| 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 α/δ 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 × 109/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/lyrnphoma 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. |
| Keywords: |
adolescent; cancer survival; child; clinical article; child, preschool; human cell; disease course; review; antigen expression; t lymphocyte; phenotype; cell growth; prevalence; chromosomes, human, pair 8; acute lymphoblastic leukemia; central nervous system; lymphoma, t-cell; infant; lymphoma; chromosome translocation; bone marrow cell; leukocyte count; chromosome aberrations; lymphocytic infiltration; childhood leukemia; karyotyping; t cell leukemia; receptors, antigen, t-cell, alpha-beta; antigens, differentiation; leukemia cell line; chromosome 14q; chromosome 8q; t lymphocyte receptor alpha chain; t lymphocyte receptor delta chain; receptors, antigen, t-cell, gamma-delta; chromosomes, human, pair 14; leukemia, t-cell, acute; chromosome disorders; t lymphocyte receptor gene; cell oncogene; human; male; female; priority journal; cell antigen; support, non-u.s. gov't; support, u.s. gov't, p.h.s.; translocation (genetics); t lymphoblast
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