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Thrombotic thrombocytopenic purpura — ABIM Board MCQ

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HardHematology/OncologyThrombotic thrombocytopenic purpuraABIM Board

A 49-year-old man has progressive demyelinating polyneuropathy, splenomegaly, edema, thrombocytosis, hyperpigmentation, and a small IgA-lambda monoclonal protein. Bone imaging shows a sclerotic pelvic lesion. Which additional test most strongly supports the suspected unifying diagnosis?

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Correct answer: A — Measure plasma vascular endothelial growth factor

The best answer is “Measure plasma vascular endothelial growth factor”. The combination of demyelinating neuropathy, lambda plasma-cell disorder, sclerotic bone lesion, organomegaly, volume overload, thrombocytosis, and skin change suggests POEMS syndrome. Elevated plasma VEGF is an additional major criterion and strongly supports the diagnosis. The alternatives may evaluate narrower neuropathy or systemic syndromes but do not integrate this characteristic multisystem plasma-cell phenotype.

Reference: American Society of Hematology: Neuropathy and Splenomegaly: https://www.hematology.org/education/trainees/fellows/case-studies/neuropathy-splenomegaly