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De novo acute B cell leukemia/lymphoma with t(14;18).
Stamatoullas, A; Buchonnet, G; Lepretre, S; Lenain, P; Lenormand, B; Duval, C; Callat, M P; Gaulard, P; Bastard, C; Tilly, H.
Afiliação
  • Stamatoullas A; Département d'Hematologie, Centre Henri Becquerel, Rouen, France.
Leukemia ; 14(11): 1960-6, 2000 Nov.
Article em En | MEDLINE | ID: mdl-11069032
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.
Assuntos
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Translocação Genética / Cromossomos Humanos Par 14 / Cromossomos Humanos Par 18 / Linfoma de Células B / Linfoma de Burkitt Tipo de estudo: Prognostic_studies Idioma: En Revista: Leukemia Assunto da revista: HEMATOLOGIA / NEOPLASIAS Ano de publicação: 2000 Tipo de documento: Article País de afiliação: França
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Translocação Genética / Cromossomos Humanos Par 14 / Cromossomos Humanos Par 18 / Linfoma de Células B / Linfoma de Burkitt Tipo de estudo: Prognostic_studies Idioma: En Revista: Leukemia Assunto da revista: HEMATOLOGIA / NEOPLASIAS Ano de publicação: 2000 Tipo de documento: Article País de afiliação: França