Kolgaeva E I, Vasilyeva V A, Kuzmina L A, Drokov M Y, Dovydenko M V, Konova Z V, Chebotarev D I, Kovrigina A M, Kamelskih D V, Gaponova T V, Sokolova M A, Subortseva I N, Melikyan A L, Maschan M A, Parovichnikova E N, Savchenko V G
National Research Center for Hematology.
Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology.
Ter Arkh. 2021 Jul 23;93(7):805-810. doi: 10.26442/00403660.2021.07.200948.
Indications of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with primary myelofibrosis are intermediate-2 and high-risk group of DIPSS (Dynamic International Prognostic Scoring System), beginning of the disease in childhood. The other adverse factors affect engraftment and survival after allo-HSCT, example partialy matched donor. But the result of allo-HSCT from matched related donors and result of allo-HSCT from haploidentical donors are comparable. The method for haploidentical hematopoietic stem cell transplantation is T-cell-depletion. This is clinical case of T-cell-depleted haploidentical hematopoietic stem cell transplantation in patient with primary myelofibrosis, the diagnosis was established in childhood.
原发性骨髓纤维化患者进行异基因造血干细胞移植(allo-HSCT)的指征为动态国际预后评分系统(DIPSS)的中-2和高危组、疾病始于儿童期。其他不利因素会影响allo-HSCT后的植入和生存,例如供体部分匹配。但来自匹配相关供体的allo-HSCT结果与来自单倍体相合供体的allo-HSCT结果相当。单倍体相合造血干细胞移植的方法是T细胞去除。这是一例原发性骨髓纤维化患者进行T细胞去除的单倍体相合造血干细胞移植的临床病例,诊断于儿童期确立。