title: De novo acute B-cell leukemia with translocation t(14;18): an entity with a poor prognosis.
abstract: Three cases of de novo acute B-cell lymphoblastic leukemia are presented, all with an unusual phenotype, involvement of translocation t(14;18) and additional chromosomal abnormalities, including translocation t(8;14) and deletion of chromosome 9. In contrast to normal FAB-L2 or FAB-L3 acute lymphoblastic leukemia (ALL), these leukemias did not express cytoplasmatic and membranous immunoglobulin. The combination of translocation t(14;18) and additional chromosomal events on the other chromosome 14 account for the lack of immunoglobulin expression. In one case a low grade follicular lymphoma was found next to a high grade Burkitt type ALL. The translocation t(14;18) takes place as a mistake in the VDJH joining in pre-B cells in the bone marrow. It is proposed that some cases of de novo ALL may arise as a blast crisis induced by genetic events, secondary to the primary t(14;18) translocation. This type of ALL seems to have a poor prognosis.
authors.affiliation: Department of Hematology, University Medical Centre, Leiden, The Netherlands.
authors.forename: M H
authors.initials: MH
authors.lastname: Kramer
authors.forename: S
authors.initials: S
authors.lastname: Raghoebier
authors.forename: G C
authors.initials: GC
authors.lastname: Beverstock
authors.forename: D
authors.initials: D
authors.lastname: de Jong
authors.forename: P M
authors.initials: PM
authors.lastname: Kluin
authors.forename: J C
authors.initials: JC
authors.lastname: Kluin-Nelemans
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D018941:Antigens, CD19; D000943:Antigens, Differentiation / Q000378:metabolism; D000944:Antigens, Differentiation, B-Lymphocyte / Q000378:metabolism; D000951:Antigens, Neoplasm / Q000378:metabolism; D002051:Burkitt Lymphoma / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D002462:Cell Membrane / Q000276:immunology; D002872:Chromosome Deletion; D002883:Chromosomes, Human, Pair 14; D002887:Chromosomes, Human, Pair 18; D002898:Chromosomes, Human, Pair 8; D002899:Chromosomes, Human, Pair 9; D003593:Cytoplasm / Q000276:immunology; D004253:DNA Nucleotidylexotransferase / Q000172:deficiency; D006801:Humans; D007136:Immunoglobulins / Q000378:metabolism; D016130:Immunophenotyping; D008224:Lymphoma, Follicular / Q000235:genetics / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D015260:Neprilysin; D011379:Prognosis; D014178:Translocation, Genetic
chemical_list: D015703:Antigens, CD; D018941:Antigens, CD19; D000943:Antigens, Differentiation; D000944:Antigens, Differentiation, B-Lymphocyte; D000951:Antigens, Neoplasm; D007136:Immunoglobulins; D004253:DNA Nucleotidylexotransferase; D015260:Neprilysin