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Cell Journal، جلد ۱۳، شماره Supplement، صفحات ۰-۰
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عنوان انگلیسی O-12: Molecular Study of Immune Thrombocytopenic Purpura in Amirkola Children Hospital
چکیده انگلیسی مقاله Objective: Immune thrombocytopenic purpura (ITP) is a disorder characterized by accelerated destruction of the antibody-sensitized platelet by phagocytic cells, especially those of the spleen.ITP is the most common cause of acquired thrombocytopenia in children. Roughly 80% of affected children have platelet counts bellow 20,000/ mm3, often less than10, 000/mm3.It should be distinguished from other type of thrombocytopenia. Some of them are; Bernard-Soulier syndrome, May-Hegglin anomaly, X-linked thrombocytopenia with thalassemia, X-linked gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, Glanzmann thrombasthenia. Materials and Methods: From October 2007 through October 2010 in Iran, Babol, Amirkola Children Hospital, all patients with acute ITP were recruited in our study. All patients have been treated with IV IGg or prednisolon and cyclosporine. If platelet count didn`t show normalization after 6 month of diagnosis, they were considered as chronic ITP. Molecular study was conducted for this group. Results: 68 patients (48 boys 70.5% and 20 girls 25.5%) were diagnosed as acute ITP. They were 1 month-11- years age (median age=2.5 years). In 56 patients platelet count raised to normal range after 6 month of diagnosis but in 12 patients platelet count didn`t normalized after 6 month of diagnosis. Molecular study showed severe deficiency of glycoproteins Ib/IX (CD42), that are diagnostic of Bernard-Soulier syndrome (BSS) in one patient. Conclusion: All patients diagnosed as ITP that are refractory to conventional therapy should be investigated to other diagnosis by molecular study of platelet.
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نشانی اینترنتی http://celljournal.org/journal/article/abstract/1520
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