Glanzmann's thrombasthenia: report of a case and review of the literature.
Publication/Presentation Date
4-25-2010
Abstract
Glanzmann's thrombasthenia is a rare congenital bleeding disorder. Patients usually present with mucocutaneous bleeding and excessive bleeding associated with trauma and/or surgery. Patients have an increased bleeding time and a normal platelet count with abnormal platelet function assays. Genetically, Glanzmann's thrombasthenia is associated with mutations in the genes which encode for glycoproteins, GPIIb or GPIIIa. Defects in these genes lead to a lack of or highly reduced expression of the glycoprotein complex (GPIIb/GPIIIa), resulting in platelet dysfunction. Bleeding is managed by platelet transfusions. Bone marrow transplants have been used successfully in rare cases. With proper supportive care Glanzmann's thrombasthenia has a very good prognosis.
Volume
3
Issue
4
First Page
443
Last Page
447
ISSN
1936-2625
Published In/Presented At
Sebastiano, C., Bromberg, M., Breen, K., & Hurford, M. T. (2010). Glanzmann's thrombasthenia: report of a case and review of the literature. International journal of clinical and experimental pathology, 3(4), 443–447.
Disciplines
Medicine and Health Sciences
PubMedID
20490335
Department(s)
Department of Pathology and Laboratory Medicine
Document Type
Article