Resumo

SOTELO-JIMENEZ, Pedro Paolo; RAMIREZ-CALDERON, Fanny Elizabeth  e  QUINONES-AVILA, María Del Pilar. Castleman disease variant of poems syndrome: a case report. Rev. Fac. Med. Hum. [online]. 2019, vol.19, n.4, pp.120-125. ISSN 1814-5469.  http://dx.doi.org/10.25176/RFMH.v19i4.2145.

POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein and skin changes) is a rare multisystemic disorder. Castleman's disease is an atypical lymphoproliferative disorder of unknown cause, which may be associated with POEMS. A 37-year-old female patient with a clinical picture of 2 years of clinical evolution due to multiple adenopathies, numbness, and weakness of the lower extremities associated with cutaneous hyperpigmentation and hypertrichosis. Electromyography shows an active, sensory-motor chronic polyneuropathy of the axonal type; and serum electrophoresis showed monoclonal band of lambda type Immunoglobulin A. Submandibular ganglion biopsy was compatible with Castleman's disease. This report highlights the fact that Castleman's disease occurs frequently as part of a variant of the POEMS syndrome, and should be ruled out in these patients. Also, plasmapheresis can be useful in patients with severe neurological symptoms.

Palavras-chave : POEMS syndrome; Castleman's disease; Plasmapheresis.

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