Irsiy trombotsitopatiyalarning klinik-laborator xususiyatlari
Keywords:
irsiy trombotsitopatiyalar, gemorragik sindrom, Glansman Trombasteniyasi, trombotsitlar morfologiyasi, optik agregometriya, Bernard - Soulie trombotsitodistrofiyasi, Marfan sindromiAbstract
Trombotsitopatiyalar - bu trombotsitlar soni normal bo‘lganda ularning sifat jihatidan pastligi natijasida yuzaga keladigan gemostazning irsiy va orttirilgan kasalliklari guruhi. Bolalardagi trombotsitopatiyalar mayda shikastlanishlar, turli darajadagi qon ketish (burun, oshqozon-ichak, bachadon), anemiya bilan petekhiya va gematomalarning shakllanishiga moyillik bilan namoyon bo‘ladi. Bugungi kunda ITP diagnostikasi uchun odatiy usul - bu ma’lum bir trombotsit nuqsonini tekshirishga imkon bermaydigan optik agregometriya (OA). T.Quiroga (Gematologiya, 2012) ma’lumotlariga ko‘ra, OA, trombotsitlarni morfologik o‘rganish va koagulogrammani qo‘llashda qon ketishining sababi mikrosirkulyatsiya turidagi gemorragik sindromli bemorlarning 49-71 foizida aniqlanmaydi. Bu esa boshqa diagnostika usullaridan foydalanish zarurligini taqozo etadi.
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