Glanzmann’s thrombasthenia: A review of literature

Abstract

Introduction: Glanzmann’s thrombasthenia is an inherited autosomal recessive bleeding disorder in which there is a deficiency of platelet glycoprotein GpIIb/IIIa. These receptors allow platelets to aggregate by sticking to each other, releasing granular contents to potentiate the process of clotting. Platelets are not able to clump together when stimulated, resulting in failure to form platelet aggregates. In such instances, bleeding time is prolonged and the clot retraction is defective, resulting in increased bleeding tendency. GT is diagnosed by tests that determine whether there is a deficiency of the α11bβ3 (GpIIb/IIIa) receptor. The diagnosis is confirmed mainly by flow cytometry and monoclonal antibodies. In the platelet function analyzer 100 (PFA-100), platelets fail to plug on the filter, as platelet aggregation response is defective. The condition may present in early childhood causing repeated nasal bleeds, or in adulthood as menorrhagia, antenatal, and postpartum hemorrhage. Patients may require many leukocyte-depleted blood products during bleeding episodes. Objectives: Objectives of this review article is to understand the implications of Glanzmann’s thrombasthenia,its pathogenesis, diagnostic modalities and treatment options as it is a rare platelet disorder. Surgeons have to be on the alert if the case is a Glanzmann’s thrombasthenia, as uncontrolled hemorrhage not responding to usual treatment can have fatal consequences. Materials and methods: An extensive search and review of current literature was undertaken via PubMed and EMBASE to know the historical background with its latest management options available. Results and evidence: Studies have suggested that GT is a hereditary disorder occurring predominantly in consanguineous populations with female preponderance. Remarks and conclusions: As consanguinity is very high in this part of the world awareness among clinicians of this medical disorder is necessary to avoid unanticipated hemorrhage and its untoward complications.