Glanzmann Thrombasthenia: A Curse to Platelet Function

https://doi.org/10.46610/JPRD.2023.v05i01.001

Abstract

A platelet function disorder that is autosomal recessive with altered platelet number and appearance is called Glanzmann thrombasthenia. Quantitative and qualitative abnormalities of αIIbβ3 integrin are the molecular basis of this condition. Adhesive proteins attach aggregating platelets under the influence of this receptor and result in thrombus formation at the location of injury in blood vessels. Some patients have minimum bruising, while others have severe and frequent fatal hemorrhages in GT. Its common symptoms include purpura, epistaxis, menorrhagia, or gastrointestinal bleeding. Both alleles of one of the genes that code for the platelet integrin receptor polypeptides are affected by mutations, which results in the disease. Chronic anemia, neurological problems, and psychological problems are complications associated with this condition. Platelet aggregation, complete blood count, and prothrombin time are the standard tests to diagnose Glanzmann's thrombasthenia. Antifibrinolytics, platelet transfusions, monoclonal antibodies, hematopoietic stem molecular transplantation, and gene remedy are interventions to deal with this uncommon hematological disorder. If the treatment measures are ineffective, or to prevent bleeding during surgery, then transfuse HLA-compatible platelet concentrates. Recombinant Factor VIIa is used as an alternative therapy for this condition. Implementation of appropriate preventive measures helps us to keep this disorder at bay.