Онкогематология (Jul 2014)

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

  • 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

DOI
https://doi.org/10.17650/1818-8346-2011-6-1-45-55
Journal volume & issue
Vol. 6, no. 1
pp. 45 – 55

Abstract

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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.

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