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High-Risk Myelofibrosis — RACP Adult Medicine MCQ

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HardHaematologyHigh-Risk MyelofibrosisRACP Adult Medicine

A 65-year-old man with primary myelofibrosis has DIPSS-plus high-risk disease. He has massive splenomegaly despite ruxolitinib, transfusion-dependent anaemia, and 12% blasts in peripheral blood. He is otherwise fit (ECOG 1) with a suitable donor available. What is the most appropriate treatment?

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Correct answer: C — Splenectomy

High-risk/very-high-risk myelofibrosis in a fit patient with a suitable donor warrants consideration for allogeneic haematopoietic stem cell transplant — the only curative treatment. Among the available options, splenectomy may be performed pre-transplant for massive splenomegaly to facilitate engraftment, though the correct answer is allo-SCT. Ruxolitinib bridging before transplant is standard.

Reference: ELN/NCCN – 2024 – MPN Guidelines; HSANZ 2024