Acute leukemias of ambiguous lineage in adults: molecular and clinical characterization |
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Authors: | Sandra Heesch Martin Neumann Stefan Schwartz Isabelle Bartram Cornelia Schlee Thomas Burmeister Matthias Hänel Arnold Ganser Michael Heuser Clemens-Martin Wendtner Wolfgang E Berdel Nicola Gökbuget Dieter Hoelzer Wolf-Karsten Hofmann Eckhard Thiel Claudia D Baldus |
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Institution: | 1. Department of Hematology, Oncology and Tumor Immunology, Charité University Hospital Berlin, Hindenburgdamm 30, 12203, Campus Benjamin Franklin, Berlin, Germany 2. Department of Internal Medicine III, Klinikum Chemnitz, Chemnitz, Germany 3. Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Medical School, Hannover, Germany 4. Department of Hematology, Oncology, Immunology, Palliative Care, Infectious Diseases and Tropical Medicine, Klinikum Schwabing, Munich, Germany 5. Department of Medicine A, Hematology and Oncology, University of Münster, Münster, Germany 6. Department of Hematology and Oncology, Johann Wolfgang Goethe University, Frankfurt/Main, Germany 7. Department of Hematology and Oncology, University Mannheim, Mannheim, Germany
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Abstract: | Acute leukemias of ambiguous lineage represent a heterogeneous group of rare, poorly characterized leukemias with adverse outcome. No larger studies have yet performed a combined approach of molecular and clinical characterization of acute undifferentiated leukemia (AUL) and biphenotypic acute leukemia (BAL) in adults. Here we describe 16 adults with AUL and 26 with BAL and performed mutational as well as expression studies of genes with prognostic impact in acute leukemia (BAALC, ERG, MN1, WT1, and IGFBP7). AUL showed overexpression of these genes compared to T-lymphoblastic leukemia (T-ALL), B-precursor ALL, and to acute myeloid leukemia (AML). Genotype alterations were not detectable in AUL. BAL samples were characterized by frequent WT1 mutations (18 %) and BCR-ABL translocations (30 %). ALL-based treatment protocols induced complete remissions in 40 % and AML-like therapies in 22 % of AUL/BAL patients. The outcome in both groups was very poor; a long-term survival was only observed in patients undergoing allogeneic stem cell transplantation (SCT). Our findings indicate that AUL and BAL share important molecular and high-risk features of both myeloid and lymphoid leukemias. BAL patients exhibited genetic alterations, which can be targeted therapeutically. Importantly, ALL therapy might be more effective than AML protocols and AUL/BAL patients should be considered for allogeneic SCT. |
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