Moyamoya syndrome following childhood acute lymphoblastic leukemia.
BACKGROUND
Long-term survivors of childhood acute lymphoblastic leukemia (ALL) sometimes suffer from adverse long-term sequelae. We analyzed the incidence, clinical course and prognosis of moyamoya syndrome (MoS) following childhood ALL.


METHODS
A total of 1,846 ALL patients were treated with four consecutive TCCSG ALL protocols (L84-11, L89-12, L92-13, and L95-14) between 1984 and 1999. We surveyed the MoS cases among these patients in the follow-up studies.


RESULTS
Six patients with MoS were identified: four boys and two girls whose ages ranged from 2 years and 1 month (abbreviated as "2y1m") to 14y 1 m at diagnosis of ALL. None of the patients had central nervous system (CNS) leukemia. All six patients received prophylactic cranial irradiation with a dosage of 18 or 24 Gy. Although one patient died of brain infarction due to MoS, no leukemic relapse was observed in the group. The cumulative incidence of MoS in our series was 0.46 +/- 0.02% at 8 years. Among several clinical characteristics, use of cranial irradiation was the only factor that appeared to be significantly related to the development of MoS.


CONCLUSIONS
MoS occurs with increased frequency in children treated for ALL, and might be associated with cranial irradiation. Prophylactic cranial irradiation should be used cautiously in ALL patients who can be cured by other CNS-directed therapies.
Kikuchi
Akira
A
Division of Hematology/Oncology, Saitama Children's Medical Center, Saitama, Japan. a1091069@pref.saitama.lg.jp
Maeda
Miho
M
Hanada
Ryoji
R
Okimoto
Yuri
Y
Ishimoto
Koichi
K
Kaneko
Takashi
T
Ikuta
Koichiro
K
Tsuchida
Masahiro
M
D000293:Adolescent; D018943:Anthracyclines / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000008:administration & dosage; D002544:Cerebral Infarction / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D016371:Cranial Irradiation / Q000009:adverse effects*; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004198:Disease Susceptibility; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008297:Male; D009072:Moyamoya Disease / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000235:genetics; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications* / Q000188:drug therapy / Q000532:radiotherapy; D011239:Prednisolone / Q000008:administration & dosage; D011788:Quality of Life; D012074:Remission Induction; D014750:Vincristine / Q000008:administration & dosage
D018943:Anthracyclines; D014750:Vincristine; D003907:Dexamethasone; D003520:Cyclophosphamide; D011239:Prednisolone; D001215:Asparaginase