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    Haematologica. 2019 May 2. pii: haematol.2019.222059. doi: 10.3324/haematol.2019.222059
    Proposed diagnostic criteria for classical CMML, CMML variants and pre-CMML conditions.
    Valent P1,  Orazi A2,  Savona MR3,  Patnaik MM4,  Onida F5,  van de Loosdrecht AA6,  Haase D7,  Haferlach T8,  Elena C9,  Pleyer L10,  Kern W11,  Pemovska T12,  Vladimer GI13,  Schanz J14,  Keller A15,  Lubbert M16,  Lion T17,  Sotlar K18,  Reiter A19,  De Witte T20,  Pfeilstocker M21,  Geissler K22,  Padron E23,  Deininger M24,  Orfao A25,  Horny HP26,  Greenberg PL27,  Arber DA28,  Malcovati L29,  Bennett JM30
    Author information
    1Dept Internal Med I and Ludwig Boltzmann Inst Hematol & Oncol, Medical University of Vienna, Austria; peter.valent@meduniwien.ac.at.
    2Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY, USA.
    3Department of Medicine, Vanderbilt University School of Medicine, Vanderbilt-Ingram Cancer Center.
    4Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
    5Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
    6Department of Hematology, VU University Medical Center, Cancer Center Amsterdam, The Netherlands.
    7Clinic of Hematology and Medical Oncology, University Medical Center Gottingen, Germany.
    8MLL Munich Leukemia Laboratory, Munich, Germany.
    9Department of Molecular Medicine, University of Pavia, Italy.
    103rd Medical Department - Paracelsus Medical University, Salzburg, Austria.
    11MLL Munich Leukemia Laboratory, Munich, Germany.
    12CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
    13CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
    14Clinic of Hematology and Medical Oncology, University Medical Center Gottingen, Germany.
    15Dept Internal Med I and Ludwig Boltzmann Inst Hematol and Oncol, Med University of Vienna, Austria.
    16Department of Medicine I, Medical Center, Faculty of Medicine, University of Freiburg, Germany.
    17Children's Cancer Research Institute and Department of Pediatrics, Medical Univ of Vienna, Austria.
    18Institute of Pathology, Paracelsus Medical University, Salzburg, Austria.
    19Department of Hematol and Oncol, Univ Hospital Mannheim, Univ of Heidelberg, Mannheim, Germany.
    20Dept of Tumor Immunol-Nijme Center Mol Life Sci, Radboud Univ Med Center, Nijmegen, The Netherlands.
    213rd Medical Department, Hanusch Hospital and Ludwig Boltzmann Inst Hematol and Oncol, Vienna,Austria.
    225th Medical Department for Hematology and Oncology, Hospital Hietzing, Vienna, Austria.
    23Malignant Hematology Department, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.
    24Huntsman Cancer Inst and Div of Hematol and Hematol Malignancies, Univ of Utah, Salt Lake City, USA.
    25Servicio Central de Citometria, Centro de Investigacion del Cancer, Universidad de Salamanca, Spain.
    26Institute of Pathology, Ludwig-Maximilians University, Munich, Germany.
    27Stanford University Cancer Institute, Stanford, CA, USA.
    28Department of Pathology, University of Chicago, Chicago, IL, USA.
    29Department of Molecular Medicine, University of Pavia, Italy.
    30Dept of Pathology, Hematopathology, Univ of Rochester Medical Center, Rochester, New York, USA.
    Abstract

    Chronic myelomonocytic leukemia (CMML) is a myeloid neoplasm characterized by dysplasia, abnormal production and accumulation of monocytic cells and an elevated risk to transform into acute leukemia. Over the past two decades, our knowledge about the pathogenesis and molecular mechanisms in CMML has increased substantially. In parallel, better diagnostic criteria and therapeutic strategies have been developed. However, many questions remain regarding prognostication and optimal therapy. In addition, there is a need to define potential pre-phases of CMML and special CMML variants, and to separate these entities from each other and from conditions mimicking CMML. To address these unmet needs, an international consensus group met in a Working Conference in August 2018 and discussed open questions and issues around CMML, its variants, and pre-CMML conditions. The outcomes of this meeting are summarized herein and include diagnostic criteria and a proposed classification of pre-CMML conditions as well as refined minimal diagnostic criteria for classical CMML and special CMML variants, including oligomonocytic CMML and CMML associated with systemic mastocytosis. Moreover, we propose diagnostic standards and tools to delineate between normal, pre-CMML and CMML entities. These criteria and standards should facilitate diagnostic and prognostic evaluations in daily practice and clinical studies in applied hematology.


    Copyright © 2019, Ferrata Storti Foundation.

    KEYWORDS: Acute Myeloid Leukemia, Adult Acute Lymphoblastic Leukemia, Hodgkin's Lymphoma

    Publikations ID: 31048353
    Quelle: öffnen
     
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