglobin A2 levels

      Explanation:

      Elevated haemoglobin A2 level is seen in beta thalassaemia trait, whereas, it is typically low in iron deficiency anaemia unless the patient has received a recent blood transfusion.

      Low mean corpuscular volume (MCV) and reduced haematocrit (Ht) are encountered in both conditions. Peripheral blood smear is grossly abnormal in both beta thalassaemia and severe iron deficiency anaemia, showing bizarre morphology, target cells, and a small number of nucleated red blood cells.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 28 - A 25-year-old man having sickle cell anaemia presents with headache, lethargy, and pallor....

    Incorrect

    • A 25-year-old man having sickle cell anaemia presents with headache, lethargy, and pallor. His blood count shows: Haemoglobin: 4.6 g/dL, Reticulocytes: 3%. Infection with parvovirus is suspected. Out of the following, what is the most likely diagnosis?

      Your Answer:

      Correct Answer: Aplastic crisis

      Explanation:

      Sickle cell anaemia is characterised by periods of good health with intervening crises. One of the main types is aplastic crisis characterised by a sudden fall in haemoglobin without marked reticulocytosis (3%—in this case—is just above the normal range). It usually occurs secondary to parvovirus infection.

      The other main types of sickle cell crises are thrombotic crisis (painful or vaso-occlusive crisis), sequestration crisis, and haemolytic crisis. Thrombotic crisis is precipitated by infection, dehydration, alcohol, change in temperature, and deoxygenation. Sequestration crisis is characterised by acute chest syndrome (i.e. fever, dyspnoea, chest/rib pain, low pO2, and pulmonary infiltrates). In haemolytic crisis, fall in haemoglobin occurs secondary to haemolysis. It is a rare type of sickle cell crises.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 29 - A 40-year-old female is receiving a course of chemotherapy for breast cancer. She...

    Incorrect

    • A 40-year-old female is receiving a course of chemotherapy for breast cancer. She is, however, experiencing troublesome vomiting which is not responding to domperidone. Which of the following is the most appropriate next step of management?

      Your Answer:

      Correct Answer: Add a 5-HT3 antagonist

      Explanation:

      Nausea and vomiting are the common side effects of chemotherapy. Risk factors for the development of these symptoms include age<50 years, anxiety, concurrent use of opioids, and the type of chemotherapy administered. For patients at low risk of these symptoms, drugs such as metoclopramide may be used. For high-risk patients, however, 5-HT3 receptor antagonists such as ondansetron are often effective, especially if combined with dexamethasone.

    • This question is part of the following fields:

      • Haematology & Oncology
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  • Question 30 - A 52-year-old man is referred to the oncology clinic by his GP. He...

    Incorrect

    • 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:

      Correct 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
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