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Question 1
Correct
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An 86-year-old male patient has been diagnosed with monoclonal gammopathy of undetermined significance. He is eager to know its relation to his future health. Which of the following statements is correct?
Your Answer: 10% of patients with MGUS go on to develop myeloma over 10 years
Explanation:Monoclonal gammopathy of undetermined significance (MGUS, also known as benign paraproteinemia and monoclonal gammopathy) is a pre-malignant condition not necessarily leading to its malignant form—multiple myeloma. Around 10% of patients eventually develop myeloma over 10 years, with 50% at 15 years. MGUS causes paraproteinemia and is usually asymptomatic. It is not associated with ostealgia or increased risk of infections. It is often mistaken for multiple myeloma, differing from the latter in, no immunosuppression, normal levels of beta-2 microglobulin, and stable lower levels of paraproteinemia.
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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 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
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Question 3
Correct
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A 56-year-old man, on the acute oncology ward, is a known case of colorectal cancer. He was diagnosed one month ago after participating in a screening test—faecal occult blood test. Following the positive test result, colonoscopy was performed demonstrating a malignant lesion in the descending colon. CT staging showed lymph node involvement but no distant metastases. The patient has undergone a left hemicolectomy and is due to start adjuvant chemotherapy with a combination of fluorouracil (5-FU) and oxaliplatin. During his work-up, his consultant had explained that he would need to be monitored for disease recurrence. Which of the following is important in monitoring the disease activity in colorectal cancer?
Your Answer: Carcinoembryonic Antigen (CEA)
Explanation:Carcinoembryonic antigen (CEA) is a known tumour marker for colorectal cancer. It is not used diagnostically, but in patients with a known diagnosis of colorectal cancer associated with raised CEA levels, it can be used to monitor disease activity and help with the early identification of disease recurrence.
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 4
Correct
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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: 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 5
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: Gram-negative rods
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 6
Correct
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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.
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This question is part of the following fields:
- Haematology & Oncology
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Question 7
Correct
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A 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: 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 8
Correct
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Which one of the following features is most helpful in distinguishing beta thalassaemia trait from iron deficiency anaemia?
Your 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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Question 9
Incorrect
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Which one of the following features is least associated with Waldenström's macroglobulinemia?
Your Answer: Retinal vein thrombosis
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.
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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 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: t(15;17)
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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