TY - JOUR
T1 - Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions
AU - Valent, Peter
AU - Orazi, Attilio
AU - Savona, Michael R.
AU - Patnaik, Mrinal M.
AU - Onida, Francesco
AU - Van De Loosdrecht, Arjan A.
AU - Haase, Detlef
AU - Haferlach, Torsten
AU - Elena, Chiara
AU - Pleyer, Lisa
AU - Kern, Wolfgang
AU - Pemovska, Tea
AU - Vladimer, Gregory I.
AU - Schanz, Julie
AU - Keller, Alexandra
AU - Lübbert, Michael
AU - Lion, Thomas
AU - Sotlar, Karl
AU - Reiter, Andreas
AU - De Witte, Theo
AU - Pfeilstöcker, Michael
AU - Geissler, Klaus
AU - Padron, Eric
AU - Deininger, Michael
AU - Orfao, Alberto
AU - Horny, Hans Peter
AU - Greenberg, Peter L.
AU - Arber, Daniel A.
AU - Malcovati, Luca
AU - Bennett, John M.
N1 - Funding Information:
We thank Sabine Sonnleitner, Sophia Rammler, Susanne Gamperl, Emir Hadzijusofovic and all other members of Peter Valent’s research group involved for their excellent support in the organization of the Working Conference. This study was supported by Austrian Science Fund (FWF) grants F4701-B20 and F4704-B20, the Ludwig Boltzmann Institute for Hematology and Oncology (LBI HO) and a Stem Cell Research grant from the Medical University of Vienna.
Publisher Copyright:
© 2019 Ferrata Storti Foundation. All rights reserved.
PY - 2019
Y1 - 2019
N2 - Chronic myelomonocytic leukemia (CMML) is a myeloid neoplasm characterized by dysplasia, abnormal production and accumulation 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 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 distinguish 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.
AB - Chronic myelomonocytic leukemia (CMML) is a myeloid neoplasm characterized by dysplasia, abnormal production and accumulation 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 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 distinguish 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.
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U2 - 10.3324/haematol.2019.222059
DO - 10.3324/haematol.2019.222059
M3 - Review article
C2 - 31048353
AN - SCOPUS:85072528726
SN - 0390-6078
VL - 104
SP - 1935
EP - 1949
JO - Haematologica
JF - Haematologica
IS - 10
ER -