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Int J Hematol. 2015 Nov;102(5):602-10. doi: 10.1007/s12185-015-1869-y. Epub 2015 Sep 26.

Favorable outcome in non-infant children with MLL-AF4-positive acute lymphoblastic leukemia: a report from the Tokyo Children's Cancer Study Group.

Author information

1
Division of Leukemia and Lymphoma, Children's Cancer Center, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan. tomizawa-d@ncchd.go.jp.
2
Division of Transplantation and Cell Therapy, Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan.
3
Department of Pediatrics, Toho University, Tokyo, Japan.
4
Department of Pediatrics and Adolescent Medicine, Juntendo University School of Medicine, Tokyo, Japan.
5
Department of Pediatrics, Yamanashi University, Kofu, Japan.
6
Department of Pediatrics, University of Tsukuba, Tsukuba, Japan.
7
Department of Pediatric Hematology and Oncology Research, National Center for Child Health and Development, Tokyo, Japan.
8
Department of Hematology/Oncology, Saitama Children's Medical Center, Saitama, Japan.
9
Department of Pediatrics, St. Luke's International Hospital, Tokyo, Japan.

Abstract

Unlike acute lymphoblastic leukemia (ALL) in infants, MLL gene rearrangement (MLL-r) is rare in ALL children (≥1 year old). The outcome and optimal treatment options for MLL-r ALL remain controversial. Among the 1827 children enrolled in the Tokyo Children's Cancer Study Group ALL studies L95-14, L99-15, L99-1502, L04-16, and L07-1602 (1995-2009), 25 MLL-r ALL patients (1.3 %) were identified. Their median age and leukocyte count at diagnosis was 2 years old (range 1-15 years) and 27,690/μL (range 1800-1,113,000/μL), respectively. All but one patient achieved complete remission (CR) after induction therapy, and 19 underwent allogeneic hematopoietic stem cell transplantation (HSCT) in first CR according to the protocol. The 5-year event-free survival (EFS) and overall survival (OS) rate were 60.0 % [standard error (SE), 9.7 %] and 64.0 % (SE 9.6 %), respectively. Notably, 9/12 cases with MLL-AF4-positive ALL are alive in continuous CR with a 75.0 % (SE 12.5 %) EFS rate. The causes of treatment failure were as follows: one induction failure, five relapses, and five transplant-related deaths. With intensive chemotherapy and allogeneic HSCT, favorable outcome of children (≥1 year old) with MLL-AF4-positive ALL was observed. However, considering the risk of acute and late toxicities associated with HSCT, its indication should be restricted.

KEYWORDS:

Acute lymphoblastic leukemia; Children; Hematopoietic stem cell transplantation; MLL

PMID:
26410102
DOI:
10.1007/s12185-015-1869-y
[Indexed for MEDLINE]

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