Affiliation

Fifth Medical Department with Hematology, Oncology and Palliative Medicine, Hospital Hietzing, Vienna, Austria

Corresponding Author

Prof. Dr. Klaus Geissler, MD, Fifth Medical Department with Hematology, Oncology and Palliative Medicine, Hospital Hietzing, Wolkersbergestraße 1, A-1130 Vienna, Austria, E-mail: klaus.geissler@wienkav.at

Citation

Geissler, K., et al. The Role of the RAS Signaling Pathway in the Pathophysiology of Chronic Myelomonocytic Leukemia - New Insights from Molecular and Biologic Characterization. (2017) Int J Hematol Ther 3(1): 1- 10.

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© 2017 Geissler, K. This is an Open access article distributed under the terms of Creative Commons Attribution 4.0 International License.

Keywords

Abstract

  Recent insights into the pathophysiology of chronic myelomonocytic leukemia (CMML) have been obtained by the molecular and biologic characterization of primary leukemic cells from patients and from animal models. Almost 3 decades ago extensive myeloid colony growth in semisolid cultures without exogenous growth factors was observed as an in vitro characteristic of a subgroup of CMML patients. Recent data suggest that this phenomenon was probably the first indication of a hyperactive RAS signaling pathway in these patients. Although the mutation landscape in CMML is heterogenous and molecular aberrations in other signaling components can be found in some patients, the RAS pathway seems to play the major pathophysiological role in the majority of CMML patients with myeloproliferation (MP), disease progression and transformation into secondary acute myeloid leukemia (AML). There is also increasing evidence indicating that MP-CMML as a RAS pathway driven disease evolves from age related clonal hematopoiesis.