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The first case of acute lymphoblastic leukemia with the e19a2 BCR–ABL1 transcript: imatinib therapy followed by unrelated donor transplantation induces a durable molecular response

Leukemia volume 25, pages 366–367 (2011)Cite this article

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The Philadelphia chromosome (Ph) is generated by a balanced reciprocal translocation t(9;22)(q34;q11.2), in which the ABL1 and BCR genes are disrupted to form a BCR–ABL1 fusion gene. In most patients with chronic myeloid leukemia (CML), and in approximately one-third of patients with Ph-positive acute lymphoblastic leukemia (ALL), the breakpoint within the BCR gene is located in the major breakpoint cluster region (M-bcr), leading to formation of e13a2 (b2a2) or e14a2 (b3a2) fusion transcripts encoding a p210BCR−ABL1 fusion protein. In the remaining patients with Ph-positive ALL, and in a few patients with CML, the breakpoint is located in the minor breakpoint cluster region, thereby resulting in production of the e1a2 fusion transcript and p190BCR−ABL1 fusion protein. A very rare breakpoint is located distally to the M-bcr region between exons 19 and 20, defined as the micro breakpoint cluster region, which produces an e19a2 fusion transcript encoding a p230BCR−ABL1 fusion protein. The e19a2 transcript was initially reported in neutrophilic-CML patients with benign clinical courses. However, this finding has subsequently been challenged by the observation that the e19a2 transcript was also present in all stages of classical CML and in acute myeloid leukemia.1, 2, 3, 4

Here, to the best of our knowledge, we report on the first case of Ph-positive ALL with the e19a2 transcript at presentation. The patient showed hematological and cytogenetic complete remission (CR) with a reduction in BCR–ABL1 transcript level after imatinib plus conventional chemotherapy, and, subsequently, achieved molecular CR after matched unrelated donor transplantation.

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Acknowledgements

We wish to acknowledge the financial support of the Industry-Academic Cooperation Research Foundation made in the program year of 2009.

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Authors and Affiliations

  1. Department of Oncology, College of Medicine, The Catholic University of Korea, Seoul, Korea

    E K Jeon

  2. Department of Laboratory Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea

    J Lim & M Kim

  3. Department of Hematology, Catholic Blood and Marrow Transplantation Center, College of Medicine, The Catholic University of Korea, Seoul, Korea

    S-A Yahng, S-E Lee, B-S Cho, Y-J Kim, H-J Kim, C-K Min, S-G Cho, D-W Kim, J-W Lee, W-S Min, C-W Park & S Lee

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  1. E K Jeon
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Correspondence to S Lee.

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Jeon, E., Lim, J., Kim, M. et al. The first case of acute lymphoblastic leukemia with the e19a2 BCR–ABL1 transcript: imatinib therapy followed by unrelated donor transplantation induces a durable molecular response. Leukemia 25, 366–367 (2011). https://doi.org/10.1038/leu.2010.265

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