Glanzmann thrombasthenia and Bernard-Soulier syndrome in south Iran.
Glanzmann thrombasthenia (GT) and Bernard-Soulier syndrome (BSS) are two rare inherited disorders of platelet function. In this study, we report the demographic, clinical and biological characteristics of 23 patients with GT and of seven patients with BSS from southern Iran who had been followed for many years but fully characterized only recently, when platelet aggregation tests and flow cytometric studies became available for the first time in the country. We found a high prevalence of both diseases that can be explained by the high rate of consanguineous marriages in south Iran. Patients affected by GT and BSS suffer mainly from mucocutaneous bleedings causing anemia and transfusion requirements.
Afrasiabi
A
A
Haemostasis and Thrombosis Unit, Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran.
Afrabiasi
A
A
Artoni
A
A
Karimi
M
M
Peyvandi
F
F
Ashouri
E
E
Mannucci
P M
PM
D000293:Adolescent; D000328:Adult; D000740:Anemia / Q000209:etiology / Q000628:therapy; D001606:Bernard-Soulier Syndrome / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*; D001803:Blood Transfusion / Q000706:statistics & numerical data; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005434:Flow Cytometry; D005500:Follow-Up Studies; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D007492:Iran / Q000453:epidemiology; D010974:Platelet Aggregation; D010979:Platelet Function Tests; D015995:Prevalence; D013915:Thrombasthenia / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*