Glanzmann's thrombasthenia.
During January 1981 to June 1991, 20 patients from 16 unrelated families were detected to have Glanzmann's thrombasthenia (GT). Twelve families (75%) had history of consanguinity, with 6 first cousins and 3 uncle-niece marriages; of these 7 were Muslims, 6 Hindus and 3 Christians. There were 12 girls and 8 boys; the mean age at diagnosis was 7.05 +/- 6.03 yr (range 1 day-22 yr). All cases had initial bleeding prior to the age of 5 yr with the mean age at the initial episode of bleeding being 2.21 +/- 1.34 yr (range 1 day-5 yr). Common pattern of bleeding included epistaxis, gingival bleeding, post-traumatic bruises, menorrhagia, gastrointestinal (2 cases), post-operative (2 cases) and spontaneous bleeding (2 cases). No patient showed hemarthrosis, intracranial bleeding or hemoptysis. Menorrhagia was a serious problem necessitating repeated transfusions and hormonal therapy. Twelve cases (60%) required 1-120 units of blood transfusions while five received platelet concentrates.
Agarwal
M B
MB
Department of Hematology, L.T.M.G. Hospital, Dadar, Bombay.
Agarwal
U M
UM
Viswanathan
C
C
Bhave
A A
AA
Billa
V
V
D002675:Child, Preschool; D005260:Female; D005808:Genes, Recessive; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008595:Menorrhagia / Q000209:etiology; D010974:Platelet Aggregation; D013915:Thrombasthenia / Q000097:blood / Q000150:complications / Q000235:genetics