Glanzmann thrombasthenia and Bernard–Soulier syndrome in south Iran

Authors

  • A. AFRABIASI,

    1. Haemostasis and Thrombosis Unit, Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran
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  • A. ARTONI,

    1. Department of Internal Medicine and Dermatology, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, University of Milan, Milan, Italy
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  • M. KARIMI,

    1. Haemostasis and Thrombosis Unit, Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran
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  • F. PEYVANDI,

    1. Department of Internal Medicine and Dermatology, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, University of Milan, Milan, Italy
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  • E. ASHOURI,

    1. Immunology Department, Shiraz University of Medical Science, Shiraz, Iran
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  • P. M. MANNUCCI

    1. Department of Internal Medicine and Dermatology, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, University of Milan, Milan, Italy
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Errata

This article is corrected by:

  1. Errata: Corrigendum Volume 28, Issue 4, 290, Article first published online: 18 July 2006

Pier Mannuccio Mannucci, Department of Internal Medicine, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Via Pace 9, 20122 Milan, Italy. Tel.: +39 02 55035421;
Fax: +39 02 50320723; E-mail: pmmannucci@libero.it

Summary

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.

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