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Aetiology of Myeloproliferative Neoplasms
 
 
Article

Clinical, Hematologic, Biologic and Molecular Characteristics of Patients with Myeloproliferative Neoplasms and a Chronic Myelomonocytic Leukemia-Like Phenotype

1
Department of Internal Medicine IV, Hospital Wels-Grieskirchen, 4600 Wels, Austria
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Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, 1090 Vienna, Austria
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Department of Laboratory Medicine, Medical University of Vienna, 1090 Vienna, Austria
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Blood Transfusion Service, Blood Transfusion Service for Upper Austria, Austrian Red Cross, 4020 Linz, Austria
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Department of Internal Medicine V with Hematology, Oncology and Palliative Care, Hospital Hietzing, 1130 Vienna, Austria
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Department of Internal Medicine I with Hematology with Stem Cell Transplantation, Hemostaseology and Medical Oncology, Ordensklinikum Linz Barmherzige Schwestern-Elisabethinen, 4020 Linz, Austria
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Department of Internal Medicine III, Hanusch Hospital, 1140 Vienna, Austria
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Center for Medical Genetics, Hanusch Hospital, 1140 Vienna, Austria
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Medical School, Sigmund Freud University, 1020 Vienna, Austria
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Central Institute of Medical and Chemical Laboratory Diagnostics, Medical University of Innsbruck, 6020 Innsbruck, Austria
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Internal Medicine V with Hematology and Oncology, Medical University of Innsbruck, 6020 Innsbruck, Austria
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School of Medicine, University of Zagreb, University Hospital Dubrava, 10000 Zagreb, Croatia
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Ludwig Boltzmann Institute for Hematology and Oncology (LBI HO), Medical University of Vienna, 1090 Vienna, Austria
*
Author to whom correspondence should be addressed.
Cancers 2020, 12(7), 1891; https://doi.org/10.3390/cancers12071891
Received: 19 June 2020 / Revised: 7 July 2020 / Accepted: 12 July 2020 / Published: 14 July 2020
(This article belongs to the Special Issue New Insights into Myeloproliferative Neoplasms)
Patients with a myeloproliferative neoplasm (MPN) sometimes show a chronic myelomonocytic leukemia (CMML)-like phenotype but, according to the 2016 WHO classification, a documented history of an MPN excludes the diagnosis of CMML. Forty-one patients with an MPN (35 polycythemia vera (PV), 5 primary myelofibrosis, 1 essential thrombocythemia) and a CMML-like phenotype (MPN/CMML) were comprehensively characterized regarding clinical, hematologic, biologic and molecular features. The white blood cell counts in MPN/CMML patients were not different from CMML patients and PV patients. The hemoglobin values and platelet counts of these patients were higher than in CMML but lower than in PV, respectively. MPN/CMML patients showed myelomonocytic skewing, a typical in vitro feature of CMML but not of PV. The mutational landscape of MPN/CMML was not different from JAK2-mutated CMML. In two MPN/CMML patients, development of a CMML-like phenotype was associated with a decrease in the JAK2 V617F allelic burden. Finally, the prognosis of MPN/CMML (median overall survival (OS) 27 months) was more similar to CMML (JAK2-mutated, 28 months; JAK2-nonmutated 29 months) than to PV (186 months). In conclusion, we show that patients with MPN and a CMML-like phenotype share more characteristics with CMML than with PV, which may be relevant for their classification and clinical management. View Full-Text
Keywords: myeloproliferative neoplasm; chronic myelomonocytic leukemia; NGS; progenitor cells; prognosis myeloproliferative neoplasm; chronic myelomonocytic leukemia; NGS; progenitor cells; prognosis
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MDPI and ACS Style

Heibl, S.; Gisslinger, B.; Jäger, E.; Barna, A.; Gurbisz, M.; Stegemann, M.; Bettelheim, P.; Machherndl-Spandl, S.; Pfeilstöcker, M.; Nösslinger, T.; Uyanik, G.; Hoermann, G.; Stauder, R.; Thaler, J.; Kusec, R.; Valent, P.; Gisslinger, H.; Geissler, K. Clinical, Hematologic, Biologic and Molecular Characteristics of Patients with Myeloproliferative Neoplasms and a Chronic Myelomonocytic Leukemia-Like Phenotype. Cancers 2020, 12, 1891. https://doi.org/10.3390/cancers12071891

AMA Style

Heibl S, Gisslinger B, Jäger E, Barna A, Gurbisz M, Stegemann M, Bettelheim P, Machherndl-Spandl S, Pfeilstöcker M, Nösslinger T, Uyanik G, Hoermann G, Stauder R, Thaler J, Kusec R, Valent P, Gisslinger H, Geissler K. Clinical, Hematologic, Biologic and Molecular Characteristics of Patients with Myeloproliferative Neoplasms and a Chronic Myelomonocytic Leukemia-Like Phenotype. Cancers. 2020; 12(7):1891. https://doi.org/10.3390/cancers12071891

Chicago/Turabian Style

Heibl, Sonja, Bettina Gisslinger, Eva Jäger, Agnes Barna, Michael Gurbisz, Maike Stegemann, Peter Bettelheim, Sigrid Machherndl-Spandl, Michael Pfeilstöcker, Thomas Nösslinger, Gökhan Uyanik, Gregor Hoermann, Reinhard Stauder, Josef Thaler, Rajko Kusec, Peter Valent, Heinz Gisslinger, and Klaus Geissler. 2020. "Clinical, Hematologic, Biologic and Molecular Characteristics of Patients with Myeloproliferative Neoplasms and a Chronic Myelomonocytic Leukemia-Like Phenotype" Cancers 12, no. 7: 1891. https://doi.org/10.3390/cancers12071891

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