{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T06:33:12Z","timestamp":1789540392030,"version":"build-2803163510"},"reference-count":25,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2014,3,4]],"date-time":"2014-03-04T00:00:00Z","timestamp":1393891200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2014,6]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Juvenile myelomonocytic leukaemia (<jats:styled-content style=\"fixed-case\">JMML<\/jats:styled-content>) 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 <jats:styled-content style=\"fixed-case\">JMML<\/jats:styled-content> were reported to the Myelodysplastic Syndrome Committee in Japan between 1989 and 2007; 15 of them (9\u00b78%) had 20% or more blasts in the bone marrow (blast crisis) during the disease course. Blast crisis occurred during observation without therapy (<jats:italic>n<\/jats:italic>\u00a0=\u00a03) or with oral 6\u2010mercaptopurine treatment (<jats:italic>n<\/jats:italic>\u00a0=\u00a09) and in relapse after haematopoietic stem cell transplantation (<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>;<jats:italic> n<\/jats:italic>\u00a0=\u00a03). 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 <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content> 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\u2010related complication in one patient. In summary, patients with blast crisis have poor prognosis and can be cured only by <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>. The emergence of monosomy 7 and complex karyotype may be characteristic of blast crisis in a substantial subset of children.<\/jats:p>","DOI":"10.1111\/bjh.12796","type":"journal-article","created":{"date-parts":[[2014,3,4]],"date-time":"2014-03-04T12:55:01Z","timestamp":1393937701000},"page":"682-687","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Clinical characteristics of 15 children with juvenile myelomonocytic leukaemia who developed blast crisis: <scp>MDS<\/scp> Committee of Japanese Society of Paediatric Haematology\/Oncology"],"prefix":"10.1111","volume":"165","author":[{"given":"Yuko","family":"Honda","sequence":"first","affiliation":[{"name":"Department of Paediatrics University of Occupational and Environmental Health Kitakyusyu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiro","family":"Tsuchida","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Ibaraki Children's Hospital Mito Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuji","family":"Zaike","sequence":"additional","affiliation":[{"name":"Clinical Laboratory Research Hospital The Institution of Medical Science The University of Tokyo Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsuko","family":"Masunaga","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Showa University Fujigaoka Hospital Yokohama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayami","family":"Yoshimi","sequence":"additional","affiliation":[{"name":"Department of Paediatrics and Adolescent Medicine University of Freiburg Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seiji","family":"Kojima","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Graduate School of Medicine Nagoya University Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masafumi","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of Pathology Nagoya Daiichi Red Cross Hospital Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Kikuchi","sequence":"additional","affiliation":[{"name":"Department of Paediatrics School of Medicine Teikyo University Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsutoshi","family":"Nakahata","sequence":"additional","affiliation":[{"name":"Department of Clinical Application Center for iPS Cell Research and Application Kyoto University Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Manabe","sequence":"additional","affiliation":[{"name":"Department of Paediatrics St. Luke's International Hospital Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,3,4]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1182\/blood.V90.2.479","article-title":"Juvenile myelomonocytic leukemia","volume":"90","author":"Aric\u00f2 M.","year":"1997","journal-title":"Blood"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2009.24.4426"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(1984)54:4<675::AID-CNCR2820540415>3.0.CO;2-Q"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2008.08.022"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1999.01435.x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1210361"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2013.01.003"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07104.x"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-05-1944"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2010.08525.x"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-01-198416"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V93.2.459"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2402407"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2404106"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-09-046649"},{"key":"e_1_2_8_17_1","first-page":"3534","article-title":"Chronic myelomonocytic leukemia in childhood: a retrospective analysis of 110 cases. 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