JSH guideline for tumors of hematopoietic and lymphoid tissues: leukemia: 2. Acute promyelocytic leukemia (APL)

Excerpt

Acute promyelocytic leukemia (APL) is referred to as M3 in the FAB classification and APL with t(15;17)(q22;q12); PML-RARA in the 2008 WHO classification. It accounts for 10–15% of acute myeloid leukemia (AML) cases, occurring most commonly in younger people in their 30–50 s and less commonly in people aged 60 years and older [1]. Treatment for APL differs greatly from that for other acute leukemias because all-trans retinoic acid (ATRA) and arsenic trioxide (ATO), which act on the PML-RARα fusion protein, have been established as effective. In the early phases of APL, patients exhibit hyperfibrinolytic disseminated intravascular coagulation (DIC), which makes them prone to fatal organ hemorrhage. However, if DIC can be controlled, cure rates are relatively better than for other types of AML, with the complete remission rate 80% to 90% or higher and disease-free survival (DFS) rate 60–80% or higher [2]. In summary, APL is a form of leukemia that can be effectively treated with targeted therapy against disease-specific molecules. …