Polyneuropathy, Organomegaly, Endocrinopathy, M-Protein, and Skin Changes (POEMS) Syndrome With Characteristic Muscle Biopsy Findings and Without M-Protein.
Josephine Yalovitser, Muhannad Seyam, John DeWitt et al.
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Case in brief
A 63-year-old woman referred with presumed CIDP had not responded to IVIG or steroids. Her presentation included marked weight loss, papilledema, skin changes, thrombocytosis, thromboembolisms, hemi-diaphragmatic paralysis, and organomegaly with lymphadenopathy; bone marrow and lymph node biopsies were negative. Repeat electrodiagnostic testing, elevated VEGF and prolactin, and nerve and muscle biopsy findings supported POEMS syndrome despite no detectable M-protein. Biopsies showed endomysial edema and angiogenesis. She improved significantly after treatment with lenalidomide and dexamethasone.
What made this case unusual
POEMS was diagnosed despite the absence of detectable M-protein; muscle biopsy showed endomysial edema and angiogenesis, including characteristic vascular proliferation.
Diagnostic / clinical pearl
In presumed CIDP that is refractory to treatment, accompanying systemic features should prompt consideration of POEMS even without M-protein. VEGF measurement and nerve or muscle biopsy may support diagnosis when clinical suspicion remains high.