JSH practical guidelines for hematological malignancies, 2018: I. Leukemia—2. Acute promyelocytic leukemia (APL)

Excerpt

Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia (AML) in which neoplastic proliferation of promyelocytes with unique cellular morphology is observed in bone marrow and peripheral blood. APL is characterized by anemia, infection, and hemorrhage due to suppression of normal hematopoiesis, as well as a strong bleeding tendency due to hyperfibrinolytic disseminated intravascular coagulation (DIC) caused by APL cells. The majority of patients have the PML-RARA chimeric gene produced by t(15;17)(q22:q21).^1,2 APL accounts for 10–15% of AML cases, occurring most commonly in people in their 30–50 s and less commonly in people aged 60 years and older. Secondary APL after anti-cancer therapy is uncommon, but does occur. Treatment outcomes for APL improved dramatically with the introduction of all-trans retinoic acid (ATRA) and arsenic trioxide (ATO), which are molecularly targeted drugs that act on the PML-RARα chimeric protein produced by the chromosomal translocation t(15;17).³ …