Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children35410.24287/1726-1708-2020-19-2-178-183LITERATURE REVIEWОБЗОР ЛИТЕРАТУРЫHematopoietic stem cell transplantation in patients with transfusion-dependent β-thalassemia. Review articleТрансплантация гемопоэтических стволовых клеток при трансфузионно-зависимой форме β-талассемии. Обзор литературыhttps://orcid.org/0000-0002-9209-7238TaishikhinaI. O.ТайшихинаИ. О.
Russian Federation

Pediatrician Admission Department, Clinical Resident, 

Russia, 117997, Moscow, Samory Mashela st., 1

врач-педиатр приемного отделения, клинический ординатор,

117997, Москва, ГСП-7, ул. Саморы Машела, 1

irina_taishihina@mail.ruhttps://orcid.org/0000-0003-4222-2915LokhmatovaM. E.ЛохматоваМ. Е.
Russian Federation
https://orcid.org/0000-0003-0520-5630ShelikhovaL. N.ШелиховаЛ. Н.
Russian Federation
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России020720201921781830207202002072020Copyright ©; 2020, «D. Rogachev NMRCPHOI»Copyright ©; 2020, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2020«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/354

Thalassemia is the most common form of hereditary anemia from the hemoglobinopathy group. The genetic disorder underlying thalassemia leads to impaired erythrocyte maturation, hemolysis, and the development of ineffective erythropoiesis with erythroid gland hyperplasia in the bone marrow and extramedullary. Regular blood transfusions and chelator therapy are standard therapy for patients with b-thalassemia. This method increases life expectancy, but does not improve its quality and does not cure the disease. Currently, allogeneic hematopoietic stem cell transplantation remains the only radical treatment for thalassemia. The paper discusses the historical aspects of the development of allogeneic hematopoietic stem cell transplantation in the context of transfusion-dependent form of b-thalassemia treatment.

Талассемия – наиболее распространенная форма наследственных анемий из группы гемоглобинопатий. Генетическое расстройство, лежащее в основе талассемии, приводит к нарушению созревания эритроцитов, их гемолизу и развитию неэффективного эритропоэза с гиперплазией эритроидного ростка в костном мозге и экстрамедуллярно. Стандартной терапией больных b-талассемией являются регулярные гемотрансфузии и хелаторная терапия. Этот метод увеличивает продолжительность жизни, но не улучшает ее качество и не излечивает от болезни. В настоящее время единственным радикальным методом лечения талассемии остается аллогенная трансплантация гемопоэтических стволовых клеток. В работе рассмотрены исторические аспекты развития аллогенной трансплантации гемопоэтических стволовых клеток в контексте лечения трансфузионно-зависимой формы b-талассемии.

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