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Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions
Ferrata Storti Foundation
Haematologica 2019 Volume 104(10):1935-1949
Peter Valent,1,2 Attilio Orazi,3 Michael R. Savona,4 Mrinal M. Patnaik,5 Francesco Onida,6 Arjan A. van de Loosdrecht,7 Detlef Haase,8 Torsten Haferlach,9 Chiara Elena,10 Lisa Pleyer,11 Wolfgang Kern,9 Tea Pemovska,12 Gregory I. Vladimer,12 Julie Schanz,8 Alexandra Keller,1 Michael Lübbert,13 Thomas Lion,14 Karl Sotlar,15 Andreas Reiter,16 Theo De Witte,17 Michael Pfeilstöcker,2,18 Klaus Geissler,19 Eric Padron,20 Michael Deininger,21 Alberto Orfao,22 Hans-Peter Horny,23 Peter L. Greenberg,24 Daniel A. Arber,25 Luca Malcovati10 and John M. Bennett26
1Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria; 2Ludwig Boltzmann Institute for Hematology & Oncology, Vienna, Austria; 3Department of Pathology, Texas Tech University Health Sciences Center, El Paso, TX, USA; 4Department of Medicine, Vanderbilt University School of Medicine, Vanderbilt-Ingram Cancer Center, Nashville, TN, USA; 5Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA; 6Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy; 7Department of Hematology, Amsterdam UMC, location VU University Medical Center, Cancer Center Amsterdam, the Netherlands; 8Clinic of Hematology and Medical Oncology, University Medical Center Göttingen, Göttingen, Germany; 9MLL Munich Leukemia Laboratory, Munich, Germany; 10Department of Molecular Medicine, University of Pavia, Pavia, Italy; 113rd Medical Department with Hematology and Medical Oncology, Hemostaseology, Rheumatology and Infectious Diseases, Paracelsus Medical University, Salzburg, Austria; 12CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria; 13Department of Medicine I, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany; 14Children's Cancer Research Institute and Department of Pediatrics, Medical University of Vienna, Vienna, Austria; 15Institute of Pathology, Paracelsus Medical University, Salzburg, Austria; 16Department of Hematology and Oncology, University Hospital Mannheim, University of Heidelberg, Mannheim, Germany; 17Department of Tumor Immunology- Nijmegen Center for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, the Netherlands; 183rd Medical Department, Hanusch Hospital, Vienna, Vienna, Austria; 19Sigmund Freud University, Vienna, Austria; 20Malignant Hematology Department, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL, USA; 21Huntsman Cancer Institute & Division of Hematology and Hematologic Malignancies, University of Utah, Salt Lake City, UT, USA; 22Servicio Central de Citometría, Centro de Investigación del Cáncer (IBMCC, CSIC-USAL), CIBERONC and IBSAL, Universidad de Salamanca, Salamanca, Spain; 23Institute of Pathology, Ludwig-Maximilians University, Munich, Germany; 24Stanford University Cancer Institute, Stanford, CA, USA; 25Department of Pathology, University of Chicago, Chicago, IL, USA and 26Department of Pathology, Hematopathology Unit and James P Wilmot Cancer Institute, University of Rochester Medical Center, Rochester, NY, USA
ABSTRACT
Chronic myelomonocytic leukemia (CMML) is a myeloid neoplasm characterized by dysplasia, abnormal production and accumula- tion of monocytic cells and an elevated risk of transforming into acute leukemia. Over the past two decades, our knowledge about the pathogenesis and molecular mechanisms in CMML has increased sub- stantially. In parallel, better diagnostic criteria and therapeutic strategies have been developed. However, many questions remain regarding prog- nostication and optimal therapy. In addition, there is a need to define potential pre-phases of CMML and special CMML variants, and to sepa- rate 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 ques- tions and issues around CMML, its variants, and pre-CMML conditions. The outcomes of this meeting are summarized herein and include diag-
Correspondence:
PETER VALENT
peter.valent@meduniwien.ac.at
Received: March 14, 2019. Accepted: April 29, 2019. Pre-published: May 2, 2019.
doi:10.3324/haematol.2019.222059
Check the online version for the most updated information on this article, online supplements, and information on authorship & disclosures: www.haematologica.org/content/104/10/1935
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