Open AccessPOEMS syndrome associated with Castleman disease: a case report and literature review
Author(s) -
Jinwu Kang,
Fang Yang,
Zhang Hong-ya,
Mengmeng Hu,
Xia Feng,
Jin-Cun Wang,
Yanchun Deng,
Gang Zhao
Publication year - 2014
Publication title -
neuroimmunolgy and neuroinflammation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.125
H-Index - 4
eISSN - 2349-6142
pISSN - 2347-8659
DOI - 10.4103/2347-8659.135577
Subject(s) - medicine , poems syndrome , castleman disease , disease , pathology , dermatology , polyneuropathy
Polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes (POEMS) syndrome is a multisystemic disorder that clinically manifests as paraneoplastic and monoclonal plasma cell dyscrasia. Its acronym is derived from its principal characteristics: polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes. Here, the authors reported a case of POEMS syndrome that was also associated with Castleman disease. A 53-year-old female patient was admitted to our hospital with limb weakness, numbness, edema, abdominal distention, and fever. Physical examination revealed tetraplegia, paraesthesia, and hyporeflexia in all four limbs, in addition to lymphadenectasis, splenomegaly, skin hyperpigmentation, hypertrichosis, and pitting edema. Laboratory tests and imaging revealed thrombocytosis, hypothyroidism, diabetes, hydropericardium, hydrothorax, splenomegaly, and lymphadenectasis. Electromyography showed the characteristic patterns of both demyelinating disease and axonal degeneration. Serum protein electrophoresis revealed monoclonal immunoglobulin G-lambda paraproteins. Histological examination clearly diagnosed the disease as the hyaline vascular subtype. The final diagnosis in this case was POEMS syndrome in association with Castleman disease