title: Acute mixed lineage leukemia in children: the experience of St Jude Children's Research Hospital.
abstract: To characterize the biology and optimal therapy of acute mixed-lineage leukemia in children, we reviewed the pathologic and clinical features, including response to therapy, of 35 patients with mixed-lineage leukemia. The majority of cases (91%) had blasts cells that simultaneously expressed either T-lineage plus myeloid markers (T/myeloid, n = 20) or B-lineage plus myeloid markers (B/myeloid, n = 12). Overall survival rates for the B/myeloid and T/myeloid subgroups were not significantly different from each other or from the rate for acute myeloid leukemia (AML) but were inferior to the outcome in children with acute lymphoblastic leukemia (ALL). Patients who failed to achieve complete remission with AML-directed therapy could often be induced with a regimen of prednisone, vincristine, and L-asparaginase. Analysis of gene-expression patterns identified a subset of biphenotypic leukemias that did not cluster with T-cell ALL, B-progenitor ALL, or AML. We propose that treatment for biphenotypic leukemia begin with one course of AML-type induction therapy, with a provision for a switch to lymphoid-type induction therapy with a glucocorticoid, vincristine, and L-asparaginase if the patient responds poorly. We also suggest that hematopoietic stem cell transplantation is often not required for cure of these patients.
authors.affiliation: Department of Oncology, St Jude Children's Research Hospital, Memphis, TN 38105-2794, USA. jeffrey.rubnitz@stjude.org
authors.forename: Jeffrey E
authors.initials: JE
authors.lastname: Rubnitz
authors.forename: Mihaela
authors.initials: M
authors.lastname: Onciu
authors.forename: Stanley
authors.initials: S
authors.lastname: Pounds
authors.forename: Sheila
authors.initials: S
authors.lastname: Shurtleff
authors.forename: Xueyuan
authors.initials: X
authors.lastname: Cao
authors.forename: Susana C
authors.initials: SC
authors.lastname: Raimondi
authors.forename: Frederick G
authors.initials: FG
authors.lastname: Behm
authors.forename: Dario
authors.initials: D
authors.lastname: Campana
authors.forename: Bassem I
authors.initials: BI
authors.lastname: Razzouk
authors.forename: Raul C
authors.initials: RC
authors.lastname: Ribeiro
authors.forename: James R
authors.initials: JR
authors.lastname: Downing
authors.forename: Ching-Hon
authors.initials: CH
authors.lastname: Pui
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000473:pathology; D002648:Child; D020869:Gene Expression Profiling; D006801:Humans; D016130:Immunophenotyping; D015456:Leukemia, Biphenotypic, Acute / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D015470:Leukemia, Myeloid, Acute; D022423:Myeloid Cells / Q000473:pathology; D017410:Practice Guidelines as Topic; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy; D012189:Retrospective Studies; D015996:Survival Rate; D013601:T-Lymphocytes / Q000473:pathology; D016896:Treatment Outcome