title: De novo acute B cell leukemia/lymphoma with t(14;18).
abstract: The t(14;18)(q32;q21) translocation is the most common translocation in B cell malignancies being found in 80% of follicular lymphomas and about 20% of diffuse large B cell lymphomas. Only rare cases of de novo acute B cell lymphoblastic leukemia with t(14;18) have been described. We describe five cases of this entity which appears to have very homogeneous clinical, phenotypic and genotypic features. None of these patients had prior history of follicular lymphoma. The disease was characterized by acute clinical features with nodal and/or extranodal disease, massive bone marrow infiltration and rapid increase of circulating blast cells of mature B cell phenotype. All patients disclosed complex chromosomal and molecular abnormalities involving at least the BCL-2 and c-MYC genes. Furthermore, three patients had evidence of BCL-6 involvement and one patient had a p53 mutation. Despite intensive chemotherapy, including for two patients allogeneic bone marrow transplantation in first complete remission, all patients died within a few months. Neuro-meningeal relapse occurred in three of the five patients in spite of neuro-meningeal prophylaxis. De novo leukemia/lymphoma with t(14;18) is a rare entity with a very poor prognosis. Whether early bone marrow transplant could modify the natural history of the disease remains to be determined. An intensive neuro-meningeal prophylaxis appears to be mandatory in these patients.
authors.affiliation: Département d'Hematologie, Centre Henri Becquerel, Rouen, France.
authors.forename: A
authors.initials: A
authors.lastname: Stamatoullas
authors.forename: G
authors.initials: G
authors.lastname: Buchonnet
authors.forename: S
authors.initials: S
authors.lastname: Lepretre
authors.forename: P
authors.initials: P
authors.lastname: Lenain
authors.forename: B
authors.initials: B
authors.lastname: Lenormand
authors.forename: C
authors.initials: C
authors.lastname: Duval
authors.forename: M P
authors.initials: MP
authors.lastname: Callat
authors.forename: P
authors.initials: P
authors.lastname: Gaulard
authors.forename: C
authors.initials: C
authors.lastname: Bastard
authors.forename: H
authors.initials: H
authors.lastname: Tilly
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001752:Blast Crisis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D016026:Bone Marrow Transplantation; D002051:Burkitt Lymphoma / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics / Q000648:ultrastructure*; D002887:Chromosomes, Human, Pair 18 / Q000235:genetics / Q000648:ultrastructure*; D004273:DNA, Neoplasm / Q000235:genetics; D004268:DNA-Binding Proteins / Q000235:genetics; D018450:Disease Progression; D005260:Female; D019254:Genes, bcl-2; D016259:Genes, myc; D016158:Genes, p53; D006801:Humans; D016130:Immunophenotyping; D017254:Leukemic Infiltration; D016393:Lymphoma, B-Cell / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D008224:Lymphoma, Follicular / Q000188:drug therapy / Q000235:genetics / Q000473:pathology / Q000628:therapy; D008297:Male; D008578:Meninges / Q000473:pathology; D008875:Middle Aged; D009360:Neoplastic Cells, Circulating; D011379:Prognosis; D011518:Proto-Oncogene Proteins / Q000235:genetics; D051560:Proto-Oncogene Proteins c-bcl-6; D012008:Recurrence; D016879:Salvage Therapy; D014157:Transcription Factors / Q000235:genetics; D014178:Translocation, Genetic; D017211:Treatment Failure
chemical_list: D000970:Antineoplastic Agents; D004273:DNA, Neoplasm; D004268:DNA-Binding Proteins; D011518:Proto-Oncogene Proteins; D051560:Proto-Oncogene Proteins c-bcl-6; D014157:Transcription Factors