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Clinical characteristics of 15 children with juvenile myelomonocytic leukaemia who developed blast crisis: MDS Committee of Japanese Society of Paediatric Haematology/Oncology.

Authors
  • Honda, Yuko
  • Tsuchida, Masahiro
  • Zaike, Yuji
  • Masunaga, Atsuko
  • Yoshimi, Ayami
  • Kojima, Seiji
  • Ito, Masafumi
  • Kikuchi, Akira
  • Nakahata, Tatsutoshi
  • Manabe, Atsushi
Type
Published Article
Journal
British Journal of Haematology
Publisher
Wiley (Blackwell Publishing)
Publication Date
Jun 01, 2014
Volume
165
Issue
5
Pages
682–687
Identifiers
DOI: 10.1111/bjh.12796
PMID: 24588411
Source
Medline
Keywords
License
Unknown

Abstract

Juvenile myelomonocytic leukaemia (JMML) is a rare haematopoietic stem cell disease of early childhood, which can progress to blast crisis in some children. A total of 153 children diagnosed with JMML were reported to the Myelodysplastic Syndrome Committee in Japan between 1989 and 2007; 15 of them (9·8%) had 20% or more blasts in the bone marrow (blast crisis) during the disease course. Blast crisis occurred during observation without therapy (n = 3) or with oral 6-mercaptopurine treatment (n = 9) and in relapse after haematopoietic stem cell transplantation (HSCT; n = 3). Six patients had a complex karyotype (5 including monosomy 7) and an additional three patients had isolated monosomy 7 at blast crisis. Seven patients received HSCT after blast crisis and four of them achieved remission. Eleven out of the 15 patients died; the cause of death was disease progression in 10 patients and transplant-related complication in one patient. In summary, patients with blast crisis have poor prognosis and can be cured only by HSCT. The emergence of monosomy 7 and complex karyotype may be characteristic of blast crisis in a substantial subset of children.

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