title: Clinical significance of early T-cell precursor acute lymphoblastic leukaemia: results of the Tokyo Children's Cancer Study Group Study L99-15.
abstract: Early T-cell precursor acute lymphoblastic leukaemia (ETP-ALL) is a recently identified subtype of T-ALL with distinctive gene expression and cell marker profiles, poor response to chemotherapy and a very high risk of relapse. We determined the reliability of restricted panel of cell markers to identify EPT-ALL using a previously classified cohort. Then, we applied the cell marker profile that best discriminated ETP-ALL to a cohort of 91 patients with T-ALL enrolled in the Tokyo Children's Cancer Study Group L99-15 study, which included allogeneic stem cell transplantation (allo-SCT) for patients with poor prednisone response. Five of the 91 patients (5·5%) met the ETP-ALL criteria. There were no significant differences in presenting clinical features between these and the remaining 86 patients. Response to early remission induction therapy was inferior in ETP-ALL as compared with T-ALL. The ETP-ALL subgroup showed a significantly poorer event-free survival (4-year rate; 40%) than the T-ALL subgroup (70%, P=0·014). Of note, three of four relapsed ETP-ALL patients survived after allo-SCT, indicating that allo-SCT can be effective for this drug-resistant subtype of T-ALL.
authors: Department of Paediatrics, School of Medicine, University of Yamanashi, Yamanashi, Japan. tinukai@yamanashi.ac.jp
authors: Takeshi
authors: T
authors: Inukai
authors: Nobutaka
authors: N
authors: Kiyokawa
authors: Dario
authors: D
authors: Campana
authors: Elaine
authors: E
authors: Coustan-Smith
authors: Akira
authors: A
authors: Kikuchi
authors: Miyuki
authors: M
authors: Kobayashi
authors: Hiroyuki
authors: H
authors: Takahashi
authors: Katsuyoshi
authors: K
authors: Koh
authors: Atsushi
authors: A
authors: Manabe
authors: Masaaki
authors: M
authors: Kumagai
authors: Koichiro
authors: K
authors: Ikuta
authors: Yasuhide
authors: Y
authors: Hayashi
authors: Masahiro
authors: M
authors: Tsuchida
authors: Kanji
authors: K
authors: Sugita
authors: Akira
authors: A
authors: Ohara
mesh_terms: D000293:Adolescent; D015703:Antigens, CD / Q000032:analysis; D000951:Antigens, Neoplasm / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D019008:Drug Resistance, Neoplasm; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D007564:Japan / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000145:classification / Q000188:drug therapy / Q000453:epidemiology* / Q000601:surgery; D011241:Prednisone / Q000008:administration & dosage; D012074:Remission Induction; D033581:Stem Cell Transplantation; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D015703:Antigens, CD; D000951:Antigens, Neoplasm; D011241:Prednisone