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Question 1
Correct
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A 51-year-old man was admitted with right-sided hemiparesis and right upper motor neurone facial nerve palsy. He was known to be on warfarin for a mitral valve replacement and had been adequately anticoagulated. He was also taking furosemide and had recently been started on St John’s wort for low mood. On examination, his pulse was 90 bpm and regular, and his blood pressure was 150/80 mmHg. Cardiac examination demonstrated normal prosthetic valve sounds with an ejection systolic murmur at the left sternal edge. CT scan showed evidence of a left middle cerebral artery infarction. What is the possible explanation for the presentation?
Your Answer: St John’s wort reduces the activity of warfarin
Explanation:St John’s wort interferes with warfarin by increasing its breakdown and decreasing its effectiveness. This leads to the need for adjustment in the dose of warfarin and careful attention to monitoring if the patient decides to continue with the drug. Ideally, an alternative antidepressant should also be considered.
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This question is part of the following fields:
- Haematology & Oncology
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Question 2
Incorrect
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A 28-year-old man is investigated for lethargy. His full blood count (FBC) report shows: Hb: 8.6 g/dL, Plts: 42 x 10^9/L, WCC: 36.4 x 10^9/L. His blood film report reveals 30% myeloblasts with Auer rods. Given the likely diagnosis, which one of the following is associated with a good prognosis?
Your Answer:
Correct Answer: Translocation between chromosome 15 and 17
Explanation:A translocation between chromosome 15 and 17 is seen in acute promyelocytic leukaemia (APL), which is known to carry a good 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 poor prognosis if:
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.APL is an aggressive form of AML. It is associated with t(15;17) and has a good prognosis. The general age of presentation is less than that in other types of AML (average age is 25 years old). On blood film, abundant Auer rods are seen with myeloperoxidase staining. Thrombocytopaenia or DIC is seen in patients presenting with this disease.
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This question is part of the following fields:
- Haematology & Oncology
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Question 3
Incorrect
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A 35-year-old man who has haemophilia B with associated arthropathy presents with a large swollen right knee, after playing football with his son. He is unable to bear weight on the affected knee joint. What should be the most appropriate first step of treatment?
Your Answer:
Correct Answer: Intravenous factor IX concentrate
Explanation:Haemophilia B (Christmas disease), is the deficiency of clotting factor IX and is inherited in an X-linked recessive pattern. The factor IX level dictates the disease severity and established arthropathy is usually seen in those with severe disease.
The aforementioned patient’s history and presentation is consistent with the development of hemarthrosis. Joint aspiration is not recommended. The treatment, therefore, should be intravenous replacement of the deficient clotting factor with plasma-derived factor IX concentrate.
The other listed options are ruled out because:
1. Joint aspiration is not preferred over the administration of clotting factor as the first step of management.
2. DDAVP (desmopressin) can increase factor VIII levels transiently in those with mild haemophilia A and is useful prior to minor surgical procedures in such patients.
3. Cryoprecipitate is rich in fibrinogen, factor VIII, and von Willebrand factor and is used in the treatment of haemophilia A. -
This question is part of the following fields:
- Haematology & Oncology
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Question 4
Incorrect
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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:
Correct 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
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Question 5
Incorrect
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Out of the following, which malignant tumour has the highest predilection for dissemination to the bones?
Your Answer:
Correct Answer: Prostate
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. ThyroidMost 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 6
Incorrect
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A 34-year-old Nigerian woman who is a known case of sickle cell anaemia presents with fever and worsening of recurrent back pain. There is no history of weight loss or night sweats. The investigations done on her arrival show: Hb: 7.8 g/dL, WCC: 10.1 x10^9/L, Plts: 475 x10^9/L, Reticulocytes: 12%, Serum total bilirubin: 88 μmol/L. What is the most likely diagnosis?
Your Answer:
Correct Answer: Vaso-occlusive event
Explanation:This patient is having vaso-occlusive event/crisis (thrombotic crisis) which is a type of sickle cell crisis. It may be associated with ostealgia.
There is no evidence of an aplastic crisis in this case as the haemoglobin level is reasonable with a good reticulocyte count. Conversely, the haemoglobin is not low enough and reticulocyte count and bilirubin are not high enough for a haemolytic crisis.
Sickle cell anaemia is characterised by periods of good health with intervening crises. The four main types of sickle cell crises are thrombotic crisis (painful or vaso-occlusive crisis), sequestration crisis, aplastic 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). Aplastic crisis is characterised by a sudden fall in haemoglobin without marked reticulocytosis. It usually occurs secondary to parvovirus infection. In haemolytic crisis, a fall in haemoglobin occurs secondary to haemolysis. It is a rare type of sickle cell crisis.
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This question is part of the following fields:
- Haematology & Oncology
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Question 7
Incorrect
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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 lessThis 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—2If 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 8
Incorrect
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A 66-year-old baker presents to the oncology clinic with six-month history of weight loss and anorexia. Tumour marker profile shows an elevated level of bombesin. Out of the following, which is the most likely cancer to account for this result?
Your Answer:
Correct Answer: Small cell lung carcinoma
Explanation:Bombesin is a tumour marker elevated in small cell lung carcinomas, as well as in gastric carcinomas and retinoblastomas.
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 cancer2. 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 cancer3. 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 9
Incorrect
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A 77-year-old female who is a known to have COPD and metastatic lung cancer is admitted with increasing shortness of breath. Following discussion with her family, it is decided to withdraw active treatment including fluids and antibiotics as the admission likely represents a terminal event. Two days after admission, she becomes agitated and restless. What is the most appropriate management for her agitation and confusion?
Your Answer:
Correct Answer: Subcutaneous midazolam
Explanation:Generally, underlying causes of confusion need to be looked for and treated as appropriate, for example, hypercalcaemia, infection, urinary retention, and medication. If specific treatments fail, the following may be tried:
1. First choice: haloperidol
2. Other options: chlorpromazine, levomepromazineIn the terminal phase of the illness, agitation or restlessness is best treated with midazolam.
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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 70-year-old male patient presents to the urology clinic with a one-month history of passing frank haematuria. Flexible cystoscopy shows a mass of the bladder wall and biopsy reveals transitional cell carcinoma. Out of the following, which industry has he most likely worked in?
Your Answer:
Correct Answer: Dyestuffs and pigment manufacture
Explanation:Exposure to aniline dyes is a risk factor for transitional cell carcinoma. Aniline dyes are used in dyestuffs and pigment manufacturing.
The other aforementioned options are ruled out because:
1. Feed production may expose to aflatoxin (hepatocellular carcinoma).2. Being a military personnel may expose to mustard gas (lung cancer).
3. Rubber industry may expose to nitrosamines (oesophageal and gastric cancer).
4. Refrigerant production before 1974 may expose to vinyl chloride (hepatic angiosarcoma).
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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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A 43-year-old man is about to be started on chemotherapy for a high-grade lymphoma. He is given intravenous rasburicase to help lower the risk of tumour lysis syndrome (TLS). What is the mechanism of action of this drug?
Your Answer:
Correct Answer: Converts uric acid to allantoin
Explanation:Rasburicase is a recombinant version of urate oxidase which is an enzyme that metabolizes uric acid to allantoin.
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.
Patients at high risk of TLS should be given IV rasburicase or IV allopurinol immediately prior to and during the first few days of chemotherapy. Allantoin is much more water soluble than uric acid and is therefore more easily excreted by the kidneys. Patients in lower-risk groups should be given oral allopurinol during cycles of chemotherapy in an attempt to avoid the condition.
TLS is graded according to the Cairo-Bishop scoring system as:
1. Laboratory tumour lysis syndrome
2. Clinical tumour lysis syndrome -
This question is part of the following fields:
- Haematology & Oncology
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Question 12
Incorrect
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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:
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.
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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 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 cancer2. 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 cancer3. 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 14
Incorrect
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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.
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This question is part of the following fields:
- Haematology & Oncology
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Question 15
Incorrect
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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.
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This question is part of the following fields:
- Haematology & Oncology
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Question 16
Incorrect
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According to the Ann Arbor staging system for Hodgkin lymphoma, which one of the following would be staged as IIIB?
Your Answer:
Correct 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 symptomsB: 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.
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This question is part of the following fields:
- Haematology & Oncology
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Question 17
Incorrect
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A 8-year-old boy who recently migrated from Nigeria was seen in A&E department with a six-week history of progressive swelling of his jaw, fever, night sweats, and weight loss. His mother reported an episode of sore throat in the past which was treated with antibiotics, but he developed a rash subsequently. Other than that, there was no other significant past medical history. On examination, a painless, nontender 4x3cm mass was found that was fixed and hard. The only other examination finding of note was rubbery symmetrical cervical lymphadenopathy. Which of the following translocation would most likely be found on biopsy karyotyping?
Your Answer:
Correct Answer: t(8;14)
Explanation:Burkitt lymphoma is associated with the c-myc gene translocation, usually t(8;14).
Burkitt lymphoma is a rare high-grade non-Hodgkin lymphoma endemic to west Africa and the mosquito belt. It has a close association with the contraction of Epstein-Barr virus (EBV). Burkitt lymphoma often presents with symmetrical painless lymphadenopathy, systemic B symptoms (fever, sweats, and weight loss), central nervous system involvement, and bone marrow infiltration. Classically in the textbooks, the patient also develops a large jaw tumour.
Other aforementioned options are ruled out because:
1. t(9;22)—Chronic myeloid leukaemia
2. t(15;17)—Acute promyelocytic leukaemia
3. t(14;18)—Follicular Lymphoma
4. t(11;14)—Mantle Cell Lymphoma -
This question is part of the following fields:
- Haematology & Oncology
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Question 18
Incorrect
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A woman is prescribed docetaxel as part of her chemotherapy for breast cancer. What is the mechanism of action of docetaxel?
Your Answer:
Correct Answer: Prevents microtubule disassembly
Explanation:The principal mechanism of action of taxanes (e.g. docetaxel) is the prevention of microtubule disassembly.
Other aforementioned options are ruled out because:
1. Doxorubicin: stabilizes DNA topoisomerase II complex and inhibits DNA and RNA synthesis.
2. Vincristine, vinblastine: inhibits formation of microtubules.
3. Cisplatin: causes cross-linking in DNA.
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This question is part of the following fields:
- Haematology & Oncology
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Question 19
Incorrect
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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.
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This question is part of the following fields:
- Haematology & Oncology
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Question 20
Incorrect
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A 60-year-old woman presents to the oncology clinic with a general feeling of being unwell and temperature of 38.1°C. She is a known case of neuroendocrine cancer of the cervix, treated with carboplatin and etoposide. Her last treatment was eight days ago. Blood cultures are taken and she is started on neutropenic sepsis protocol. What will gram-staining of the blood cultures most likely show?
Your Answer:
Correct Answer: Gram-positive cocci
Explanation:Gram-staining of the blood cultures of this patient will show gram-positive cocci. Gram-negative bacilli used to be the most common pathogen isolated in neutropenic sepsis, but currently, the most common pathogens are gram-positive organisms. Staphylococcus epidermidis is the most frequent causative agent, and following this are other staphylococci and streptococci species.
Neutropenic sepsis is a relatively common complication of cancer therapy—usually chemotherapy. It most commonly occurs 7-14 days after the treatment and is usually 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 includes starting empirical antibiotic therapy (piperacillin with tazobactam—Tazocin) immediately. Following this initial treatment, the patient is usually assessed by a specialist and risk-stratified to see if outpatient treatment may be possible. However, if the patient remains febrile and unwell after 48 hours, an alternative antibiotic such as meropenem is often prescribed with or without vancomycin. If patient is still not responding after 4-6 days, then an antifungal, such as amphotericin B, is started after carrying out investigations (e.g. HRCT and Aspergillus PCR) to determine the likelihood of systemic fungal infection.
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This question is part of the following fields:
- Haematology & Oncology
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Question 21
Incorrect
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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 22
Incorrect
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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.
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This question is part of the following fields:
- Haematology & Oncology
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Question 23
Incorrect
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A 23-year-old woman presents with lethargy. Her blood picture shows: Hb: 10.4 g/dL, Plts: 278 x 10^9/L, WCC: 6.3 x 10^9/L, MCV: 68 fL. Blood film: Microcytic hypochromic RBCs, marked anisocytosis and basophilic stippling noted, HbA2: 3.9%. What is the most likely diagnosis?
Your Answer:
Correct Answer: Beta-thalassaemia trait
Explanation:Thalassaemias are a group of genetic disorders characterised by decreased production of either alpha or beta chains of haemoglobin (Hb). Beta thalassaemia trait is an autosomal recessive condition in which a disproportionate hypochromic, microcytic anaemia occurs—microcytosis is disproportionate to the Hb level. It is usually asymptomatic.
Microcytic anaemia in a female should raise the possibility of either gastrointestinal blood loss or menorrhagia. However, there is no history to suggest this in the aforementioned patient. This, combined with characteristic disproportionate microcytosis and raised HbA2 levels ( >3.5%), point towards beta thalassaemia trait.
Basophilic stippling is also seen in lead poisoning but the raised HbA2 levels cannot be explained in such a case.
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This question is part of the following fields:
- Haematology & Oncology
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Question 24
Incorrect
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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:
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.
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This question is part of the following fields:
- Haematology & Oncology
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Question 25
Incorrect
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A 50-year-old woman is investigated for weight loss and anaemia. Clinical examination reveals splenomegaly associated with pale conjunctivae. Her full blood count (FBC) report shows: Hb: 10.9 g/dL, Plts: 702 x 10^9/L, WCC: 56.6 x 10^9/L. Moreover, all stages of granulocyte maturation are seen on her blood film. Given the likely diagnosis, what should be the most appropriate treatment?
Your Answer:
Correct Answer: Imatinib
Explanation:This patient is a case of chronic myeloid leukaemia (CML) and should be started on imatinib as the first-line drug of choice.
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This question is part of the following fields:
- Haematology & Oncology
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Question 26
Incorrect
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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.
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This question is part of the following fields:
- Haematology & Oncology
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Question 27
Incorrect
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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 28
Incorrect
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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:
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 departmentA 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
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This question is part of the following fields:
- Haematology & Oncology
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Question 29
Incorrect
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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:
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 = 40mgThe 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.
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This question is part of the following fields:
- Haematology & Oncology
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Question 30
Incorrect
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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.
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This question is part of the following fields:
- Haematology & Oncology
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