Acquired Glanzmann thrombasthenia: From antibodies to anti-platelet drugs.
Acquired Glanzmann thrombasthenia
Anti-thrombotic therapy
Antibodies to αIIbβ3
Drug-dependent antibodies
Infections and inflammation
Leukemia and cancer
Primary and secondary immune thrombocytopenia
Journal
Blood reviews
ISSN: 1532-1681
Titre abrégé: Blood Rev
Pays: England
ID NLM: 8708558
Informations de publication
Date de publication:
07 2019
07 2019
Historique:
received:
08
01
2019
revised:
15
03
2019
accepted:
19
03
2019
pubmed:
24
4
2019
medline:
8
1
2020
entrez:
24
4
2019
Statut:
ppublish
Résumé
In contrast to the inherited platelet disorder given by mutations in the ITGA2B and ITGB3 genes, mucocutaneous bleeding from a spontaneous inhibition of normally expressed αIIbβ3 characterizes acquired Glanzmann thrombasthenia (GT). Classically, it is associated with autoantibodies or paraproteins that block platelet aggregation without causing a fall in platelet count. However, inhibitory antibodies to αIIbβ3 are widely associated with primary immune thrombocytopenia (ITP), occur in secondary ITP associated with leukemia and related disorders, solid cancers and myeloma, other autoimmune diseases, following organ transplantation while cytoplasmic dysregulation of αIIbβ3 function features in myeloproliferative and myelodysplastic syndromes. Antibodies to αIIbβ3 occur during viral and bacterial infections, while drug-dependent antibodies reacting with αIIbβ3 are a special case. Direct induction of acquired GT is a feature of therapies that block platelets in coronary artery disease. This review looks at these conditions, emphasizing molecular mechanisms, therapy, patient management and future directions for research.
Identifiants
pubmed: 31010659
pii: S0268-960X(19)30002-5
doi: 10.1016/j.blre.2019.03.004
pii:
doi:
Substances chimiques
Antibodies
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
10-22Informations de copyright
Copyright © 2019 Elsevier Ltd. All rights reserved.