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Thromb Haemost 2009; 102(06): 1157-1164
DOI: 10.1160/TH09-04-0225
Review Article
Schattauer GmbH

Glanzmann’s thrombasthenia (defective platelet integrin αIIb-β3): proposals for management between evidence and open issues

Giovanni Di Minno
1   Regional Reference Centre for Coagulation Disorders, Department of Clinical and Experimental Medicine, Federico II University, Naples, Italy
,
Antonio Coppola
1   Regional Reference Centre for Coagulation Disorders, Department of Clinical and Experimental Medicine, Federico II University, Naples, Italy
,
Matteo N. D. Di Minno
1   Regional Reference Centre for Coagulation Disorders, Department of Clinical and Experimental Medicine, Federico II University, Naples, Italy
,
Man-Chiu Poon
2   Departments of Medicine, Pediatrics and Oncology, University of Calgary and Southern Alberta Rare Blood and Bleeding Disorders Comprehensive Care Program, Calgary Health Region, Calgary, Canada
› Institutsangaben
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Publikationsverlauf

Received: 04. April 2009

Accepted after minor revision: 22. Juli 2009

Publikationsdatum:
28. November 2017 (online)

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Summary

Glanzmann’s Thrombasthenia (GT) is a rare autosomal recessive bleeding disorder, characterized by a quantitative or qualitative defect of platelet surface αIIb-β3 integrin. Presently, no specific guideline/algorithm for clinical management for GT is available. Due to the rarity and heterogeneity of inherited platelet abnormalities, recommendations and guidelines are based on reports from opinions and clinical experience of panel of experts, and refer to the general management of platelet disorders. Based on the limited evidence in the area and on the strategies in clinical settings of inherited/acquired platelet defects, proposals for management of minor bleeding; moderate/major bleeding unresponsive to conservative management; major surgery; minor surgery and dental procedures for GT patients without, or with anti-platelet isoantibodies are reported. In addition to life-style advices and continuous patient education programs, when and how to employ/combine local measures, antifibrinolytic agents, hormone treatment, platelet transfusions and recombinant activated Factor VII is described. The prospective collection of treatments in GT patients recently established (Glanzmann’s Thrombasthenia Registry, GTR), based on a careful definition of clinical settings and outcomes, is likely to provide newer insight for optimising clinical management in GT.

Keywords

Anti-platelet antibodies - clinical management - Glanzmann’s thrombasthenia - platelet transfusion - recombinant activated factor VII
 

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