Eric Padron1, David P Steensma. 1. aH. Lee Moffitt Cancer Center, Tampa, Florida bDana-Farber Cancer Institute, Boston, Massachusetts, USA.
Abstract
PURPOSE OF REVIEW: Chronic myelomonocytic leukemia (CMML) is a troublesome hematologic malignancy characterized by peripheral blood monocytosis, marrow dysplasia, cytopenias, frequent extramedullary involvement by clonal cells, and a propensity for progression to acute myeloid leukemia. Although previously considered a subtype of the myelodysplastic syndromes (MDS), CMML is now recognized as a distinct entity with unique biologic and clinical features. This change has created a scientific and clinical research landscape that makes it difficult to discern CMML-specific validated conclusions versus speculative extrapolation from more general MDS data. RECENT FINDINGS: Here, we review recent biologic observations that support the current CMML WHO classification, such as the high frequency of SRSF2 and ASXL1 mutations compared with MDS and critical dependence of CMML cells on granulocyte-macrophage colony-stimulating factor signaling. In addition, we discuss CMML-specific prognostic tools and therapeutic results of agents developed for MDS in patients with CMML. SUMMARY: The present review focuses on evidence supporting CMML ontology and identifies key clinical differences in the management of CMML and that of MDS subtypes.
PURPOSE OF REVIEW: Chronic myelomonocytic leukemia (CMML) is a troublesome hematologic malignancy characterized by peripheral blood monocytosis, marrow dysplasia, cytopenias, frequent extramedullary involvement by clonal cells, and a propensity for progression to acute myeloid leukemia. Although previously considered a subtype of the myelodysplastic syndromes (MDS), CMML is now recognized as a distinct entity with unique biologic and clinical features. This change has created a scientific and clinical research landscape that makes it difficult to discern CMML-specific validated conclusions versus speculative extrapolation from more general MDS data. RECENT FINDINGS: Here, we review recent biologic observations that support the current CMML WHO classification, such as the high frequency of SRSF2 and ASXL1 mutations compared with MDS and critical dependence of CMML cells on granulocyte-macrophage colony-stimulating factor signaling. In addition, we discuss CMML-specific prognostic tools and therapeutic results of agents developed for MDS in patients with CMML. SUMMARY: The present review focuses on evidence supporting CMML ontology and identifies key clinical differences in the management of CMML and that of MDS subtypes.
Authors: Eric Padron; Amy Dezern; Marcio Andrade-Campos; Kris Vaddi; Peggy Scherle; Qing Zhang; Yan Ma; Maria E Balasis; Sara Tinsley; Hanadi Ramadan; Cassandra Zimmerman; David P Steensma; Gail J Roboz; Jeffrey E Lancet; Alan F List; Mikkael A Sekeres; Rami S Komrokji Journal: Clin Cancer Res Date: 2016-02-08 Impact factor: 12.531
Authors: Akihide Yoshimi; Maria E Balasis; Alexis Vedder; Kira Feldman; Yan Ma; Hailing Zhang; Stanley Chun-Wei Lee; Christopher Letson; Sandrine Niyongere; Sydney X Lu; Markus Ball; Justin Taylor; Qing Zhang; Yulong Zhao; Salma Youssef; Young Rock Chung; Xiao Jing Zhang; Benjamin H Durham; Wendy Yang; Alan F List; Mignon L Loh; Virginia Klimek; Michael F Berger; Elliot Stieglitz; Eric Padron; Omar Abdel-Wahab Journal: Blood Date: 2017-06-02 Impact factor: 22.113
Authors: Jane Merlevede; Nathalie Droin; Tingting Qin; Kristen Meldi; Kenichi Yoshida; Margot Morabito; Emilie Chautard; Didier Auboeuf; Pierre Fenaux; Thorsten Braun; Raphael Itzykson; Stéphane de Botton; Bruno Quesnel; Thérèse Commes; Eric Jourdan; William Vainchenker; Olivier Bernard; Noemie Pata-Merci; Stéphanie Solier; Velimir Gayevskiy; Marcel E Dinger; Mark J Cowley; Dorothée Selimoglu-Buet; Vincent Meyer; François Artiguenave; Jean-François Deleuze; Claude Preudhomme; Michael R Stratton; Ludmil B Alexandrov; Eric Padron; Seishi Ogawa; Serge Koscielny; Maria Figueroa; Eric Solary Journal: Nat Commun Date: 2016-02-24 Impact factor: 14.919
Authors: Klaus Geissler; Eva Jäger; Agnes Barna; Michael Gurbisz; Temeida Graf; Elmir Graf; Thomas Nösslinger; Michael Pfeilstöcker; Heinz Tüchler; Thamer Sliwa; Felix Keil; Christoph Geissler; Sonja Heibl; Josef Thaler; Sigrid Machherndl-Spandl; Otto Zach; Ansgar Weltermann; Peter Bettelheim; Reinhard Stauder; Armin Zebisch; Heinz Sill; Ilse Schwarzinger; Bruno Schneeweiss; Leopold Öhler; Ernst Ulsperger; Rajko Kusec; Ulrich Germing; Wolfgang R Sperr; Paul Knöbl; Ulrich Jäger; Gregor Hörmann; Peter Valent Journal: Int J Mol Sci Date: 2020-04-24 Impact factor: 5.923
Authors: 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 Witte; Michael Pfeilstöcker; Klaus Geissler; Eric Padron; Michael Deininger; Alberto Orfao; Hans-Peter Horny; Peter L Greenberg; Daniel A Arber; Luca Malcovati; John M Bennett Journal: Haematologica Date: 2019-05-02 Impact factor: 9.941
Authors: Sigrid Machherndl-Spandl; Eva Jäger; Agnes Barna; Michael Gurbisz; Renate Marschon; Temeida Graf; Elmir Graf; Christoph Geissler; Gregor Hoermann; Thomas Nösslinger; Michael Pfeilstöcker; Peter Bettelheim; Otto Zach; Ansgar Weltermann; Sonja Heibl; Josef Thaler; Armin Zebisch; Heinz Sill; Reinhard Stauder; Gerald Webersinke; Rajko Kusec; Ernst Ulsperger; Bruno Schneeweiss; Leopold Öhler; Ulrich Germing; Peter Valent; Heinz Tüchler; Klaus Geissler Journal: Eur J Haematol Date: 2021-06-01 Impact factor: 2.997