Frontiers in Oncology (Nov 2021)

Molecular Pathogenesis of BCR-ABL-Negative Atypical Chronic Myeloid Leukemia

  • Diletta Fontana,
  • Carlo Gambacorti-Passerini,
  • Carlo Gambacorti-Passerini,
  • Rocco Piazza,
  • Rocco Piazza,
  • Rocco Piazza

DOI
https://doi.org/10.3389/fonc.2021.756348
Journal volume & issue
Vol. 11

Abstract

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Atypical chronic myeloid leukemia is a rare disease whose pathogenesis has long been debated. It currently belongs to the group of myelodysplastic/myeloproliferative disorders. In this review, an overview on the current knowledge about diagnosis, prognosis, and genetics is presented, with a major focus on the recent molecular findings. We describe here the molecular pathogenesis of the disease, focusing on the mechanisms of action of the main mutations as well as on gene expression profiling. We also present the treatment options focusing on emerging targeted therapies.

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