Glanzmann's thrombasthenia.
Glanzmann's thrombasthenia (GT) is an uncommon cause of bleeding in children. We diagnosed two siblings as having GT on the basis of flow cytometric studies. Both had cutaneous bleedings and epistaxis since early childhood. Hematological investigations revealed prolonged bleeding time and a normal platelet count. Both the patients had absence of aggregation of platelets with the agonist adenosine diphosphate. Absence of the GPIIb/IIIa receptor was confirmed by flow cytometry. A short review of the disorder is presented.
Tullu
M S
MS
Department of Pediatrics, Seth G.S. Medical College and KEM Hospital, Parel, Mumbai-400 012, Maharashtra, India. milindtullu@yahoo.com
Dixit
P S
PS
Nair
S B
SB
Kamat
J R
JR
Vaswani
R K
RK
Shetty
S D
SD
Pawar
A R
AR
D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005434:Flow Cytometry; D006474:Hemorrhagic Disorders / Q000235:genetics; D006801:Humans; D008297:Male; D019039:Platelet Glycoprotein GPIIb-IIIa Complex / Q000235:genetics; D013915:Thrombasthenia / Q000175:diagnosis / Q000235:genetics*
D019039:Platelet Glycoprotein GPIIb-IIIa Complex