An atypical case of POEMS syndrome with IgG kappa M protein and end stage renal failure

POEMS syndrome is a rare plasma cell dyscrasia which is characterized by small amounts of monoclonal protein, and a multisystem complex manifested by various combinations of polyneuropathy, organomegaly, endocrinopathy and skin changes. Here, we presented an atypical case of POEMS syndrome with IgG...

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Bibliographic Details
Published in:International urology and nephrology 2005-09, Vol.37 (3), p.581-585
Main Authors: Dursun, Belda, Artac, Mehmet, Varan, Halil Ibrahim, Akkaya, Bahar Kilicarslan, Karpuzoglu, Gulten, Suleymanlar, Gultekin
Format: Article
Language:English
Subjects:
Cachexia
Fatal Outcome
Humans
Immunoglobulin G - blood
Kidney Failure, Chronic - blood
Kidney Failure, Chronic - epidemiology
Male
Middle Aged
POEMS Syndrome - blood
POEMS Syndrome - diagnosis
POEMS Syndrome - epidemiology
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Summary:POEMS syndrome is a rare plasma cell dyscrasia which is characterized by small amounts of monoclonal protein, and a multisystem complex manifested by various combinations of polyneuropathy, organomegaly, endocrinopathy and skin changes. Here, we presented an atypical case of POEMS syndrome with IgG kappa monoclonal protein, chronic demyelinating polyneuropathy, hepatosplenomegaly, hypothyroidism, gynecomastia and severe renal impairment. The finding of IgG kappa type of monoclonal protein in our patient was interesting because the majority of cases were reported to have lambda light chain. Also, the absence of typical skin and bone lesions were atypical. Though speculative, these atypical features may account for the unusual presentation of this case. Our patient rapidly progressed to end-stage renal failure and died of cachexia. Renal involvement in POEMS syndrome is rare but may show substantial clinical and pathological variations. Proteinuria, hematuria, renal dysfunction and renal failure requiring hemodialysis can be seen. The pathogenesis of renal dysfunction is unclear. As a conclusion, POEMS syndrome may present with diverse clinicopathologic manifestations. In this syndrome, renal involvement may lead to end stage renal failure and the course may be fatal due to severe polyneuropathy and wasting.
ISSN:0301-1623
1573-2584
DOI:10.1007/s11255-004-0008-0