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Int J Clin Exp Pathol 2010;3(4):443-447
Case Report
Glanzmann’s thrombasthenia: report of a case and review of the literature
Christopher Sebastiano, Michael Bromberg, Karen Breen, Matthew T. Hurford
Department of Pathology and Laboratory Medicine, Temple University Hospital, Philadelphia, PA, USA
Received November 17, 2009; accepted November , 2009; available online November , 2009
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. (IJCEP911005).
Key words: Glanzmann’s thrombasthenia, glycoprotein complex, GPIIb/GPIIIa, platelet dysfunction
Full text PDF
Address all correspondence to:
Mathew T. Hurford, MD
Department of Pathology and Laboratory Medicine
Temple University Hospital
3401 North Broad Street Philadelphia
PA 19140. Tel: 215-707-7740; Fax: 215-707-2053
Email: matthewh@temple.edu
Case Report
Glanzmann’s thrombasthenia: report of a case and review of the literature
Christopher Sebastiano, Michael Bromberg, Karen Breen, Matthew T. Hurford
Department of Pathology and Laboratory Medicine, Temple University Hospital, Philadelphia, PA, USA
Received November 17, 2009; accepted November , 2009; available online November , 2009
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. (IJCEP911005).
Key words: Glanzmann’s thrombasthenia, glycoprotein complex, GPIIb/GPIIIa, platelet dysfunction
Full text PDF
Address all correspondence to:
Mathew T. Hurford, MD
Department of Pathology and Laboratory Medicine
Temple University Hospital
3401 North Broad Street Philadelphia
PA 19140. Tel: 215-707-7740; Fax: 215-707-2053
Email: matthewh@temple.edu
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