Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions

Peter Valent, Attilio Orazi, Michael R. Savona, Mrinal M. Patnaik, Francesco Onida, Arjan A. Van De Loosdrecht, Detlef Haase, Torsten Haferlach, Chiara Elena, Lisa Pleyer, Wolfgang Kern, Tea Pemovska, Gregory I. Vladimer, Julie Schanz, Alexandra Keller, Michael Lübbert, Thomas Lion, Karl Sotlar, Andreas Reiter, Theo De WitteMichael Pfeilstöcker, Klaus Geissler, Eric Padron, Michael Deininger, Alberto Orfao, Hans Peter Horny, Peter L. Greenberg, Daniel A. Arber, Luca Malcovati, John M. Bennett

Research output: Contribution to journalReview article

Abstract

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.

Original languageEnglish
Pages (from-to)1935-1949
Number of pages15
JournalHaematologica
Volume104
Issue number10
DOIs
Publication statusPublished - Jan 1 2019

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Leukemia, Myelomonocytic, Chronic
Systemic Mastocytosis
Hematology

ASJC Scopus subject areas

  • Hematology

Cite this

Valent, P., Orazi, A., Savona, M. R., Patnaik, M. M., Onida, F., Van De Loosdrecht, A. A., ... Bennett, J. M. (2019). Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions. Haematologica, 104(10), 1935-1949. https://doi.org/10.3324/haematol.2019.222059

Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions. / Valent, Peter; Orazi, Attilio; Savona, Michael R.; Patnaik, Mrinal M.; Onida, Francesco; Van De Loosdrecht, Arjan A.; Haase, Detlef; Haferlach, Torsten; Elena, Chiara; Pleyer, Lisa; Kern, Wolfgang; Pemovska, Tea; Vladimer, Gregory I.; Schanz, Julie; Keller, Alexandra; Lübbert, Michael; Lion, Thomas; Sotlar, Karl; Reiter, Andreas; De Witte, Theo; Pfeilstöcker, Michael; Geissler, Klaus; Padron, Eric; Deininger, Michael; Orfao, Alberto; Horny, Hans Peter; Greenberg, Peter L.; Arber, Daniel A.; Malcovati, Luca; Bennett, John M.

In: Haematologica, Vol. 104, No. 10, 01.01.2019, p. 1935-1949.

Research output: Contribution to journalReview article

Valent, P, Orazi, A, Savona, MR, Patnaik, MM, Onida, F, Van De Loosdrecht, AA, Haase, D, Haferlach, T, Elena, C, Pleyer, L, Kern, W, Pemovska, T, Vladimer, GI, Schanz, J, Keller, A, Lübbert, M, Lion, T, Sotlar, K, Reiter, A, De Witte, T, Pfeilstöcker, M, Geissler, K, Padron, E, Deininger, M, Orfao, A, Horny, HP, Greenberg, PL, Arber, DA, Malcovati, L & Bennett, JM 2019, 'Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions', Haematologica, vol. 104, no. 10, pp. 1935-1949. https://doi.org/10.3324/haematol.2019.222059
Valent, Peter ; Orazi, Attilio ; Savona, Michael R. ; Patnaik, Mrinal M. ; Onida, Francesco ; Van De Loosdrecht, Arjan A. ; Haase, Detlef ; Haferlach, Torsten ; Elena, Chiara ; Pleyer, Lisa ; Kern, Wolfgang ; Pemovska, Tea ; Vladimer, Gregory I. ; Schanz, Julie ; Keller, Alexandra ; Lübbert, Michael ; Lion, Thomas ; Sotlar, Karl ; Reiter, Andreas ; De Witte, Theo ; Pfeilstöcker, Michael ; Geissler, Klaus ; Padron, Eric ; Deininger, Michael ; Orfao, Alberto ; Horny, Hans Peter ; Greenberg, Peter L. ; Arber, Daniel A. ; Malcovati, Luca ; Bennett, John M. / Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions. In: Haematologica. 2019 ; Vol. 104, No. 10. pp. 1935-1949.
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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.

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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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