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Primary Myelofibrosis — RACP Adult Medicine MCQ

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ModerateHaematologyPrimary MyelofibrosisRACP Adult Medicine

A 55-year-old man presents with progressive breathlessness and early satiety. Splenomegaly extends 15 cm below the costal margin. His blood film shows leucoerythroblastic features with teardrop poikilocytes. What is the most likely diagnosis?

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Correct answer: C — Primary myelofibrosis

Massive splenomegaly with a leucoerythroblastic blood film (nucleated red blood cells and immature myeloid precursors) and teardrop poikilocytes (dacrocytes) is the hallmark of primary myelofibrosis. Bone marrow biopsy shows fibrosis with reticulin/collagen. JAK2, CALR, or MPL mutations are present in ~90% of cases. The DIPSS-plus score guides prognosis and transplant eligibility.

Reference: eTG – 2025 – Haematology; WHO – 2022 – MPN Classification