2011 yılında çocukluk çağı immün trombositopenik purpura hastalığı izlem ve tedavisinde değişiklikler

İmmün trombositopenik purpura (İTP) en sık çocukluk çağında görülen kanama nedenidir. Trombositler etkileştikleri antikorlara bağlı olarak immünolojik yıkıma uğrar ve bunu dengelemesi gereken kemik iliği yetersiz kaldığında, çevre kanında trombosit sayısı azalır. Klinikte üç şekilde karşımıza çıkar: akut ya da yeni tanı, kronik ve israrcı İTP. Akut formu birkaç günle –hafta süresinde düzelir, genellikle altı aydan önce sonlanır, 2011 yılında yayınlanan Amerikan Hematoloji Derneği kılavuzu bu süreyi bir yıla uzatmıştır. Klinik olarak başka bulgusu olmayan çocukta ani başlangıç öyküsü vardır. En sık 2-4 yaş ve viral enfeksiyonlardan sonra görülür. On yaştan büyük çocuklarda kronikleşme ve diğer otoimmün olaylarla birlikte olma olasılığı fazladır. Peteşi, purpura, burun kanaması, bağırsak ve üriner sistem kanaması olabilir. Bu kanamalar trombositopeni derecesine bağlı olarak gelişse de kanama miktar ve şiddeti ile trombosit sayısı arasında her zaman açık bir ilişki yoktur. Tedavide çocukluk çağında iki seçenek en sık kullanılmaktadır: kortikosteroidler ve intravenöz immünoglobülin. Tedavide immünosupresifler, anti D, siklosporin, sitostatikler, danazol, rituksimab ve trombopoietin reseptör analogları da denenmektedir. Çok nadiren hayatı tehdit eden kanamalarda tedavide trombosit süspansiyonu denenebilir. Son zamanlarda splenektomi 1/3 olguda başarısız olunduğu ve tedavi seçenekleri arttığı için pek tercih edilmemektedir.

Changes in chilhood ITP treatment and follow-up in 2011

Immune thrombocytopenic purpura (ITP) is the most frequent hemorrhagic disease in children. The shortened life of platelets because of immunologic damage (antibodies absorbed by platelets) and insufficient compensatory increased function of the bone marrow result with reduced number of platelets in the peripheral blood. There are three forms of ITP: acute, chronic and persistent. The acute form occurs in 80-90% of cases with bleeding episodes lasting a few days or weeks, but no longer than 6 months, the new 2011 American Society of Hematology guideline increased this period to one year. It is typical for the phenomenon of bleeding that it starts suddenly and without any other sign of illness. The most frequent acute form appears between the second and fourth year, and is occurrence usually after acute viral infections. Children older than 10 years of age, like adults, often have the chronic form associated with other immunologic disorders. Hemorrhagic manifestations include: petechiae, purpura, epistaxis, gastrointestinal and genitourinary bleeding. They depend on the grade of thrombocytopenia, although there is no strict correlation between the number of platelets and volume of bleeding. In cases of acute ITP in children, usually there are two therapeutic options or therapies of choice: corticosteroids and high doses of intravenous immunoglobulin. There are also immunosupressive therapy, anti Rh(D) immunoglobulin, cyclosporine, cytostatics, danazol, rituximab, and thrombopoietin receptor agonists. In cases of distinctive hemorrhagic syndrome there are also indications for platelet transfusion. Nowadays splenectomy is more restricted, because one third of cases is unsuccessful.

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