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2015 ; 2015
(3
): 237-40
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Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS
syndrome): a paraneoplastic syndrome
#MMPMID26634133
Kumar S
; Sharma S
Oxf Med Case Reports
2015[Mar]; 2015
(3
): 237-40
PMID26634133
show ga
POEMS syndrome (Crow-Fukase syndrome) is a rare paraneoplastic disorder. It is
characterized by peripheral neuropathy, elevated vascular endothelial growth
factors (VEGFs), monoclonal gammopathy, sclerotic bone lesions and Castleman
disease. Other important clinical features are organomegaly, edema, ascites,
papilledema, endocrinopathy, skin changes and thrombocytosis. A high index of
suspicion, a detailed clinical history and examination followed by appropriate
laboratory investigations like VEGF level, radiological skeletal survey and bone
marrow biopsy are required to diagnose POEMS syndrome. We report a case of POEMS
syndrome who presented with insidious onset, progressive sensorimotor
polyneuropathy, pedal edema, ascites, hepatomegaly, skin changes and
hypothyroidism. X-ray of the pelvis showed osteosclerotic lesions.
Immunoelectrophoresis using the immunofixation method revealed lambda chain
monoclonal gammopathy. The patient was given radiotherapy, followed by a
combination therapy of melphalan and dexamethasone. We emphasize the importance
of recognizing a challenging diagnosis of a rare disease, which is shown to be
treatment responsive.