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

DURSUN B., ARTAC M., VARAN H. I., Akkaya B., Karpuzoğlu G., Suleymanlar G.

INTERNATIONAL UROLOGY AND NEPHROLOGY, vol.37, no.3, pp.581-585, 2005 (SCI-Expanded, Scopus)

  • Publication Type: Article / Article
  • Volume: 37 Issue: 3
  • Publication Date: 2005
  • Doi Number: 10.1007/s11255-004-0008-0
  • Journal Name: INTERNATIONAL UROLOGY AND NEPHROLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.581-585
  • Keywords: IgG kappa monoclonal protein, POEMS syndrome, renal failure, PLASMA-CELL DYSCRASIA, ENDOTHELIAL GROWTH-FACTOR, CROW-FUKASE SYNDROME, PROINFLAMMATORY CYTOKINES, CASTLEMANS-DISEASE, MULTIPLE-MYELOMA, POLYNEUROPATHY, ENDOCRINOPATHY, COAGULOPATHY, INVOLVEMENT
  • Akdeniz University Affiliated: Yes

Abstract

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.