DOENÇA DE VON WILLEBRAND DO TIPO 2B: UMA REVISÃO DE LITERATURA

Pedro Erbet Belém Filho Morais; Eduarda Rabêlo Lima; Beatriz Rodrigues Neri; Maria Fernanda Brunetta de Almeida

doi:10.51161/rems/641

DOENÇA DE VON WILLEBRAND DO TIPO 2B: UMA REVISÃO DE LITERATURA

Autores

Pedro Erbet Belém Filho Morais
Eduarda Rabêlo Lima
Beatriz Rodrigues Neri
Maria Fernanda Brunetta de Almeida

DOI:

https://doi.org/10.51161/rems/641

Palavras-chave:

Von Willebrand, Tipo 2B, Revisão

Resumo

Introdução: A doença de Von Willebrand (DVW) é um distúrbio hereditário hemorrágico, ocasionado pela deficiência quantitativa e/ou qualitativa do fator de Von Willebrand (FVW). A DVW é subdividida nos tipos 1, 2A, 2B, 2M, 2N e 3. O subtipo 2B consiste no defeito qualitativo e será abordado neste artigo. Objetivo: Revisar e descrever os principais aspectos da DVW tipo 2B, incluindo aspectos clínicos e laboratoriais, fisiopatologia, condução médica adequada e particularidades em relação às demais formas da doença. Material e métodos: Foi realizada uma pesquisa na literatura, utilizando a base de dados PubMed. Foram revisados 7 artigos científicos, utilizando o descritor “Doença de Von Willebrand tipo 2B”, publicados entre os anos de 2016 a 2020. Resultados: A DVW tipo 2B é caracterizada pelo aumento da afinidade do fator de von willebrand (FVW) com a glicoproteína Ibα presente na plaqueta, levando à depuração excessiva de FVW. A DVW tipo 2 se manifesta como um sangramento mucocutâneo de leve a moderada intensidade, podendo incluir trombocitopenia transitória em certas circunstâncias, como na administração de desmopressina. O diagnóstico do tipo 2 é realizado através de testes laboratoriais, sendo os principais a baixa dosagem do cofator de ristocetina (FVW:RCo), seguida pela razão entre FVW:RCo e FVW:Ag menor que 0.7. Para a confirmação do subtipo 2B, faz-se uso do RIPA e do teste genético, em que há predomínio de FVW A1. O tratamento inclui a administração de antifibrinolíticos, desmopressina e concentrados ricos em FVW para resgate. Em relação às gestantes, o uso de bloqueio peridural não é recomendado e os níveis plaquetários devem ser constantemente monitorados no trabalho de parto destas pacientes. Conclusão: Em suma, a DVW é a desordem hemorrágica hereditária mais comum, possuindo diversas manifestações clínicas, e devendo ser identificada precocemente a fim de evitar complicações futuras.

Revista Multidisciplinar em Saúde - V. 2, N. 1, 2021

Publicado

2021-02-01

Como Citar

Morais, P. E. B. F., Lima, E. R., Neri, B. R., & Almeida, M. F. B. de. (2021). DOENÇA DE VON WILLEBRAND DO TIPO 2B: UMA REVISÃO DE LITERATURA. Revista Multidisciplinar Em Saúde, 2(1), 20. https://doi.org/10.51161/rems/641

Baixar Citação

Edição

v. 2 n. 1 (2021): Edição Especial: Anais de Eventos

Seção

Anais do Congresso Brasileiro de Hematologia Clínico-laboratorial On-line

Licença

Este trabalho está licenciado sob uma licença Creative Commons Attribution 4.0 International License.

O conteúdo desta revista foi migrado para https://editoraintegrar.com.br

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Dispatcher.dispatch(Object:Request) % line  281, file: PKPApplication.inc.php
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Dispatcher.dispatch(Object:Request) % line  281, file: PKPApplication.inc.php
PKPApplication.execute() % line   68, file: index.php

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            PKPTemplateManager.display(frontend/pages/article.tpl) % line  292, file: ArticleHandler.inc.php
         ArticleHandler.view(Array[1], Object:Request) % line  391, file: PKPRouter.inc.php
      PKPRouter._authorizeInitializeAndCallRequest(Array[2], Object:Request, Array[1], false) % line  231, file: PKPPageRouter.inc.php
   PKPPageRouter.route(Object:Request) % line  143, file: Dispatcher.inc.php
Dispatcher.dispatch(Object:Request) % line  281, file: PKPApplication.inc.php
PKPApplication.execute() % line   68, file: index.php

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