OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press10510.17650/1818-8346-2011-6-1-45-55HEMATOPOIETIC STEM CELL TRANSPLANTATIONТРАНСПЛАНТАЦИЯ ГЕМОПОЭТИЧЕСКИХ СТВОЛОВЫХ КЛЕТОК У ПАЦИЕНТОВ С ГЕМОБЛАСТОЗАМИHematopoietic stem cell transplantation in juvenile myelomonocytic leukemia: analyse one centre experience and literature reviewТрансплантация гемопоэтических стволовых клеток при ювенильном миеломоноцитарном лейкозе: анализ опыта одного центра и обзор литературыMaschanM. A.МасчанМ. А.
Russian Federation
mmaschan@yandex.ruKhachatryanL. A.ХачатрянЛ. А.
Russian Federation
SkvortsovaYu. V.СкворцоваЮ. В.
Russian Federation
KurnikovaE. E.КурниковаЕ. Е.
Russian Federation
ShashelevaD. A.ШашелеваД. А.
Russian Federation
BobryninaV. O.БобрынинаВ. О.
Russian Federation
BalashovD. N.БалашовД. Н.
Russian Federation
SkorobogatovaE. V.СкоробогатоваЕ. В.
Russian Federation
BaydildinaD. D.БайдильдинаД. Д.
Russian Federation
NovichkovaG. A.НовичковаГ. А.
Russian Federation
MaschanA. A.МасчанА. А.
Russian Federation
Federal Research Center of Pediatric Hematology, Oncology and ImmunologyФГУ Федеральный научно-клинический центр детской гематологии, онкологии ФГУ и иммунологии Минздравсоцразвития РоссииRussian Children Clinical HospitalРоссийская детская клиническая больница2402201161455524072014240720142011https://oncohematology.abvpress.ru/ongm/article/view/105

Allogeneic hematopoietic stem cell transplantation (HSCT) is the standard curative therapy for juvenile myelomonocytic leukemia (JMML). Seventeen patients with JMML received myeloablative conditioning (busulfan-based — 15, treosulfan-based — 2). Donors included 5 matched related siblings, 8 — matched unrelated volunteer or cord blood (2), 4 — mismatched relatives. Primary engraftment was achieved in 75 %. The rate of acute GVHD grade II–IV was 58 %, grade III–IV — 23 %. Chronic GVHD occurred in 33 % of patients. Five JMML relapses occurred. Relapse-free survival is 66 ± 12 %. Four patients died of transplant-related complications. TRM was 28 ± 12 %.Five patients died of disease progression. Overall survival is 38 ± 13 % with median follow-up of 13 months. A review of most important publications related to HSCT in JMML is provided.

Аллогенная трансплантация гемопоэтических стволовых клеток (ТГСК) — стандарт терапии пациентов с ювенильным миеломоноцитарным лейкозом (ЮММЛ). В статье проанализирован опыт ТГСК в группе 17 пациентов с ЮММЛ. Во всех случаях использовалось миелоаблативное кондиционирование на основе бусульфана (15 пациентов) или треосульфана (2 пациента). Пять трансплантаций выполнено от родственного совместимого донора, 8 — от неродственного донора (2 — пуповинная кровь), 4 — от родственного частично совместимого донора. Первичное приживление достигнуто у 75 % пациентов. Частота острой РТПХ II–IV степени составила 58 %, III–IV степени — 23 %. Частота хронической РТПХ составила 33 %. Рецидив заболевания развился у 5 пациентов. Безрецидивная выживаемость составила 66 ± 12 %. Четыре пациента умерли от осложнений трансплантации. Трансплантационная смертность составила 28 ± 12 %. Пять пациентов умерли от прогрессии основного заболевания. Общая выживаемость составила 38 ± 13 % при медиане наблюдения 13 месяцев. В статье дан обзор основных публикаций, посвященных ТГСК при ЮММЛ.

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