ESSENТSIAL TROMBOTSITEMIYA - BOLALAR VA O‘SMIRLARDAGI ASOSIY TAHLILLAR
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essenтsial trombotsitemiya, bolalar, o‘smirlik, fon Villebrand omil faolligi, surunkali mieloproliferativ kasallik, JAK 2, MPL, CALR##article.abstract##
Essenтsial trombotsitemiya (ET) bolalik davrida kam uchraydigan kasallikdir. Ushbu kasallikda proliferatsiya bilan bog‘liq suyak iligidagi atipik megakariotsitlar hisobiga periferik qonda trombotsitlar sonining ko‘payishi kuzatiladi.
Bolalik va o‘smirlik davridagi bemorlarda ET kursining klinik xususiyatlarini va terapiyaga javobni tahlil qilinadi. 19 yoshgacha bo‘lgan 23 bemorda ET kursi tahlil qilindi. ET tashxisi JSST mezonlari asosida o‘rnatildi, unda klinik malumotlarni baholash, laboratoriya testlari (umumiy klinik tadqiqotlar; suyak iligining morfologik, genetik, gistologik tadqiqotlari), instrumental tadqiqotlar va davolanishga javobni baholash kiradi.
Kasallikning o‘rtacha namoyon bo‘lish yoshi 9 yil 9 oyni, o‘rtacha ko‘rsatkichi 9 yil 6 oyni tashkil etdi. 13 (52%) bemorda organomegaliya aniqlangan, ulardan 7 nafarida (37,5%) izolyatsiya qilingan splenomegaliya va 6 nafarida (37,5%) gepatosplenomegaliya aniqlangan. Qon ketishi ristotsetin kofaktor faolligining sezilarli pasayishi bilan 6 (19,4%) bemorda sodir bo‘ldi. Fon Villebrand omili (15% dan ko‘p bo‘lmagan), trombotsitoz (> 2000 × 10 9 / l). 10 (38,7%) bemorlarda mikrosirkulyatsiya buzilishi (bosh og‘rishi, bosh aylanishi, ovqat hazm qilishni buzilishi), ularning yarmida trombotsitoz 1000-2000 × 10 9/l bo‘lgan. JAK 2 V6 17F mutatsiyasi 3 (9,7%) bemorda, CALR gen mutatsiyasi 7 (29,0%), MPL geni - 1 (3,2%) bemorda, qolgan 13 (58,1%) bemorda odatiy drayver genlar aniqlandi. Translokatsiya t (12;12) 1 (3,2%) bemorda aniqlandi. Yosh bemorlarda bitta komponentli sitoreduktiv terapiyaga to‘liq yoki qisman javob 70-80% ni tashkil etdi. Terapiya davomida to‘liq javob darajasi anagrelid va interferonlar gidroksikarbamid terapiyasiga qaraganda yuqori (mos ravishda 42,9%, 47,4% va 0%). Gidroksikarbamid bolalarda birinchi darajali terapiyada qo‘llanilmagan.
ET bilan og‘rigan bemorlarning pediatrik populyatsiyasida "uch karra salbiy" guruh ustunlik qildi, bu esa ikkilamchi trombotsitoz bilan differensial tashxisni qiyinlashtirdi. Bolalardagi kattalar populyatsiyasiga nisbatan qon ketish xavfi yuqori, bu juda og‘ir trombotsitozli bemorlarning ko‘pligi bilan bog‘liq.
Gemorragik sindromning rivojlanishi bilan, davolanishga javob bermaydigan mikrosirkulyatsiya buzilishlari antiagregant agentlari, birinchi darajali sitoreduktiv terapiya sifatida ANA terapiyasi INF va HU ga nisbatan ustunlik berishini tavsiya etamiz.
Farmakokinetik xususiyatlariga ko‘ra anagrelid emas, balki interferon va gidroksikarbamid va anagrelid bilan mielofibroz rivojlanishining potentsial xavfi yuqori.
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