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Symptomatic Myelofibrosis — RACP Adult Medicine MCQ

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ModerateHaematologySymptomatic MyelofibrosisRACP Adult Medicine

A 55-year-old man with a 15 cm spleen (palpable 8 cm below costal margin), Hb 88 g/L, WCC 3.2 × 10⁹/L, and platelets 75 × 10⁹/L. Blood film shows teardrop cells and nucleated red blood cells. He has constitutional symptoms (fever, night sweats, weight loss). JAK2 V617F is positive. Bone marrow biopsy shows grade 3 reticulin fibrosis. What is the most appropriate treatment for his symptomatic splenomegaly and constitutional symptoms?

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Correct answer: D — Ruxolitinib (JAK inhibitor)

Ruxolitinib (JAK1/2 inhibitor) is first-line for symptomatic splenomegaly and constitutional symptoms in myelofibrosis (COMFORT-I and COMFORT-II trials). It significantly reduces spleen size and improves quality of life. It does not significantly alter disease course or reticulin fibrosis. Allogeneic SCT remains the only curative option for eligible patients.

Reference: ELN/NCCN – 2024 – MPN Guidelines; COMFORT Trials