Clin Hematol Int. 2021 Jul 19;3(4):142-152. doi: 10.2991/chi.k.210703.001. eCollection 2021 Dec.

History of Acute Promyelocytic Leukemia.

Clinical hematology international

Miguel A Sanz, Eva Barragán

PMID: 34938986 PMCID: PMC8690702 DOI: 10.2991/chi.k.210703.001

Abstract

In this article, we discuss the history of acute promyelocytic leukemia (APL) from the pre-therapeutic era, which began after its recognition by Hillestad in 1947 as a nosological entity, to the present day. It is a paradigmatic history that has transformed the "most malignant leukemia form" into the most curable one. The identification of a balanced reciprocal translocation between chromosomes 15 and 17, resulting in fusion between the promyelocytic leukemia gene and the retinoic acid receptor alpha, has been crucial in understanding the mechanisms of leukemogenesis, and responsible for the peculiar response to targeted therapy with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO). We review the milestones that marked successive therapeutic advances, beginning with the introduction of the first successful chemotherapy in the early 1970s, followed by a subsequent incorporation of ATRA and ATO in the late 1980s and early 1990s which have revolutionized the treatment of this disease. Over the past two decades, treatment optimization has relied on the combination of ATRA, ATO, and chemotherapy according to risk-adapted approaches, which together with improvements in supportive therapy have paved the way for cure for most patients with APL.

© 2021 International Academy for Clinical Hematology. Publishing services by Atlantis Press International B.V.

Keywords: Acute promeloctic leukemia; all-trans retinoic acid; arsenic trioxide

Conflict of interest statement

Publication Types

• Journal Article
• Review