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Proposed minimal diagnostic criteria for myelodysplastic syndromes (MDS) and potential pre-MDS conditions

Peter Valent _, Attilio Orazi, David P. Steensma, Benjamin L. Ebert, Detlef Haase, Luca Malcovati, Arjan A. van de Loosdrecht, Torsten Haferlach, Theresia M. Westers, Denise A. Wells, Aristoteles Giagounidis, Michael Loken, Alberto Orfao, Michael Lübbert, Arnold Ganser, Wolf-Karsten Hofmann, Kiyoyuki Ogata, Julie Schanz, Marie C. Béné, Gregor Hoermann, Wolfgang R. Sperr, Karl Sotlar, Peter Bettelheim, Reinhard Stauder, Michael Pfeilstöcker, Hans-Peter Horny, Ulrich Germing, Peter Greenberg and John M. Bennett

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Oncotarget. 2017; 8:73483-73500. https://doi.org/10.18632/oncotarget.19008

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Abstract

Peter Valent1,2, Attilio Orazi3, David P. Steensma4, Benjamin L. Ebert5, Detlef Haase6, Luca Malcovati7, Arjan A. van de Loosdrecht8, Torsten Haferlach9, Theresia M. Westers8, Denise A. Wells10, Aristoteles Giagounidis11, Michael Loken10, Alberto Orfao12, Michael Lübbert13, Arnold Ganser14, Wolf-Karsten Hofmann15, Kiyoyuki Ogata16, Julie Schanz6, Marie C. Béné17, Gregor Hoermann18, Wolfgang R. Sperr1,2, Karl Sotlar19, Peter Bettelheim20, Reinhard Stauder21, Michael Pfeilstöcker22, Hans-Peter Horny23, Ulrich Germing24, Peter Greenberg25 and John M. Bennett26

1 Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria

2 Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria

3 Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY, USA

4 Division of Hematological Malignancies, Department of Medical Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA

5 Division of Hematology, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

6 Clinic of Hematology and Medical Oncology, Universitymedicine Göttingen, Göttingen, Germany

7 Department of Molecular Medicine, University of Pavia, Pavia, Italy

8 Department of Hematology Cancer Center Amsterdam, VU University Medical Center, Amsterdam, The Netherlands

9 Munich Leukemia Laboratory (MLL), Munich, Germany

10 Hematologics, Inc., Seattle, WA, USA

11 Department of Internal Medicine II, Marien Hospital, Düsseldorf, Germany

12 Servicio Central de Citometría, Centro de Investigación del Cáncer (IBMCC, CSIC-USAL) and IBSAL, Universidad de Salamanca, Salamanca, Spain

13 Department of Medicine I, Medical Center-University of Freiburg, Freiburg, Germany

14 Department of Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany

15 Department of Hematology and Oncology, University Hospital Mannheim, Medical Faculty Mannheim of the University of Heidelberg, Mannheim, Germany

16 Metropolitan Research and Treatment Center for Blood Disorders (MRTC Japan), Tokyo, Japan

17 Laboratoire d’Hématologie CHU de Nantes, Nantes, France

18 Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria

19 Institute of Pathology, Paracelsus Medical University Salzburg, Salzburg, Austria

20 Elisabethinen Hospital, Linz, Austria

21 Department of Internal Medicine V (Haematology and Oncology) Innsbruck Medical University, Innsbruck, Austria

22 3rd Medical Department, Hanusch Hospital, Vienna, Austria

23 Institute of Pathology, Ludwig-Maximilians University, Munich, Germany

24 Department of Hematology, Oncology, and Clinical Immunology, Heinrich-Heine-University, Düsseldorf, Germany

25 Stanford University Cancer Institute, Stanford, CA, USA

26 Department of Pathology, Hematopathology Unit and James P Wilmot Cancer Institute, University of Rochester Medical Center, Rochester, New York, USA

Correspondence to:

Peter Valent, email:

Keywords: myelodysplasia, diagnostic criteria, standardization, pre-MDS conditions

Received: May 03, 2017 Accepted: June 26, 2017 Published: July 05, 2017

Abstract

Myelodysplastic syndromes (MDS) comprise a heterogeneous group of myeloid neoplasms characterized by peripheral cytopenia, dysplasia, and a variable clinical course with about 30% risk to transform to secondary acute myeloid leukemia (AML). In the past 15 years, diagnostic evaluations, prognostication, and treatment of MDS have improved substantially. However, with the discovery of molecular markers and advent of novel targeted therapies, new challenges have emerged in the complex field of MDS. For example, MDS-related molecular lesions may be detectable in healthy individuals and increase in prevalence with age. Other patients exhibit persistent cytopenia of unknown etiology without dysplasia. Although these conditions are potential pre-phases of MDS they may also transform into other bone marrow neoplasms. Recently identified molecular, cytogenetic, and flow-based parameters may add in the delineation and prognostication of these conditions. However, no generally accepted integrated classification and no related criteria are as yet available. In an attempt to address this challenge, an international consensus group discussed these issues in a working conference in July 2016. The outcomes of this conference are summarized in the present article which includes criteria and a proposal for the classification of pre-MDS conditions as well as updated minimal diagnostic criteria of MDS. Moreover, we propose diagnostic standards to delineate between ´normal´, pre-MDS, and MDS. These standards and criteria should facilitate diagnostic and prognostic evaluations in clinical studies as well as in clinical practice.


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