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Artigo Acesso aberto Produção Nacional Revisado por pares
BIBTEX RIS

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2011; Elsevier BV; Volume: 86; Issue: 5 Linguagem: Inglês

10.1590/s0365-05962011000500032

ISSN

1806-4841

Autores

Giovana Tadéia Fantinato, Silmara da Costa Pereira Cestari, João Paulo Junqueira Magalhães Afonso, Letícia Siqueira Sousa, Mílvia Maria Simões e Silva Enokihara,

Tópico(s)

T-cell and Retrovirus Studies

Resumo

Chediak-Higashi syndrome is characterized by varying degrees of oculocutaneous albinism, recurrent infections, bleeding disorders and variable neurological involvement. The treatment consists of bone marrow transplantation, which corrects the immunologic and hematologic defects. Untreated patients die as the result of bacterial infections or develop "accelerated phase" lymphoproliferation. We present a case of Chediak-Higashi syndrome and discuss the clinical and laboratorial features that determine its diagnosis.

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