An atypical presentation of POEMS syndrome with IgG kappa type M protein and normal VEGF level: Case report and review of literature.

POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. We present an atypical case of POEMS syndrome with subclinical polyneuropathy, splenomegaly, hypogonadism, and myeloma of immunoglobulin G kappa type. The typical osteosclerotic lesions and skin lesions were absent, and vascular endothelial growth factor (VEGF) level was normal. Our patient also had chronic kidney disease due to focal segmental glomerulosclerosis. The patient died due to multisystem complications nine months after presenting with acute on chronic renal failure. POEMS syndrome is an uncommon, potentially fatal paraneoplastic syndrome presenting with plasma cell neoplasm and multisystem involvement. Despite the normal VEGF level, our patient was diagnosed to have possible POEMS syndrome due to other clinical manifestations. It may be reasonable to expand the diagnostic criteria of POEMS syndrome if further atypical cases are reported in the future.