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POEMS Syndrome — RACP Adult Medicine MCQ

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HardHaematologyPOEMS SyndromeRACP Adult Medicine

A 50-year-old man presents with progressive cerebellar ataxia, peripheral neuropathy, and skin hyperpigmentation. He has bilateral lower limb oedema. Serum protein electrophoresis shows a lambda light chain paraprotein. His VEGF level is markedly elevated. Bone survey shows sclerotic lesions. What is the diagnosis?

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Correct answer: E — Waldenström macroglobulinaemia

POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes) is a paraneoplastic syndrome associated with a plasma cell dyscrasia (usually lambda light chain). Markedly elevated VEGF is the hallmark and drives many of the clinical features. Sclerotic (not lytic) bone lesions distinguish it from myeloma. Diagnosis requires both mandatory criteria (polyneuropathy + clonal PCD) plus one major and one minor criterion. Treatment targets the underlying plasma cell clone (radiation for localised, chemotherapy for disseminated).

Reference: eTG – 2025 – Haematology; Dispenzieri – 2019 – POEMS Syndrome Review