title: [Prognosis of 75 patients with juvenile myelomonocytic leukemia: prospective study by MDS committee in the Japanese Society of Pediatric Hematology].
abstract: Juvenile myelomonocytic leukemia (JMML) is a myelodysplastic/myeloproliferative disorder of young children. Because the disease is rare and the diagnosis is difficult, a prospective registration of patients suspected of having JMML with a pathological central review have been conducted by the MDS Committee of the Japanese Society of Pediatric Hematology. Between 1999 and 2006, 75 children with JMML were enrolled and diagnosed through this system. Median age at diagnosis was 20 months (1∼85 months). Cytogenetic abnormalities were detected in 21 patients, including 11 with monosomy 7. The 5-year overall survival (OS) was 60%. Regarding the treatment, 61 of the 75 patients received stem cell transplantation (SCT). Conditioning regimen varied widely, and the source of grafts was bone marrow for 43 patients, peripheral blood for 5, and cord blood for 13. The 5-year OS after SCT was 61%. Notably, patients who received cord blood transplantation had inferior survival than those who received grafts from other sources (38 vs. 68%; P=0.03). Given better recognition of the disease, a multi-center protocol study on SCT, JMML11, is now being planned by the Japanese Pediatric Leukemia/Lymphoma Study Group.
authors.affiliation: Department of Hematology and Oncology, Children's Medical Center, Japanese Red Cross Nagoya First Hospital.
authors.forename: Nao
authors.initials: N
authors.lastname: Yoshida
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Hirabayashi
authors.forename: Shizuka
authors.initials: S
authors.lastname: Watanabe
authors.forename: Yuji
authors.initials: Y
authors.lastname: Zaike
authors.forename: Masahiro
authors.initials: M
authors.lastname: Tsuchida
authors.forename: Ayami
authors.initials: A
authors.lastname: Yoshimi
authors.forename: Atsuko
authors.initials: A
authors.lastname: Masunaga
authors.forename: Yoshitoshi
authors.initials: Y
authors.lastname: Otsuka
authors.forename: Masafumi
authors.initials: M
authors.lastname: Ito
authors.forename: Seiji
authors.initials: S
authors.lastname: Kojima
authors.forename: Tatsutoshi
authors.initials: T
authors.lastname: Nakahata
authors.forename: Atsushi
authors.initials: A
authors.lastname: Manabe
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006405:Hematology; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D054429:Leukemia, Myelomonocytic, Juvenile / Q000401:mortality* / Q000628:therapy; D008297:Male; D054437:Myelodysplastic-Myeloproliferative Diseases; D010372:Pediatrics; D011446:Prospective Studies; D012955:Societies, Medical