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
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Comparative Study
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Research Support, Non-U.S. Gov't
MeSH terms
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Adolescent
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Antigens, CD / analysis*
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Antigens, Neoplasm / analysis*
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Asparaginase / administration & dosage
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Central Nervous System / pathology
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Child
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Child, Preschool
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Clonal Deletion*
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Combined Modality Therapy
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Cranial Irradiation
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Cyclophosphamide / administration & dosage
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Cytarabine / administration & dosage
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Daunorubicin / administration & dosage
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Dexamethasone / administration & dosage
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Disease-Free Survival
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Doxorubicin / administration & dosage
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Female
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Follow-Up Studies
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Germany / epidemiology
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Humans
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Immunophenotyping*
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Infant
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Leukemia-Lymphoma, Adult T-Cell / classification*
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Leukemia-Lymphoma, Adult T-Cell / drug therapy
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Leukemia-Lymphoma, Adult T-Cell / immunology
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Leukemia-Lymphoma, Adult T-Cell / mortality
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Leukemia-Lymphoma, Adult T-Cell / pathology
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Leukemia-Lymphoma, Adult T-Cell / radiotherapy
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Leukemic Infiltration
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Male
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Mercaptopurine / administration & dosage
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Methotrexate / administration & dosage
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Multicenter Studies as Topic
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Neoplastic Stem Cells / immunology
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Neoplastic Stem Cells / pathology*
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Prednisolone / administration & dosage
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Remission Induction
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Risk
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T-Lymphocyte Subsets / immunology
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T-Lymphocyte Subsets / pathology*
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Teniposide / administration & dosage
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Thioguanine / administration & dosage
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Treatment Outcome
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Vincristine / administration & dosage
Substances
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Antigens, CD
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Antigens, Neoplasm
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Cytarabine
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Vincristine
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Dexamethasone
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Doxorubicin
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Cyclophosphamide
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Teniposide
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Prednisolone
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Mercaptopurine
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Asparaginase
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Thioguanine
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Methotrexate
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Daunorubicin