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Acute Promyelocytic Leukemia

Special Issue Information

Dear Colleagues,

Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML) ,cytogenetically characterized by a balanced reciprocal translocation between chromosomes 15 and 17, which results in the fusion between promyelocytic leukemia (PML) gene and retinoic acid receptor-α (RARα). Because of a severe bleeding tendency often resulting in an early fatal course, APL has historically been considered as one of the most fatal forms of acute leukemia. Advances in therapy, including anthracycline- and cytarabine-based chemotherapy, have significantly improved the outcome. Additionally, the introduction of all-trans retinoic acid (ATRA) and, more recently, the development of arsenic trioxide (ATO)-containing regimens has transformed APL to the most curable form of AML in adults. Treatment with these new agents introduced the concept of cure through targeted therapy. With the revolutionary ATRA–ATO combination therapies, chemotherapy may now safely be omitted, at least in low-risk APL patients. Concomitantly, expert panels have recommended that molecular remission should be considered a therapeutic objective in APL, and molecular response has been adopted as a study endpoint in modern clinical trials.

APL is the best example of how targeted therapies can trigger definitive cures. ATRA–ATO combination therapy is the first model of molecular target-based therapy and represents, with the introduction of tyrosine kinase inhibitor therapy in chronic myeloid leukemia, one of the major advances in therapy for hematological disease over the last three decades, having paved the way in which cancer should be treated. However, open issues remain, notably regarding the type of ATO schedule and the refinements in strategies in high-risk APL. Other areas of ongoing needs include efforts to decrease the early death rate, to define APL treatments in children and in case of post-ATO relapses, and to introduce a new drug formula with the attempt to give only oral ambulatory therapy.

This Special Issue will highlight the current state of the art in APL with future prospects for improving therapies and recall the constant progresses made over the last decades that have yielded APL status to evolve from highly fatal to highly curable.

Dr. Xavier Thomas
Dr Maël Heiblig
Guest Editors

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Keywords

  • acute promyelocytic leukemia
  • prognosis
  • treatment
  • all-trans retinoic acid
  • arsenic trioxide
  • chemotherapy
  • PML-RARA

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Cancers - ISSN 2072-6694