Castleman disease of the hyaline vascular variant transforming to POEMS syndrome as endpoint: a case report.

ABSTRACT: BACKGROUND:POEMS syndrome is a rare neoplastic syndrome reflected by plasma cell disorder. It is composed by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. It is also reported to associate with Castleman disease. The early identification and treatment are pivotal to reduce the morbidity and mortality. CASE PRESENTATION:Here we report a 66-year-old man with treated Castleman disease developing with sequential presence of endocrinopathy polyneuropathy, skin changes, organomegaly and extravascular volume overload within 18 years, which was finally confirmed as POEMS syndrome by positive monoclonal protein. He was thereafter successfully treated with prednisone and azathioprine as primary therapy and thalidomide as maintenance therapy. CONCLUSION:The diagnosis of POEMS is based on a cluster of disorder involved in varied organs. We report a rare case that triggers the need to consider POEMS syndrome diagnosis for patients carrying Castleman disease and polyneuropathy.

SUBMITTER: Ge Y 

PROVIDER: S-EPMC6176504 | biostudies-other | 2018 Oct

REPOSITORIES: biostudies-other