Acute Myeloid Leukemia in Adults: Mast Cell Leukemia and Other Mast Cell Neoplasms

Abstract

Overview Acute myeloid leukemia (AML) is a heterogeneous disease characterized by unbridled proliferation of myeloid stem cells resulting in bone marrow failure and death without effective therapy. The median age of AML is approximately 70; predisposing factors include inherited mutations, exposure to industrial solvents, ionizing radiation, or certain chemotherapy given for other malignancies or conditions. The disease results from the accumulation of mutations in pathways that promote cellular proliferation or impairing differentiation. Prognosis is based on a combination of host and disease factors, especially diagnostic cytogenetics and genomics. In younger fit patients, therapy consists of myelosuppressive induction chemotherapy to minimize disease burden and allow restoration of normal hematopoiesis. Postremission chemotherapy with additional myelo‐intense chemotherapy and/or allogeneic stem cell transplant is required to potentiate cure. In older and less fit patients (perhaps those with adverse prognosis) the current standard is repetitive cycles of a hypomethylating agent or low‐dose cytarabine plus the BCL‐2 inhibitor, venetoclax. The application of molecularly targeted therapies at both diagnosis and relapse may change the natural history of the disease, the management of which requires a comprehensive integration of disparate fields including social work, nutrition, pharmacy, blood bank, and medical oncology.