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Kumar S, Sharma S. Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome. Oxf Med Case Reports. 2015;2015(3):237-40doi: 10.1093/omcr/omv023.
Kumar, S., & Sharma, S. (2015). Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome. Oxford medical case reports, 2015(3), 237-40. https://doi.org/10.1093/omcr/omv023
Kumar, Sunil, and Sharma, Shruti. "Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome." Oxford medical case reports vol. 2015,3 (2015): 237-40. doi: https://doi.org/10.1093/omcr/omv023
Kumar S, Sharma S. Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome. Oxf Med Case Reports. 2015 Mar 21;2015(3):237-40. doi: 10.1093/omcr/omv023. eCollection 2015 Mar. PMID: 26634133; PMCID: PMC4664845.
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Oxf Med Case Reports. 2015 Mar 21;2015(3):237-40. doi: 10.1093/omcr/omv023. eCollection 2015 Mar.

Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome.

Oxford medical case reports

Sunil Kumar, Shruti Sharma

Affiliations

  1. Department of Neurology , Sawai Man Singh Medical College , Jaipur, Rajasthan , India.
  2. Department of Endocrinology , Apex Hospital , Jaipur, Rajasthan , India.

PMID: 26634133 PMCID: PMC4664845 DOI: 10.1093/omcr/omv023

Abstract

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.

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