title: Mixed-phenotype acute leukemia: clinical and laboratory features and outcome in 100 patients defined according to the WHO 2008 classification.
abstract: The features of 100 mixed-phenotype acute leukemias (MPALs), fulfilling WHO 2008 criteria, are documented. Myeloid and T-lineage features were demonstrated by cytoplasmic myeloperoxidase and CD3; B-lineage features were demonstrated by at least 2 B-lymphoid markers. There were 62 men and 38 women; 68% were adults. Morphology was consistent with acute lymphoblastic leukemia (ALL; 43%), acute myeloid leukemia (AML; 42%), or inconclusive (15%). Immunophenotyping disclosed B + myeloid (59%), T + myeloid (35%), B + T (4%), or trilineage (2%) combinations. Cytogenetics evidenced t(9;22)/(Ph(+)) (20%), 11q23/MLL rearrangements (8%), complex (32%), aberrant (27%), or normal (13%) karyotypes. There was no correlation between age, morphology, immunophenotype, or cytogenetics. Response to treatment and outcome were available for 67 and 70 patients, respectively; 27 received ALL, 34 AML, 5 a combination of ALL + AML therapy, and 1 imatinib. ALL treatment induced a response in 85%, AML therapy in 41%; 3 of 5 patients responded to the combination therapy. Forty (58%) patients died, 33 of resistant disease. Overall median survival was 18 months and 37% of patients are alive at 5 years. Age, Ph(+), and AML therapy were predictors for poor outcome (P < .001; P = .002; P = .003). MPAL is confirmed to be a poor-risk disease. Adults and Ph(+) patients should be considered for transplantation in first remission.
authors.affiliation: Department of Haemato-Oncology, Royal Marsden Hospital, Institute of Cancer Research, London, UK. estella.matutes@icr.ac.uk
authors.forename: Estella
authors.initials: E
authors.lastname: Matutes
authors.forename: Winfried F
authors.initials: WF
authors.lastname: Pickl
authors.forename: Mars
authors.initials: M
authors.lastname: Van't Veer
authors.forename: Ricardo
authors.initials: R
authors.lastname: Morilla
authors.forename: John
authors.initials: J
authors.lastname: Swansbury
authors.forename: Herbert
authors.initials: H
authors.lastname: Strobl
authors.forename: Andishe
authors.initials: A
authors.lastname: Attarbaschi
authors.forename: Georg
authors.initials: G
authors.lastname: Hopfinger
authors.forename: Sue
authors.initials: S
authors.lastname: Ashley
authors.forename: Marie Christine
authors.initials: MC
authors.lastname: Bene
authors.forename: Anna
authors.initials: A
authors.lastname: Porwit
authors.forename: Alberto
authors.initials: A
authors.lastname: Orfao
authors.forename: Petr
authors.initials: P
authors.lastname: Lemez
authors.forename: Richard
authors.initials: R
authors.lastname: Schabath
authors.forename: Wolf-Dieter
authors.initials: WD
authors.lastname: Ludwig
mesh_terms: D000293:Adolescent; D000328:Adult; D001402:B-Lymphocytes / Q000473:pathology; D001752:Blast Crisis / Q000276:immunology / Q000473:pathology; D002454:Cell Differentiation; D019070:Cell Lineage; D020732:Cytogenetic Analysis; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000145:classification* / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D013601:T-Lymphocytes / Q000473:pathology; D016896:Treatment Outcome; D014944:World Health Organization