A classification based on T cell selection-related phenotypes identifies a subgroup of childhood T-ALL with favorable outcome in the COALL studies

Leukemia. 1999 Apr;13(4):614-7. doi: 10.1038/sj.leu.2401382.

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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Antigens, CD / analysis*
  • Antigens, Neoplasm / analysis*
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Asparaginase / administration & dosage
  • Central Nervous System / pathology
  • Child
  • Child, Preschool
  • Clonal Deletion*
  • Combined Modality Therapy
  • Cranial Irradiation
  • Cyclophosphamide / administration & dosage
  • Cytarabine / administration & dosage
  • Daunorubicin / administration & dosage
  • Dexamethasone / administration & dosage
  • Disease-Free Survival
  • Doxorubicin / administration & dosage
  • Female
  • Follow-Up Studies
  • Germany / epidemiology
  • Humans
  • Immunophenotyping*
  • Infant
  • Leukemia-Lymphoma, Adult T-Cell / classification*
  • Leukemia-Lymphoma, Adult T-Cell / drug therapy
  • Leukemia-Lymphoma, Adult T-Cell / immunology
  • Leukemia-Lymphoma, Adult T-Cell / mortality
  • Leukemia-Lymphoma, Adult T-Cell / pathology
  • Leukemia-Lymphoma, Adult T-Cell / radiotherapy
  • Leukemic Infiltration
  • Male
  • Mercaptopurine / administration & dosage
  • Methotrexate / administration & dosage
  • Multicenter Studies as Topic
  • Neoplastic Stem Cells / immunology
  • Neoplastic Stem Cells / pathology*
  • Prednisolone / administration & dosage
  • Remission Induction
  • Risk
  • T-Lymphocyte Subsets / immunology
  • T-Lymphocyte Subsets / pathology*
  • Teniposide / administration & dosage
  • Thioguanine / administration & dosage
  • Treatment Outcome
  • Vincristine / administration & dosage

Substances

  • Antigens, CD
  • Antigens, Neoplasm
  • Cytarabine
  • Vincristine
  • Dexamethasone
  • Doxorubicin
  • Cyclophosphamide
  • Teniposide
  • Prednisolone
  • Mercaptopurine
  • Asparaginase
  • Thioguanine
  • Methotrexate
  • Daunorubicin