Acute promyelocytic leukemia

Definition:

Acute promyelocytic leukemia (APL) is the most malignant form of acute myeloid leukemia (AML) with a severe bleeding tendency and a fatal course of only weeks. Morphologically, it is identified as AML-M3 by the FAB classification. APL is characterized by a balanced reciprocal translocation between chromosomes 15 and 17, which results in the fusion between the promyelocytic leukemia (PML) gene and retinoic acid receptor alpha (RARA). The majority of APL patients present with the classic PML-RARA translocation. However, approximately 5% of patients with APL have a non- characteristic translocation. Other chimeric proteins have been observed including PLZF-RARA, NUMA1-RARA, and so on.

References:

[1]. Joaquin J Jimenez, et al. Acute promyelocytic leukemia (APL): a review of the literature. Oncotarget. 2020 Mar 17;11(11):992-1003. [Content Brief]

[2]. R A Wells, et al. Fusion of retinoic acid receptor alpha to NuMA, the nuclear mitotic apparatus protein, by a variant translocation in acute promyelocytic leukaemia. Nat Genet. 1997 Sep;17(1):109-13. [Content Brief]

[3]. S J Chen, et al. Rearrangements of the retinoic acid receptor alpha and promyelocytic leukemia zinc finger genes resulting from t(11;17)(q23;q21) in a patient with acute promyelocytic leukemia. J Clin Invest. 1993 May;91(5):2260-7. [Content Brief]

[4]. Zhen-Yi Wang, et al. Acute promyelocytic leukemia: from highly fatal to highly curable. Blood. 2008 Mar 1;111(5):2505-15. [Content Brief]

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