title: How I treat mixed-phenotype acute leukemia.
abstract: Mixed-phenotype acute leukemia (MPAL) encompasses a heterogeneous group of rare leukemias in which assigning a single lineage of origin is not possible. A variety of different terms and classification systems have been used historically to describe this entity. MPAL is currently defined by a limited set of lineage-specific markers proposed in the 2008 World Health Organization monograph on classification of tumors of hematopoietic and lymphoid tissues. In adult patients, MPAL is characterized by relative therapeutic resistance that may be attributed in part to the high proportion of patients with adverse cytogenetic abnormalities. No prospective, controlled trials exist to guide therapy. The limited available data suggest that an "acute lymphoblastic leukemia-like" regimen followed by allogeneic stem-cell transplant may be advisable; addition of a tyrosine kinase inhibitor in patients with t(9;22) translocation is recommended. The role of immunophenotypic and genetic markers in guiding chemotherapy choice and postremission strategy, as well as the utility of targeted therapies in non-Ph-positive MPALs is unknown.
authors.affiliation: Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA.
authors.forename: Ofir
authors.initials: O
authors.lastname: Wolach
authors.affiliation: Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA.
authors.forename: Richard M
authors.identifier: http://orcid.org/0000-0002-7526-2633
authors.initials: RM
authors.lastname: Stone
mesh_terms: D000208:Acute Disease; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology; D014408:Biomarkers, Tumor / Q000378:metabolism*; D002869:Chromosome Aberrations; D003131:Combined Modality Therapy; D000069439:Dasatinib; D005434:Flow Cytometry; D016044:Fusion Proteins, bcr-abl / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D007938:Leukemia / Q000235:genetics / Q000378:metabolism / Q000628:therapy*; D008875:Middle Aged; D022423:Myeloid Cells / Q000378:metabolism / Q000473:pathology; D011743:Pyrimidines / Q000008:administration & dosage; D033581:Stem Cell Transplantation / Q000379:methods*; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D013844:Thiazoles / Q000008:administration & dosage; D014184:Transplantation, Homologous
chemical_list: C581634:BCR-ABL1 fusion protein, human; D014408:Biomarkers, Tumor; D011743:Pyrimidines; D013844:Thiazoles; D016044:Fusion Proteins, bcr-abl; D000069439:Dasatinib