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Spliced MLL fusions: a novel mechanism to generate functional chimeric MLL-MLLT1 transcripts in t(11;19)(q23;p13.3) leukemia

Leukemia volume 21, pages 588–590 (2007)Cite this article

Rearrangements of the MLL (mixed lineage leukemia) gene are associated with pediatric, adult and therapy-related acute leukemias of both myeloid (AML) and lymphoid lineage (ALL).1, 2 Today, 55 partner genes have been characterized at the molecular level and additional 36 genetic loci have been identified by cytogenetics.3 The most common translocation partner genes are AFF1 (AF4), MLLT3 (AF9), MLLT1 (ENL), MLLT10 (AF10), MLLT4 (AF6) and ELL, respectively. These partner genes account for about 80% of all rearrangements of the MLL gene.3, 4

The MLLT1 gene is a frequent translocation partner and accounts for about 5% of all MLL rearrangements. In most cases, the MLLT1 gene is rearranged with the MLL gene via a balanced reciprocal translocation, but also more complex aberrations have been described including three way translocations.5, 6, 7 The t(11;19)(q23;p13.3) has been identified in patients with lymphoid, myeloid and biphenotypic malignancies. Therapy-related translocations between MLL and MLLT1 are very rare, and so far only one t-AML case has been described in the literature.8 Although MLL rearrangements are associated with a dismal outcome, two distinct subsets within the group of MLL-MLLT1 fusions have been identified that seem to have a good prognosis: ALL patients between 1and 9 years and patients with T-lineage ALL.9

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Authors and Affiliations

  1. Institute of Pharmaceutical Biology/ZAFES/DCAL, JWG-University Frankfurt, Biocenter, Frankfurt/Main, Germany

    C Meyer, B Schneider, T Dingermann & R Marschalek

  2. Campus Benjamin Franklin, Charité Universitätsmedizin Berlin, Hindenburgdamm, Berlin, Germany,

    T Burmeister & D Hubert

  3. CCRI, Children's Cancer Research Institute, Kinderspitalgasse, Vienna, Austria

    S Strehl

  4. 1st Deptartment of Internal Medicine, Elisabethinen Hospital, Fadingerstr., Linz, Austria

    O Zach

  5. St Anna Kinderspital, Kinderspitalgasse, Vienna, Austria

    O Haas

  6. ZKI, Medical Faculty III, JWG-University Frankfurt, Frankfurt/Main, Germany

    T Klingebiel

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Correspondence to R Marschalek.

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Meyer, C., Burmeister, T., Strehl, S. et al. Spliced MLL fusions: a novel mechanism to generate functional chimeric MLL-MLLT1 transcripts in t(11;19)(q23;p13.3) leukemia. Leukemia 21, 588–590 (2007). https://doi.org/10.1038/sj.leu.2404542

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