title: Presentation and pattern of symptoms in 382 patients with Glanzmann thrombasthenia in Iran.
abstract: Glanzmann thrombasthenia (GT) is a rare autosomal recessive disease characterized by prolonged bleeding time with normal platelet count and morphology. It is caused by the quantitative or qualitative deficiency of the platelet glycoprotein IIb-IIIa. In 382 Iranian patients with GT diagnosed at a single center during the period 1969-2001, consanguinity between parents was 86.6%, in accord with the high frequency of intrafamilial marriages in Iran. Almost all patients had had abnormal mucocutaneous bleeding (epistaxis and gum bleeding); at follow-up, 4/5 of the patients had been transfused at least once to control hemorrhagic episodes. As expected, almost all the patients had a normal platelet count while the leukocyte count was increased in 19.3%. Among women, an unexpected low rate of pregnancies was observed.
authors.affiliation: Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: G
authors.initials: G
authors.lastname: Toogeh
authors.forename: R
authors.initials: R
authors.lastname: Sharifian
authors.forename: M
authors.initials: M
authors.lastname: Lak
authors.forename: R
authors.initials: R
authors.lastname: Safaee
authors.forename: A
authors.initials: A
authors.lastname: Artoni
authors.forename: F
authors.initials: F
authors.lastname: Peyvandi
mesh_terms: D000328:Adult; D003241:Consanguinity; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006470:Hemorrhage / Q000453:epidemiology* / Q000517:prevention & control; D006801:Humans; D007492:Iran / Q000453:epidemiology; D007958:Leukocyte Count; D008297:Male; D008875:Middle Aged; D010976:Platelet Count; D017713:Platelet Transfusion; D012189:Retrospective Studies; D013915:Thrombasthenia / Q000097:blood / Q000453:epidemiology* / Q000209:etiology
chemical_list: D006454:Hemoglobins