Clinical characteristics of 15 children with juvenile myelomonocytic leukaemia who developed blast crisis: MDS Committee of Japanese Society of Paediatric Haematology/Oncology
Yuko Honda
Department of Paediatrics, University of Occupational and Environmental Health, Kitakyusyu, Japan
Search for more papers by this authorMasahiro Tsuchida
Department of Paediatrics, Ibaraki Children's Hospital, Mito, Japan
Search for more papers by this authorYuji Zaike
Clinical Laboratory, Research Hospital, The Institution of Medical Science, The University of Tokyo, Tokyo, Japan
Search for more papers by this authorAtsuko Masunaga
Department of Diagnostic Pathology, Showa University Fujigaoka Hospital, Yokohama, Japan
Search for more papers by this authorAyami Yoshimi
Department of Paediatrics and Adolescent Medicine, University of Freiburg, Freiburg, Germany
Search for more papers by this authorSeiji Kojima
Department of Paediatrics, Graduate School of Medicine, Nagoya University, Nagoya, Japan
Search for more papers by this authorMasafumi Ito
Department of Pathology, Nagoya Daiichi Red Cross Hospital, Nagoya, Japan
Search for more papers by this authorAkira Kikuchi
Department of Paediatrics, School of Medicine, Teikyo University, Tokyo, Japan
Search for more papers by this authorTatsutoshi Nakahata
Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University, Kyoto, Japan
Search for more papers by this authorCorresponding Author
Atsushi Manabe
Department of Paediatrics, St. Luke's International Hospital, Tokyo, Japan
Correspondence: Atsushi Manabe, Department of Paediatrics, St. Luke's International Hospital, 9-1, Akashi-cho, Chuo-ku, Tokyo 104-8560, Japan.
E-mail: [email protected]
Search for more papers by this authorYuko Honda
Department of Paediatrics, University of Occupational and Environmental Health, Kitakyusyu, Japan
Search for more papers by this authorMasahiro Tsuchida
Department of Paediatrics, Ibaraki Children's Hospital, Mito, Japan
Search for more papers by this authorYuji Zaike
Clinical Laboratory, Research Hospital, The Institution of Medical Science, The University of Tokyo, Tokyo, Japan
Search for more papers by this authorAtsuko Masunaga
Department of Diagnostic Pathology, Showa University Fujigaoka Hospital, Yokohama, Japan
Search for more papers by this authorAyami Yoshimi
Department of Paediatrics and Adolescent Medicine, University of Freiburg, Freiburg, Germany
Search for more papers by this authorSeiji Kojima
Department of Paediatrics, Graduate School of Medicine, Nagoya University, Nagoya, Japan
Search for more papers by this authorMasafumi Ito
Department of Pathology, Nagoya Daiichi Red Cross Hospital, Nagoya, Japan
Search for more papers by this authorAkira Kikuchi
Department of Paediatrics, School of Medicine, Teikyo University, Tokyo, Japan
Search for more papers by this authorTatsutoshi Nakahata
Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University, Kyoto, Japan
Search for more papers by this authorCorresponding Author
Atsushi Manabe
Department of Paediatrics, St. Luke's International Hospital, Tokyo, Japan
Correspondence: Atsushi Manabe, Department of Paediatrics, St. Luke's International Hospital, 9-1, Akashi-cho, Chuo-ku, Tokyo 104-8560, Japan.
E-mail: [email protected]
Search for more papers by this authorSummary
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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