Clinical features and prognostic implications of TCF3-PBX1 and ETV6-RUNX1 in adult acute lymphoblastic leukemia.

作者: T. Burmeister , N. Gokbuget , S. Schwartz , L. Fischer , D. Hubert

DOI: 10.3324/HAEMATOL.2009.011346

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摘要: Background The t(9;22) and t(4;11) chromosomal translocations, which generate the BCR-ABL MLL-AF4 fusion genes, define high-risk subtypes of acute lymphoblastic leukemia in adults. However, prognostic impact other rarer genes is less well established adult than childhood form.Design Methods In context German Multicenter Therapy Study Group for Adult Acute Lymphoblastic Leukemia (GMALL) we used reverse transcriptase polymerase chain reaction to investigate 441 cases BCR-ABL- MLL-AF4-negative B-precursor TCF3-PBX1 (E2A-PBX1) ETV6-RUNX1 (TEL-AML1) transcripts generated by t(1;19)(q23;p13.3) t(12;21)(p13;q22) translocations. Both are well-known molecular alterations pediatric they have favorable implications.Results We identified 23 patients with ten ETV6-RUNX1. contrast previous reports found no significant difference overall survival between TCF3-PBX1-positive -negative patients. At 2 years after diagnosis all ETV6-RUNX1-positive were alive continuous complete remission, but their long-term outcome was negatively affected late relapses. exhibited a characteristic CD34−/CD33− mostly cyIg+ immunophenotype. only occurred under 35 old associated significantly lower white blood count.Conclusions suggestions, do not appear worse negative counterparts. had very performance status during first few groups characterized distinct clinicobiological features facilitate diagnostic identification.

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