Defeitos Congênitos das plaquetas
Palabras clave:
trombocitopenia hereditária, trombocitopatia hereditária, plaquetas, plaquetopenia congênita, defeitos congênitos das plaquetas.Resumen
Introdução: As plaquetas desempenham um papel importante no processo de hemostasia e alterações numéricas (trombocitopenias) ou qualitativas (trombocitopatias) das plaquetas levam a sangramento espontâneo, tempo de sangramento prolongado, hemorragias mucocutâneas, gastrintestinais e urinárias. A maioria das alterações plaquetárias tem suas manifestações clínicas durante a infância, porém algumas são diagnosticadas em idade mais avançada. Quando a trombocitopenia ou a trombocitopatia é diagnosticada, a forma congênita deve ser distinguida das formas adquiridas. A forma congênita pode ser uma enfermidade isolada ou fazer parte de uma síndrome. Uma vez estabelecido que a desordem é hereditária, o modo de herança é útil no diagnóstico diferencial. Objetivo: Revisar os mecanismos patológicos relacionados aos defeitos congênitos das plaquetas. Metodologia: A revisão foi realizada por levantamento bibliográfico de banco de dados obtidos através de pesquisa direta, MEDLINE, PUBMED e LILACS-BIREME; sendo selecionados artigos publicados abordando as alterações congênitas das plaquetas. Conclusão: Defeitos congênitos que envolvem as plaquetas levam a sangramentos cutâneos, mucocutâneos e gastrintestinal e urinário. Uma vez que estes defeitos são de caráter congênito e hereditário, as crianças são os pacientes predominantes. Em casos graves, o tratamento de escolha é o transplante de células tronco hematopoiéticas.
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