title: Pediatric leukemia/lymphoma with t(8;14)(q24;q11).
abstract: A variety of chromosomal translocations occur in pediatric T-cell acute lymphoblastic leukemia (T-ALL) in which a cellular oncogene or growth-related gene is translocated to the alpha/delta locus of the T-cell receptor gene. The t(8;14)(q24;q11) has been described at the cytogenetic and molecular level, but the disease associated with this translocation has not been defined clinically. Fifteen pediatric cases of leukemia/lymphoma with a t(8;14)(q24;q11) chromosomal translocation were collected from previous publications and institutional records. The estimated prevalence of this abnormality among all cases of ALL was 1%. The t(8;14)(q24;q11) disease was characterized by male predominance (10/15), a median age of 5.5 years (range 1.8-17 years), high white blood cell count (median 95 x 10(9)/l), central nervous system infiltration (4/11), bulky extramedullary leukemia (10/11), and T-cell immunophenotype (12/15). The median event-free survival was 4 months, and the median survival, 11 months. Seven cell lines with t(8;14)(q24;q11) were established from six of the cases; four were T-lymphoblastic, one was T-lymphoblastic, but expressed myeloid-related antigens, and two were predominantly myeloid. t(8;14)(q24;q11) leukemia/lymphoma and other ALLs involving 13(q11) have in common a high tumor burden, early spread to extramedullary sites, a propensity to form T-lymphoblastic or T-myeloid cell lines and, usually, an aggressive clinical course.
authors.affiliation: Division of Oncology, Children's Hospital of Philadelphia, PA 19104.
authors.forename: B J
authors.initials: BJ
authors.lastname: Lange
authors.forename: S C
authors.initials: SC
authors.lastname: Raimondi
authors.forename: N
authors.initials: N
authors.lastname: Heerema
authors.forename: P C
authors.initials: PC
authors.lastname: Nowell
authors.forename: J
authors.initials: J
authors.lastname: Minowada
authors.forename: P E
authors.initials: PE
authors.lastname: Steinherz
authors.forename: E B
authors.initials: EB
authors.lastname: Arenson
authors.forename: R
authors.initials: R
authors.lastname: O'Connor
authors.forename: D
authors.initials: D
authors.lastname: Santoli
mesh_terms: D000293:Adolescent; D000943:Antigens, Differentiation / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations / Q000235:genetics*; D025063:Chromosome Disorders; D002883:Chromosomes, Human, Pair 14; D002898:Chromosomes, Human, Pair 8; D005260:Female; D006801:Humans; D007223:Infant; D007621:Karyotyping; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000235:genetics*; D016399:Lymphoma, T-Cell / Q000235:genetics* / Q000276:immunology; D008297:Male; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics*; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics*; D014178:Translocation, Genetic
chemical_list: D000943:Antigens, Differentiation; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta