Doença de Castleman Retroperitoneal

Caso Clínico e Revisão da Literatura

 

Luísa Valle*; Patrícia Pinto†; Sofia Pimenta†; Manuela Dias*; Fernando Príncipe‡; Rui de Sousa*

 

Serviços de *Medicina A; †Reumatologia e ‡ Hematologia Clínica do Hospital de São João, Porto.

 

A doença de Castleman é uma entidade linfoproliferativa atípica, pouco frequente, com evolução crónica e sintomas inespecíficos. Os autores apresentam um caso clínico de doença de Castleman do tipo misto, de localização retroperitoneal cuja manifestação inicial foi uma anemia hemolítica autoimune por anticorpos frios. Fazem ainda uma revisão dos diferentes subtipos da doença, formas de apresentação, abordagem diagnóstica e terapêutica.

Palavras-chave: Doença de Castleman, forma localizada, forma multicêntrica.

 

Retroperitoneal Castleman’s Disease

Castleman´s disease is an atypical lymphoproliferative disorder, relatively rare, with chronic evolution and nonspecific symptoms. We report a case of retroperitoneal mixed histologic type Castleman´s disease with cold haemolytic anemia as initial clinical manifestation. We also review the different subtypes, clinical presentation, diagnostic and therapeutic approach.

Key-words: Castleman´s disease, unicentric form, multicentric form.

 

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Correspondência:

Dr.ª Luisa Valle

Serviço de Medicina A

Hospital de São João

Alameda Prof. Hernâni Monteiro

4200-319 Porto

e-mail: luisavalle@sapo.pt