BIRLAMCHI MIYELOFIBROZNING ASORATLARI VA ULARNI DAVOLASH TAKTIKASI
##article.subject##:
birlamchi miyelofibroz, o'sma intoksikatsiyasi, splenomegaliya, anemiya, yuqumli asoratlar, gemorragik sindrom, ekstramedullar qon yaratish o'choqlari, tromboz, blast transformatsiyasi, siydik kislotasi diatezi, gemosideroz##article.abstract##
Birlamchi miyelofibroz (BMF) kam uchraydigan qonning tizimli kasalligi bo’lib, birinchi marta aniqlangan bemorlar soni yiliga taxminan
1:100000 aholini tashkil qiladi. Ilgari ushbu kasallikni tavsiflash uchun agnogen miyeloid metaplaziya, surunkali idiopatik miyelofibroz, osteomiyelofibroz, subleykemik miyeloz sinonimlari qo’llanilgan.
Birlamchi miyelofibrozni klinik kechishida eng ko'p uchraydigan asoratlar o'sma intoksikatsiyasi, splenomegaliya, anemiya, yuqumli asoratlar, trombotsitopeniya va gemorragik sindrom, ekstramedullar qon yaratish o'choqlari, tromboz, blast transformatsiyasi, siydik kislotasi diatezi (ikkilamchi podagra), ikkilamchi gemosideroz bo’lishi mumkin.
Ushbu kasallikni o‘rganish davomida quyidagi natijalar aniqlab olindi. Birinchi marta miyeloproliferativ kasallikning mustaqil nozologik shakli sifatida 1951 yilda William Dameshek idiopatik yoki agnogen miyeloid metaplaziya deb nomlagan. Keyinchalik kasallikning leykotsitoz, splenomegaliya va suyak iligi fibrozi bilan bog'liqligi turli mamlakatlarda birlamchi osteoskleroz/ osteomiyelofibroz, agnogen miyeloid metaplaziya, surunkali idiopatik miyelofibroz, osteomiyelofibroz, subleykemik miyeloz deb ta'riflangan. Kasallikni, ya’ni birlamchi miyelofibrozni davolashda tarkibida eritrotsitlar mavjud gemokomponentlar transfuziyalaridan foydalanish bemorning ahvolini tezda yaxshilashga va anemik sindromning namoyon bo'lishining kamayishiga olib keladi. Shu bilan birga, organizmda temirni yo‘q qilishning faol mexanizmlari yo‘qligi va uni jigarda saqlash imkoniyati cheklanganligi sababli transfuziyalardan uzoq muddat foydalanish, transfuziyalar soni 20-25 dozadan ortiq bo‘lganda, a’zolar va to‘qimalarda temirning to‘planishi bilan tavsiflanadigan ikkilamchi gemosideroz rivojlanishiga olib keladi.
Bemorlarda gemosideroz rivojlanish darajasi yuqori bo‘lishi mumkin, chunki surunkali anemiya fonida oshqozon-ichak traktidan temirning so'rilishi kuchayadi. Oksidlanish – tiklanish holatining buzilishi, lipidlarning perekisli oksidlanishining rag’batlantirilishi hujayralarning zararlanishiga va ichki a’zolarning disfunktsiyasiga olib kelishi isbotlangan.
Maqolada ushbu asoratlarning profilaktikasi va davolash bo'yicha tavsiyalar keltirilgan.
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Статья поступила в редакцию 03.03.2024; одобрена после рецензирования 20.04.2024; принята к публикации 26.04.2024.
The article was submitted 03.03.2024; approved after reviewing 20.04.2024; accepted for publication 26.04.2024.
Информация об авторах:
Абдиев Каттабек Махматович-к.м.н.доцент. Самаркандский Государственный медицинский университет. Е-mail: sammi@sammi.uz, https://orcid.org/0000-0001-5468-9657
Источники финансирования: Работа не имела специального финансирования.
Конфликт интересов: Авторы декларируют отсутствие явных и потенциальных конфликтов интересов, связанных с публикацией настоящей статьи.
Вклад авторов:
Абдиев Каттабек Махматович — идеологическая концепция работы, редактирование статьи; сбор и анализ источников литературы, написание текста.
Information about the authors:
Kattabek Makhmutovich Abdiev-PhD, Associate Professor. Samarkand State Medical University. E-mail: sammi@sammi.uz , https://orcid.org/0000-0001-5468-9657
Sources of funding: The work did not receive any specific funding.
Conflict of interest: The authors declare no explicit or potential conflicts of interest associated with the publication of this article.
Contribution of the authors:
Kattabek Makhmutovich Abdiev — ideological concept of the work, editing the article; collection and analysis of literature sources, writing the text.
