A rare “common” acute lymphoblastic leukemia with t(17;19)(q22;p13) with extremely poor prognosis

DOI
  • Inaba Toshiya
    Department of Molecular Oncology, Research Institute for Radiation Biology and Medicine, Hiroshima University

Bibliographic Information

Other Title
  • 17;19転座型ALL：留意すべき極めて治療抵抗性のcommon ALL

Abstract

<p>Hematologists should consider t(17;19)-positive ALL because patients with this rare type of common ALL have extremely poor prognosis when administered standard chemotherapy, even in combination with stem cell transplantation. TCF3::HLF (E2A-HLF) fusion transcription factor, produced by 17;19 translocation, protects leukemic cells from apoptosis, causing the extremely refractory nature of ALL harboring this translocation despite a relatively low WBC count at diagnosis. Clinicians must pay attention to the chromosomal analysis report because patients with 17;19 translocation are frequently misdiagnosed as having a normal karyotype. TCF3::HLF aberrantly induces the expression of many genes, including those encoding apoptosis regulators, LMO2, CD33, and ANNEXIN II. Consequently, more than half the patients with t(17;19)-ALL express CD33 on the surface of leukemic cells and have hypercalcemia or coagulopathy, all of which are relatively rare in common ALL. Thus, RT-PCR to detect TCF3::HLF chimeric transcript should be performed for patients with common ALL with normal karyotype, particularly for those who have the above-mentioned clinical features. Patients positive for TCF3::HLF chimeric fusion transcript should undergo powerful cutting-edge treatments, including CAR-T cell therapy and blinatumomab.</p>

Journal

Keywords

Details 詳細情報について

  • CRID
    1390016526854648320
  • DOI
    10.11412/jspho.60.199
  • ISSN
    21895384
    2187011X
  • Text Lang
    ja
  • Data Source
    • JaLC
  • Abstract License Flag
    Disallowed

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