Mesenchymal stem cells in myeloproliferative disorders – focus on primary myelofibrosis

Abstract

Primary myelofibrosis (PMF) is the most aggressive Philadelphia-negative (Ph-) myeloproliferative neoplasm (MPN), characterized by bone marrow (BM) insufficiency, myelofibrosis (MF), osteosclerosis, neoangiogenesis, and extramedullary hematopoiesis (EMH) in spleen and liver. Presently, there is no curative treatment for this disease and therapy consists primarily of symptom relief and, in selected cases, allogeneic hematopoietic stem cell transplant (alloHSCT). PMF’s major defining characteristics, as well as several recently described aspects of its cellular and molecular pathophysiology all support a critical role for dysregulated cell-cell/cell-extracellular matrix interactions and cytokine/chemokine signaling within the BM niche in the natural history of this disease. This review will highlight current data concerning the involvement of the BM niche, particularly of mesenchymal stem cells (MSC), in PMF, and will then discuss the rationale for a stroma-directed treatment, and the advantages such an approach would offer over the current treatments focused on targeting the malignant clone.

Keywords:

• Myeloproliferative neoplasms
• bone marrow microenvironment
• cellular crosstalk

Potential conflict of interest

Disclosure forms provided by the authors are available with the full text of this article online at https://doi.org/10.1080/10428194.2018.1516881.

Additional information

Funding

This work was supported by an internal grant awarded by the Iuliu Haţieganu University of Medicine and Pharmacy, under Grant number 4995/12/08.03.2016, awarded to Sonia Emilia Selicean.