POEMS syndrome associated with Castleman disease, hyaline vascular type: a rare case report
Keywords:
POEMS syndrome, Castleman disease, polyneuropathy, monoclonal gammopathy, ascitesAbstract
POEMS (Peripheral Neuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin
changes) syndrome, a rare paraneoplastic disease, presents multisystemic disorder related to underlying plasma cell dyscrasia. Approximately 10-30% of patients with POEMS are associated with Castleman disease. Castleman disease has a wide variety of presentations ranging from simple B-symptoms to various autoimmune symptoms or a frank POEMS syndrome. The spectrum of disease may overlap between osteosclerotic myeloma (OSM), Castleman disease and POEMS syndrome. Herein, we reported a 68-year-old Thai woman who presented with generalized lymphadenopathy compatible with Castleman disease together with a new onset of ascites, splenomegaly, polyneuropathy and history of arterial thrombosis. Accordingly, the diagnosis was POEMS syndrome with Casrtleman disease. Treatment with cyclophosphamide, thalidomide and dexamethasone (CTD) regimen was used. Clinical symptoms of extravascular volume overload (ascites and legs edema) gradually improved after 2 cycles of CTD regimen
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