Myeloproliferative & plasma cell flashcards
20 high-yield MRCP Part 1 cards from the Intellect Medicos Haematology & Oncology course. Try to answer each question before you open it — then study them properly with spaced repetition, which brings each card back just before you would forget it.
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Which mutation is found in over 95% of patients with polycythaemia vera?
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JAK2 V617F.
Why? This gain-of-function mutation makes JAK2 signalling constantly active, driving red cell, white cell and platelet production.
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What is the first-line treatment of polycythaemia vera?
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Venesection (keeping the haematocrit below 0.45) plus low-dose aspirin.
Why? Lowering the haematocrit reduces hyperviscosity and thrombosis; aspirin reduces thrombotic events.
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What is the mechanism of action of ruxolitinib, used in myelofibrosis and polycythaemia vera?
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JAK1/JAK2 inhibitor.
Why? It damps down overactive JAK–STAT signalling, shrinking the spleen and easing constitutional symptoms.
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What are the first-line laboratory tests when myeloma is suspected?
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Serum protein electrophoresis plus serum free light chain assay (then serum immunofixation if electrophoresis is abnormal).
Why? Together they detect the monoclonal paraprotein, including light-chain-only myeloma that serum electrophoresis can miss.
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What is the first-line imaging for bone disease in suspected myeloma?
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Whole-body MRI (whole-body low-dose CT if MRI is unsuitable).
Why? These detect lytic lesions far more sensitively than a plain-film skeletal survey.
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Myeloma CRAB criteria: calcium above ______ mmol/L, creatinine above 177 µmol/L, haemoglobin below 100 g/L, plus bone lesions.
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2.75
Why? These define myeloma-related end-organ damage and separate myeloma from MGUS and smouldering myeloma.
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Myeloma CRAB criteria: calcium above 2.75 mmol/L, creatinine above ______ µmol/L, haemoglobin below 100 g/L, plus bone lesions.
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177
Why? These define myeloma-related end-organ damage and separate myeloma from MGUS and smouldering myeloma.
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Myeloma CRAB criteria: calcium above 2.75 mmol/L, creatinine above 177 µmol/L, haemoglobin below ______ g/L, plus bone lesions.
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100
Why? These define myeloma-related end-organ damage and separate myeloma from MGUS and smouldering myeloma.
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What is the mechanism of bortezomib, and what is its main dose-limiting toxicity?
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Proteasome inhibitor; peripheral neuropathy.
Why? Blocking the proteasome makes plasma cells accumulate misfolded proteins and die.
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What prophylaxis is needed when myeloma is treated with thalidomide or lenalidomide plus dexamethasone?
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Thromboprophylaxis (aspirin or LMWH according to risk).
Why? These immunomodulatory drugs markedly increase VTE risk, especially combined with steroids.
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A 68-year-old with an IgM paraprotein, lymphadenopathy and splenomegaly presents with blurred vision, headache and "sausage-link" retinal veins. What is the immediate treatment?
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Plasmapheresis (plasma exchange).
Why? This is hyperviscosity from Waldenström macroglobulinaemia; IgM is large and mostly intravascular, so plasma exchange lowers viscosity quickly.
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How does serum erythropoietin help separate polycythaemia vera from secondary polycythaemia?
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EPO is low or normal in PV but raised in secondary polycythaemia.
Why? In PV, red cell production is autonomous (JAK2-driven) and suppresses EPO; in secondary causes, EPO drives the erythrocytosis.
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Name three erythropoietin-secreting tumours that cause secondary polycythaemia.
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Any three of: renal cell carcinoma, hepatocellular carcinoma, cerebellar haemangioblastoma, uterine fibroids.
Why? Inappropriate EPO secretion drives red cell production even though oxygen delivery is normal.
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What is the first-line cytoreductive drug for high-risk polycythaemia vera (e.g. older age or previous thrombosis)?
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Hydroxycarbamide (interferon-alfa is an alternative, e.g. in younger patients or pregnancy).
Why? Cytoreduction lowers the thrombosis risk when venesection and aspirin are not enough.
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Which drug is used in transfusion-dependent lower-risk MDS with isolated del(5q)?
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Lenalidomide.
Why? del(5q) MDS cells are especially sensitive to lenalidomide, which can make patients transfusion-independent.
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Roughly what is the annual risk of MGUS progressing to myeloma or a related disorder?
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About 1% per year.
Why? The risk persists lifelong, so patients are monitored with paraprotein level, FBC, calcium and creatinine.
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Which bisphosphonate is used for myeloma bone disease, and which serious adverse effect requires a dental check before starting?
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Zoledronic acid; osteonecrosis of the jaw.
Why? Bisphosphonates inhibit osteoclast bone resorption, reducing fractures, bone pain and hypercalcaemia.
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Daratumumab targets which antigen, and how does it interfere with blood transfusion testing?
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CD38; it binds CD38 on reagent red cells, making the antibody screen (indirect antiglobulin test) falsely positive with all cells.
Why? CD38 is highly expressed on myeloma plasma cells but is also present at low levels on red cells.
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What is the standard treatment pathway for a fit, transplant-eligible patient with newly diagnosed myeloma?
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Daratumumab-based quadruplet induction (dara-VTd, or dara-VRd with lenalidomide), then high-dose melphalan with autologous stem cell transplant, then lenalidomide-based maintenance.
Why? Autologous transplant deepens and prolongs remission; it is not curative.
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In JAK2-negative essential thrombocythaemia or primary myelofibrosis, which driver gene is most often mutated?
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CALR (calreticulin).
Why? JAK2 V617F, CALR and MPL mutations all switch on JAK–STAT signalling; after JAK2, CALR is the commonest driver in ET and myelofibrosis.