Hematopoietic stem cell transplantation in juvenile myelomonocytic leukemia: analyse one centre experience and literature review

• Main Authors: M. A. Maschan, L. A. Khachatryan, Yu. V. Skvortsova, E. E. Kurnikova, D. A. Shasheleva, V. O. Bobrynina, D. N. Balashov, E. V. Skorobogatova, D. D. Baydildina, G. A. Novichkova, A. A. Maschan
• Format: Article
• Language: Russian
• Published: ABV-press 2014-07-01
• Series: Onkogematologiâ
• Subjects: hematopoietic stem cell transplantation; juvenile myelomonocytic leukemia; transplant-related mortality; relapse
• Online Access: https://oncohematology.abvpress.ru/ongm/article/view/105
• ISSN: 1818-8346

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.