Glanzmann's Thrombasthenia: The Clinicopathological Spectrum and its Diversities

Belurkar, Sushma V and Kurien, Annamma and Singh, Pankaj Bir (2012) Glanzmann's Thrombasthenia: The Clinicopathological Spectrum and its Diversities. Annals of Nigerian Medicine, 6 (1). pp. 35-40.

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Abstract

Aim: To study the clinical spectrum of patients with Glanzmann's Thrombasthenia (GT) in a tertiary care hospital in South India and to correlate the clinical & laboratory parameters of GT.Materials and Methods: This study includes 15 patients who were diagnosed as GT by characteristic clinical features, basic lab parameters (prolonged bleeding time, abnormal clot retraction and absence of platelet clumps on direct smear) and platelet aggregometry. Results: 66.6% of our patients were below 10 years of age. The common forms of bleeding in our patients was epistaxis,gum bleeding and prolonged bleeding from trauma site which was seen in >50% of our patients.The significant findings on routine laboratory testing was a prolonged bleeding time (93.33%) and an abnormal clot retraction test (66.6%). Platelet aggregation studies showed decreased aggregation with ADP, Collagen and Epinephrine in 100% of our patients whereas 50% also showed decreased aggregation with Ristocetin.Hence most of the patients in this study presented with the classical symptoms and laboratory findings of GT however some had unusual findings. Conclusion: The prevelance of GT is higher in southern India due to increased incidence of consanguineous marriages in this area.Though basic hemostatic screening tests and platelet aggregometry are sufficient to establish the diagnosis in majority of the patients,however some may require more sophisticated tests like flowcytometry for glycoprotein (GP) IIb-IIIa

Item Type: Article
Uncontrolled Keywords: Glanzmann′s thrombasthenia; Platelet aggregation; Glycoprotein IIb-IIIa; haemostasis; mucocutaneous bleeding; consanguinity
Subjects: Medicine > MMMC Manipal > Pathology
Medicine > KMC Manipal > Pathology
Depositing User: KMC Manipal
Date Deposited: 25 Mar 2013 06:27
Last Modified: 16 Sep 2015 12:39
URI: http://eprints.manipal.edu/id/eprint/79272

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