Clinico-hematological and thromboelastographic profiles in glanzmann’s thrombasthenia

Ahammad, Javed and Kamath, Asha and Shastry, Shamee and Kurien, Annamma (2019) Clinico-hematological and thromboelastographic profiles in glanzmann’s thrombasthenia. Blood Coagul Fibrinolysis, 31 (1). pp. 29-34. ISSN 0957-5235

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Abstract

Glanzmann’s thrombasthenia is a rare inherited bleeding disorder characterized by the quantitative or qualitative defect of glycoprotein IIb/IIIa receptor on platelets which leads to ineffective aggregation. Light transmittance aggregometry is considered as the gold standard for diagnosis of Glanzmann’s thrombasthenia. Thromboelastography (TEG) is a global hemostatic assay which measures clot formation, clot strengthening and fibrinolysis. This study evaluates the clinical, laboratory and TEG profiles in patients with Glanzmann’s thrombasthenia. Bleeding score by (International Society on Thrombosis and Haemostasis) ISTH-bleeding assessment tool (bleeding score), laboratory tests to diagnose Glanzmann’s thrombasthenia, and TEG parameters were correlated in 11 Glanzmann’s thrombasthenia patients. Seventeen participants with normal bleeding score were included as controls. Bleeding score was increased in all patients. The highest bleeding score was in an adult female (26), whereas the lowest score (4) was in two children of less than 1 year. Majority of TEG parameters (except R-time) showed a statistically significant difference between Glanzmann’s thrombasthenia patients and controls (K-time: P< 0.001, angle: P< 0.001, maximum amplitude: P< 0.001). The average time required to record the maximum amplitude was 23 min. Maximum amplitude was markedly reduced in all Glanzmann’s thrombasthenia patients with an average of 20.9 mm (reference range 44–68 mm) having 100% sensitivity. The thromboelastographic profile of Glanzmann’s thrombasthenia showed a consistently reduced maximum amplitude. Hence reduced maximum amplitude with a normal platelet count, significant bleeding score and prolonged bleeding time could potentially be used as a preliminary algorithm for the diagnosis of Glanzmann’s thrombasthenia.

Item Type: Article
Uncontrolled Keywords: Bleeding score; Glanzmann’s thrombasthenia; light transmittance aggregometry; maximum amplitude; thromboelastography.
Subjects: Departments at MU > Public Health
Medicine > KMC Manipal > Immunohematology and Blood Transfusion
Medicine > MMMC Manipal > Pathology
Depositing User: KMC Library
Date Deposited: 15 Oct 2020 03:54
Last Modified: 15 Oct 2020 03:54
URI: http://eprints.manipal.edu/id/eprint/155827

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