title: A classification based on T cell selection-related phenotypes identifies a subgroup of childhood T-ALL with favorable outcome in the COALL studies.
abstract: During T cell selection in the thymic cortex more than 90% of the thymocytes are eliminated by apoptosis. Based on this biology, we propose to define blasts of T cell acute lymphoblastic leukemia (ALL) with the phenotype of cortical thymocytes (CD1+ and/or CD4+ 8+) as selection-related (SR) and all other T-ALL immunophenotypes as non-selection-related (NSR). The COALL cooperative treatment studies for childhood ALL offer a tool to study the outcome in T-ALL subgroups as children with T-ALL are allocated uniformly to the high risk arm of the protocol. In the COALL-85, -89 and -92 protocols, 39/83 cases presented as SR and 44/83 cases as NSR. Five-year event-free survival of SR phenotype is significantly better compared to the NSR group (0.87 +/- 0.06 vs 0.66 +/- 0.07, log rank test, P = 0.01). T-ALL with SR phenotype is a distinct subgroup of leukemia with excellent prognosis under a high risk treatment protocol.
authors.affiliation: Department of Pediatric Hematology, University of Düsseldorf, Germany.
authors.forename: T
authors.initials: T
authors.lastname: Niehues
authors.forename: P
authors.initials: P
authors.lastname: Kapaun
authors.forename: D O
authors.initials: DO
authors.lastname: Harms
authors.forename: S
authors.initials: S
authors.lastname: Burdach
authors.forename: C
authors.initials: C
authors.lastname: Kramm
authors.forename: D
authors.initials: D
authors.lastname: Körholz
authors.forename: G
authors.initials: G
authors.lastname: Janka-Schaub
authors.forename: U
authors.initials: U
authors.lastname: Göbel
mesh_terms: D000293:Adolescent; D015703:Antigens, CD / Q000032:analysis*; D000951:Antigens, Neoplasm / Q000032:analysis*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000008:administration & dosage; D002490:Central Nervous System / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D017637:Clonal Deletion; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D003520:Cyclophosphamide / Q000008:administration & dosage; D003561:Cytarabine / Q000008:administration & dosage; D003630:Daunorubicin / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D005858:Germany / Q000453:epidemiology; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000145:classification* / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy; D017254:Leukemic Infiltration; D008297:Male; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D015337:Multicenter Studies as Topic; D014411:Neoplastic Stem Cells / Q000276:immunology / Q000473:pathology*; D011239:Prednisolone / Q000008:administration & dosage; D012074:Remission Induction; D012306:Risk; D016176:T-Lymphocyte Subsets / Q000276:immunology / Q000473:pathology*; D013713:Teniposide / Q000008:administration & dosage; D013866:Thioguanine / Q000008:administration & dosage; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D015703:Antigens, CD; D000951:Antigens, Neoplasm; D003561:Cytarabine; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide; D013713:Teniposide; D011239:Prednisolone; D015122:Mercaptopurine; D001215:Asparaginase; D013866:Thioguanine; D008727:Methotrexate; D003630:Daunorubicin