Anti-αIIb ß3 immunization in Glanzmann thrombasthenia: review of literature and treatment recommendations.
Br J Haematol
; 181(2): 173-182, 2018 04.
Article
em En
| MEDLINE
| ID: mdl-29611179
ABSTRACT
Glanzmann thrombasthenia (GT) is caused by inherited defects of the αIIb ß3 platelet glycoprotein. This bleeding disorder can be treated with platelet transfusion therapy, but some patients will be immunized and begin to form anti-human leucocyte antigen (HLA) and/or anti-αIIb ß3 antibodies. These antibodies can bind and interfere with the function of the transfused platelets, rendering treatment ineffective. However, platelet transfusion refractoriness attributable to HLA antibodies may be managed by the selection of compatible donors, although they are not always readily available, particularly in an emergency. Thus, anti-αIIb ß3 antibodies represent one of the most severe complications in GT. Both genetic and environmental factors may contribute to the risk of anti-αIIb ß3 development, but the underlying pathogenic mechanisms are still unknown. This review will summarize the current knowledge of the risk factors for development of anti-αIIb ß3 antibodies in patients with GT and discuss how these findings may influence the clinical management of patients.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Autoanticorpos
/
Trombastenia
/
Imunização
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Transfusão de Plaquetas
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Complexo Glicoproteico GPIIb-IIIa de Plaquetas
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Reação Transfusional
Tipo de estudo:
Etiology_studies
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Guideline
/
Risk_factors_studies
Limite:
Humans
Idioma:
En
Revista:
Br J Haematol
Ano de publicação:
2018
Tipo de documento:
Article
País de afiliação:
França