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Vol. 12. Issue 3.
Pages 275-280 (May - June 2006)
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Vol. 12. Issue 3.
Pages 275-280 (May - June 2006)
Cadernos de Anatomia Patológica\Pathology Note Books
Open Access
Hiperplasia linfóide angiofolicular – Doença de Castleman – do mediastino: Apresentação de um caso clínico
Mediastinal angiofolicular lymphoid hyperplasia – Castleman’s disease – Case report
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Vítor Sousa*, Lina Carvalho**
* Assistente Hospitalar e Assistente Estagiário de Anatomia Patológica, Hospitais da Universidade de Coimbra. Serviço de Anatomia Patológica
** Chefe de Serviço e Professora Associada de Anatomia Patológica, Hospitais da Universidade de Coimbra. Serviço de Anatomia Patológica
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Resumo

A hiperplasia linfóide angiofolicular foi inicialmente descrita por Castleman como uma hiperplasia do gânglio linfático, caracterizada por folículos anormais, com centros germinativos pequenos e marcada proliferação capilar.

Os autores apresentam o caso de um homem de 37 anos com toracalgia e massa mediastínica com 4cm de diâmetro, constituída por tecido vermelho-escuro e carnudo, com pequena área central branca. Foi diagnosticada a variante plasmocitária da hiperplasia linfóide angiofolicular em gânglio linfático mediastínico. Apresentava mais dois gânglios “diafragmáticos” envolvidos pela mesma doença. A hiperplasia linfóide angiofolicular, ou doença de Castleman, é uma doença linfoproliferativa rara que atinge frequentemente o mediastino ou os gânglios linfáticos do hilo pulmonar. Pode ter, no entanto, outras localizações, na forma multisistémica. Estão descritas duas variantes morfológicas: a vascular hialina e a plasmocitária. A IL-6 parece ter um papel importante na sua patogenia, quando se trata da variante plasmocitária. A doença de Castleman deve ser considerada no diagnóstico diferencial de massas mediastínicas, nomeadamente com linfomas.

Rev Port Pneumol 2006; XII (3): 275-280

Palavras-chave:
Doença de Castleman
hiperplasia linfóide angiofolicular do medistino
doenças do mediastino
Abstract

Angiofolicular lymphoid hyperplasia was first described by Castleman as a lymph node hyperplasia characterized by abnormal follicles with small germinal centres and high capillary proliferation.

The authors present a case of a 37 year old man with thoracalgy and a mediastinal mass of 4cm diameter, fleshy and red with a white central area. The diagnosis of plasma cell type of angiofolicular lymphoid hyperplasia in an mediastinal lymph node was made. He also had involvement of two “diaphragmatic” lymph nodes.

Angiofolicular lymphoid hyperplasia – Castleman’s disease - is a rare lymphoproliferative disease that involves the mediastine or the pulmonary hilus when of solitary form but it can also have other localizations (multicentric or systemic form). Two histological variants are described, the hyaline vascular type and the plasma cell type. IL-6 seems to be in the plasma cell type, due to plasma cells proliferation. Castleman’s Disease must be considered in the differential diagnosis of mediastinal masses, namely lymphomas.

Rev Port Pneumol 2006; XII (3): 275-280

Key-words:
Castleman’s disease
mediastinal angiofolicular lymphoid hyperplasia
mediastinal disease
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Copyright © 2006. Sociedade Portuguesa de Pneumologia/SPP
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