Abstract

GONZALEZ DE LA ALEJA, P.; GARCIA-NAVARRO, M.; SANCHEZ-MARTINEZ, R.  and  RAMOS, J. M.. Incomplete POEMS syndrome with multicentric Castleman’s disease and amyloidosis. Anales Sis San Navarra [online]. 2017, vol.40, n.3, pp.475-478. ISSN 1137-6627.  https://dx.doi.org/10.23938/assn.0041.

Castleman’s disease (CD) is an atypical lymphoproliferative disorder of unknown cause, characterized by non-clonal nodal hyperplastic growth. Two forms of clinical presentation are currently recognized, one localized and the other multicentric, and four histopathologic variants. It is characterized by generalized lymphadenopathy, hepatosplenomegaly, fever and night sweats.

CD may present severe pancytopenia, multi-organ failure, lymphoma evolution and it can sometimes be associated with paraneoplastic syndromes such as POEMS syndrome. Associations of these two entities have been widely described in the current literature, although its less common association with amyloidosis is described as isolated clinical cases. We report a case with this triple association: EC, POEMS and amyloidosis.

Keywords : POEMS syndrome; Castleman’s disease; Amyloidosis.

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