title: Transitional pre-B-cell acute lymphoblastic leukemia of childhood is associated with favorable prognostic clinical features and an excellent outcome: a Pediatric Oncology Group study.
abstract: The presenting characteristics and survival of children with the newly recognized transitional cell pre-B immunophenotype of acute lymphoblastic leukemia (ALL) are compared with those of children with pre-B ALL to determine the clinical significance of the new phenotype. Patients with transitional pre-B ALL (n = 17), defined by lymphoblasts expressing cytoplasmic and surface mu heavy chains without kappa or lambda light chains, have lower initial leukocyte counts (p = 0.02) and a higher frequency of DNA indexes > 1.16 (p < 0.001) than patients with pre-B ALL (n = 501), whether or not cases with the unfavorable prognostic (1;19) translocation are included in the analysis. Patients with transitional pre-B ALL lack FAB L3 morphology, bulky extramedullary disease, surface kappa or lambda chains, and the (8;14), (8;22), and (2;8) translocations, features that characterize the syndrome of B-cell ALL. The 4-year relapse-free survival result for children with transitional pre-B ALL appears better than that for children with pre-B ALL (93.3 +/- 17% versus 72.9% +/- 4.6%), but this difference is not statistically significant. We conclude that patients with transitional pre-B ALL have a very favorable prognosis in the context of the therapy used in this study.
authors.affiliation: Department of Hematology-Oncology, St Jude Children's Research Hospital, Memphis, TN 38101.
authors.forename: M
authors.initials: M
authors.lastname: Koehler
authors.forename: F G
authors.initials: FG
authors.lastname: Behm
authors.forename: J
authors.initials: J
authors.lastname: Shuster
authors.forename: W
authors.initials: W
authors.lastname: Crist
authors.forename: M
authors.initials: M
authors.lastname: Borowitz
authors.forename: A T
authors.initials: AT
authors.lastname: Look
authors.forename: D
authors.initials: D
authors.lastname: Head
authors.forename: A J
authors.initials: AJ
authors.lastname: Carroll
authors.forename: V
authors.initials: V
authors.lastname: Land
authors.forename: P
authors.initials: P
authors.lastname: Steuber
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003593:Cytoplasm / Q000276:immunology; D005260:Female; D006801:Humans; D007148:Immunoglobulin mu-Chains / Q000032:analysis; D016130:Immunophenotyping; D007223:Infant; D008297:Male; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000145:classification* / Q000276:immunology / Q000401:mortality; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000145:classification* / Q000276:immunology / Q000401:mortality; D011379:Prognosis; D015996:Survival Rate; D014481:United States
chemical_list: D007148:Immunoglobulin mu-Chains