Endocrinopathy in polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome

摘要

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome due to underlying plasma cell disorder. It belongs to group of plasma cell dyscrasias such as multiple myeloma, waldenstrom’s macroglobulinemia, castleman disease and monoclonal gammopathy of undetermined significance. The term POEMS syndrome was first coined in 1980 by Bardwick. In the last 10 years clinical criteria to diagnose POEMS syndrome have been elaborated to avoid overlap with other haematological disorders. Due to disease rarity there is very few case series and case reports described in literature. This is a haematological disease which frequently presents with peripheral neuropathy but endocrine dysfunction can be a presenting or an associated feature. We report two cases of POEMS syndrome who presented with endocrinopathy as a predominant feature with literature review of this syndrome.