Clinical spectrum of Glanzmann's thrombasthenia.
Glanzmann's thrombasthenia is a well defined inherited disorder of platelet function characterized by qualitative and qualitative defect in cytoadhesive membrane protein, glycoprotein IIb-IIIa (the platelet fibrinogen receptor). From January 1990 to October, 1999, five patients who presented with mucocutaneous bleeding were detected to have Glanzmann's thrombasthenia. Clinical and laboratory spectrum of this rare disorder was studied which revealed heterogeneity of disease with respect to nature and severity of bleeding unpredictable by laboratory findings.
Badhe
B A
BA
Departments of Pathology and Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry.
Jayanthi
S
S
Datta
T
T
D000293:Adolescent; D001792:Blood Platelets / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006470:Hemorrhage; D006487:Hemostasis; D006801:Humans; D008297:Male; D010974:Platelet Aggregation; D019039:Platelet Glycoprotein GPIIb-IIIa Complex / Q000378:metabolism; D013915:Thrombasthenia / Q000503:physiopathology*
D019039:Platelet Glycoprotein GPIIb-IIIa Complex