The results of hematopoietic stem cell transplantation in patients with juvenile myelomonocytic leukemia at the Russian Children’s Clinical Hospital ; Результаты трансплантации гемопоэтических стволовых клеток у пациентов с ювенильным миеломоноцитарным лейкозом в Российской детской клинической больнице
| Title: | The results of hematopoietic stem cell transplantation in patients with juvenile myelomonocytic leukemia at the Russian Children’s Clinical Hospital ; Результаты трансплантации гемопоэтических стволовых клеток у пациентов с ювенильным миеломоноцитарным лейкозом в Российской детской клинической больнице |
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| Authors: | E. V. Machneva; V. V. Konstantinova; Yu. V. Skvortsova; А. E. Burya; N. V. Sidorova; L. V. Olkhova; A. V. Mezentseva; O. A. Filina; E. A. Pristanskova; K. I. Kirgizov; E. V. Skorobogatova; Е. Б. Мачнева; В. В. Константинова; Ю. В. Скворцова; А. Е. Буря; Н. В. Сидорова; Л. В. Ольхова; А. В. Мезенцева; О. А. Филина; Е. А. Пристанскова; А. И. Киргизов; Е. В. Скоробогатова |
| Source: | Russian Journal of Pediatric Hematology and Oncology; Том 6, № 2 (2019); 20-29 ; Российский журнал детской гематологии и онкологии (РЖДГиО); Том 6, № 2 (2019); 20-29 ; 2413-5496 ; 2311-1267 ; 10.21682/2311-1267-2019-6-2 |
| Publisher Information: | LTD “Graphica” |
| Publication Year: | 2019 |
| Collection: | Russian Journal of Children Hematology and Oncology / Российский журнал детской гематологии и онкологии (РЖДГиО) |
| Subject Terms: | пуповинная кровь; hematopoietic stem cell transplantation; conditioning; survival; bone marrow; cord blood; трансплантация гемопоэтических стволовых клеток; кондиционирование; выживаемость; костный мозг |
| Description: | Relevance. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only effective treatment method for the majority of patients with juvenile myelomonocytic leukemia (JMML). The authors of the article presented the experience of conducting HSCT in patients with JMML in the Russian Children’s Clinical Hospital.Materials and methods. 55 HSCT for the period from 2003 to 2019 were performed in forty-two patients with JMML. 14 (33.3 %) patients from a related HLA-identical donor were given primary HSCT, 1 (2.4 %) from a related 9/10 HLA-compatible, 16 (38.1 %) – from unrelated HLA-identical, 6 (14.3 %) – from unrelated 9/10 HLA-compatible, 5 (11.9 %) – from haploidentical. The source of hematopoietic stem cells (HSC) in primary HSCT for 22 (52.4 %) patients was bone marrow (BM), for 13 (31.0 %) – peripheral blood stem cells (PBSC), for 4 (9.5 %) – cord blood (CB), for 3 (7.1 %) – BM in combination with CB. Twenty-two (52.4 %) patients received a myeloablative busulfan-containing conditioning regimen, 20 (47.6 %) – treosulfan-containing.Results. The overall survival (OS) of patients for the entire observation period was 53 ± 8.3 %; transplantation lethality (TL) – 21.2 ± 6.8 %, relapse-free survival (RFS) – 72.0 ± 7.7 %, event-free survival (EFS) – 49.4 ± 7.8 %. The factors negatively influencing the results of HSCT in patients with JMML were the progression of the underlying disease at the time of HSCT, incomplete compatibility of the HSC donor, the use of CB as a source of HSC.Conclusion. Indicators of OS, RFS, EFS patients with JMLL after HSCT are low. The reasons for treatment failure are TL, graft failure and relapse after transplantation. To improve the results of treatment of patients with JMML, careful selection of the donor and the source of HSC, the maximum possible reduction in the toxicity of conditioning regimens is necessary. ; Актуальность. Аллогенная трансплантация гемопоэтических стволовых клеток (ТГСК) является единственным эффективным методом терапии для большинства пациентов с ювенильным ... |
| Document Type: | article in journal/newspaper |
| File Description: | application/pdf |
| Language: | Russian |
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| DOI: | 10.21682/2311-1267-2019-6-2-20-29 |
| Availability: | https://journal.nodgo.org/jour/article/view/494; https://doi.org/10.21682/2311-1267-2019-6-2-20-29 |
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| Accession Number: | edsbas.5B7C4D7F |
| Database: | BASE |