title: Acute lymphoblastic leukemia with t(4;11) in children 1 year and older: The 'big sister' of the infant disease?
abstract: The t(4;11)-positive acute lymphoblastic leukemia (ALL) is a rare disease in children above the age of 1 year. We studied the clinical and biological characteristics in 32 consecutively diagnosed childhood cases (median age 10.0 years, range 1.0-17.1 years). Immunophenotyping revealed a pro-B and a pre-B stage in 24 and eight cases, respectively. IGH genes were rearranged in 84% of leukemias with a predominance of incomplete DJ(H) joints. Whereas IGK-Kde and TCRD rearrangements were rare, TCRG rearrangements were present in 50% of cases and involved mainly Vgamma11 or Vgamma9 together with a Jgamma1.3./2.3 gene segment, an unusual combination among t(4;11)-negative B-cell precursor ALL. Oligoclonality was found in about 30% as assessed by heterogeneous IGH and TCRG rearrangements. Our data are in line with transformation of a precursor cell at an early stage of B-cell development but retaining the potential to differentiate to the pre-B cell stage in vivo. Although a distinct difference between infant and older childhood cases with t(4;11) became evident, no age-related biological features were found within the childhood age group. In contrast to infants with t(4;11)-positive ALL, childhood cases had a relatively low cumulative incidence of relapse of 25% at 3.5 years with BFM-based high-risk protocols.
authors.affiliation: St. Anna Kinderspital, Department of Pediatric Hematology/Oncology, Vienna, Austria.
authors.forename: G
authors.initials: G
authors.lastname: Mann
authors.forename: G
authors.initials: G
authors.lastname: Cazzaniga
authors.forename: V H J
authors.initials: VH
authors.lastname: van der Velden
authors.forename: T
authors.initials: T
authors.lastname: Flohr
authors.forename: E
authors.initials: E
authors.lastname: Csinady
authors.forename: M
authors.initials: M
authors.lastname: Paganin
authors.forename: A
authors.initials: A
authors.lastname: Schrauder
authors.forename: A M
authors.initials: AM
authors.lastname: Dohnal
authors.forename: M
authors.initials: M
authors.lastname: Schrappe
authors.forename: A
authors.initials: A
authors.lastname: Biondi
authors.forename: H
authors.initials: H
authors.lastname: Gadner
authors.forename: J J M
authors.initials: JJ
authors.lastname: van Dongen
authors.forename: E R
authors.initials: ER
authors.lastname: Panzer-Grümayer
mesh_terms: D002880:Chromosomes, Human, Pair 11; D002894:Chromosomes, Human, Pair 4; D005803:Genes, Immunoglobulin; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D016130:Immunophenotyping; D007223:Infant; D009367:Neoplasm Staging; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D011948:Receptors, Antigen, T-Cell / Q000235:genetics; D014178:Translocation, Genetic
chemical_list: D007143:Immunoglobulin Heavy Chains; D011948:Receptors, Antigen, T-Cell