Mixed-phenotype acute leukemia: clinical and laboratory features and outcome in 100 patients defined according to the WHO 2008 classification
| Autor: | Alberto Orfao, Winfried F. Pickl, Ricardo Morilla, John Swansbury, Marie C. Béné, Herbert Strobl, Sue Ashley, Estella Matutes, Georg Hopfinger, Petr Lemez, Mars B. van 't Veer, Richard Schabath, Andishe Attarbaschi, Wolf-Dieter Ludwig, Anna Porwit |
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| Rok vydání: | 2011 |
| Předmět: |
Adult
Male medicine.medical_specialty Pathology Myeloid Adolescent Combination therapy T-Lymphocytes Immunology World Health Organization Biochemistry Gastroenterology Immunophenotyping hemic and lymphatic diseases Internal medicine medicine Humans Cell Lineage B-Lymphocytes Acute leukemia Hematology business.industry Infant Myeloid leukemia Cell Differentiation Imatinib Cell Biology Precursor Cell Lymphoblastic Leukemia-Lymphoma Flow Cytometry Transplantation Treatment Outcome medicine.anatomical_structure Cytogenetic Analysis Female Blast Crisis business medicine.drug |
| Zdroj: | Digital.CSIC. Repositorio Institucional del CSIC instname |
| ISSN: | 1528-0020 0006-4971 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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