Hiperplasia linfóide angiofolicular – Doença de Castleman – do mediastino: Apresentação de um caso clínico

 

Mediastinal angiofolicular lymphoid hyperplasia – Castleman’s disease – Case report 

 

Coordenadora: Lina Carvalho

Vitor Sousa *

Lina Carvalho **

 

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 4 cm de diâmetro, constituída por tecido vermelho-escuro e carnudo, com pequena área central branca. Foi diagnosticada a variante plasmocitária da hiperplasia infóide 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.

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 4 cm 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.

Key-words: Castleman’s disease, mediastinal angiofolicular lymphoid hyperplasia, mediastinal disease.

 

 

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Bibliografia

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* Assistente Hospitalar e Assistente Estagiário de Anatomia Patológica

** Chefe de Serviço e Professora  Associada de Anatomia Patológica

Hospitais da Universidade de Coimbra. Serviço de Anatomia Patológica (Directora: Dra. Fernanda Xavier da Cunha)

 

 

Recebido para publicação/received for publication: 06.03.02

Aceite para publicação/accepted for publication: 06.03.09