Features and outcome of neonatal leukemia in Japan: experience of the Japan infant leukemia study group.
BACKGROUND
Neonatal leukemia characterized by early stem cell origin and extramedullary infiltration in the first 4 weeks of life is rare. We analyzed the features and outcome of neonatal leukemia in Japan to establish an appropriate treatment strategy for this rare disorder.


METHODS
Patients with infant leukemia registered and treated in the Japan Infant Leukemia Study between 1996 and 2001 were analyzed.


RESULTS
Among 162 infant leukemia patients, 11 exhibited neonatal leukemia; frequencies for all infant leukemias were 6.9% (8/116) for acute lymphoblastic leukemia (ALL) and 7.3% (3/41) for acute myeloid leukemia (AML). Positive MLL gene rearrangement was observed in all eight patients with ALL; a single patient with AML displayed germline configuration. Acute monoblastic leukemia was apparent in all three patients with AML (M5a in the FAB classification). Most of the patients demonstrated hepatoplenomegaly and hyperleukocytosis at diagnosis. Cutaneous and central nervous system involvement were detected in half of the patients. Four patients (one with AML, and three with ALL) have survived following stem cell transplantation (SCT); however, growth impairment related to SCT was observed in these patients.


CONCLUSIONS
These results suggest an improvement attributable to treatment of neonatal leukemia. International-based collaborative studies are necessary to investigate the biology of this condition and to establish appropriate therapeutic strategies.
Ishii
Eiichi
E
Department of Pediatrics, Saga University, Nabeshima, Saga, Japan. ishiei@med.saga-u.ac.jp
Oda
Megumi
M
Kinugawa
Naoko
N
Oda
Takanori
T
Takimoto
Tetsuya
T
Suzuki
Nobuhiro
N
Kosaka
Yoshiyuki
Y
Ohara
Akira
A
Ogawa
Atsushi
A
Ishii
Mutsuo
M
Sakata
Naoki
N
Okamura
Takayuki
T
Koike
Kenichi
K
Kojima
Seiji
S
Horibe
Keizo
K
Mizutani
Shuki
S
D000208:Acute Disease; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D005260:Female; D011495:Histone-Lysine N-Methyltransferase; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D007951:Leukemia, Myeloid / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D008297:Male; D051788:Myeloid-Lymphoid Leukemia Protein / Q000235:genetics; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D012042:Registries; D033581:Stem Cell Transplantation; D015996:Survival Rate; D016896:Treatment Outcome
C497422:KMT2A protein, human; D051788:Myeloid-Lymphoid Leukemia Protein; D011495:Histone-Lysine N-Methyltransferase