Efficacy allogeneic hematopoietic stem cell transplantation with different conditioning regimens in pediatric myelodysplastic syndrome

Osipova, Anna A.; Семенова, Е. В.; Морозова, Е. В.; Быкова, Т А; Ovechkina, Varvara N.; Боровкова, А С; Kozhokar, P.V.; Паина, О. В.; Разумова, С. В.; Babenko, Elena V.; Алянский, А Л; Гиндина, Т. Л.; Barkhatov, Ildar; Смирнов, Б. И.; Зубаровская, Л. С.; Афанасьев, Б. В.

doi:10.17650/2311-1267-2017-4-2-70-77

Cited by 1 publication

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“…Общая выживаемость (ОВ) детей с МДС/МПН и МДС после алло-ТГСК, по данным нашей клиники, не зависит от интенсивности режима кондиционирования (миелоаблативный (МАК) против режима со сниженной интенсивностью доз (РИК)) и составляет до 50 %, при этом выявлена зависимость от уровня бластов в КМ на момент выполнения трансплантации -58 % и 17 % при уровне бластов < 20 % и > 20 % соответственно (рис. 11) [37].…”

Section: терапия при миелопролиферативной неоплазии/ миелодиспластичеunclassified

Pediatric myelodysplastic syndrome

Владимирович¹,

Зубаровская²

2018

Ross. ž. det. gematol. onkol.

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Pediatric myelodysplastic syndrome (MDS) are a heterogeneous group of clonal disorders often occur in the context of inherited bone marrow failure syndromes, acquired aplastic anemia or gene predisposition. Germ line syndromes predisposing individuals to develop familial MDS or acute myeloid leukemia have recently been identified – mutations in RUNX1, ANKRD, GATA2, ETV6, SRP72, DDX41. Juvenile myelomonocytic leukemia (JMML) occurs in context of inherited and somatic mutations PTPN11, KRAS, NRAS, CBL, NF1. In pathogenesis of these disorders there are a several factors – hypermethylation, clonal hematopoiesis/cytopenia of undetermined significance, disturbances of bone marrow microenvironment, telomeres, immune mechanisms. Allogeneic hematopoietic stem cell transplantation is the main method of MDS and JMML treatment but it is necessary to take into account special indications for refractory cytopenia (infections, dependence on blood transfusions) and be careful for JMML with CBL mutation.

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Section: терапия при миелопролиферативной неоплазии/ миелодиспластичеunclassified

Pediatric myelodysplastic syndrome

Владимирович¹,

Зубаровская²

2018

Ross. ž. det. gematol. onkol.

View full text Add to dashboard Cite

show abstract

Efficacy allogeneic hematopoietic stem cell transplantation with different conditioning regimens in pediatric myelodysplastic syndrome

Cited by 1 publication

References 10 publications

Pediatric myelodysplastic syndrome

Pediatric myelodysplastic syndrome

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