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 2 3 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
Glanzmann thrombasthenia and Bernard-Soulier syndrome in South Iran / A. Afrabiasi, A. Artoni, M. Karimi, F. Peyvandi, E. Ashouri, P.M. Mannucci. - In: CLINICAL AND LABORATORY HAEMATOLOGY. - ISSN 0141-9854. - 27:5(2005 Oct), pp. 324-327. [10.1111/j.1365-2257.2005.00725.x]
Glanzmann thrombasthenia and Bernard-Soulier syndrome in South Iran
F. Peyvandi;P.M. MannucciUltimo
2005
Abstract
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 2 3 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 requirementsFile | Dimensione | Formato | |
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