Medicina Molecular e Genética

The Emerging Role of the Hematopoietic Stem Cell Niche in Myeloproliferative Neoplasms

Abstract

Biquan Luo

Myeloproliferative neoplasms (MPN) are chronic hematological neoplasms arising from the accumulation of genetic mutations in hematopoietic stem cells (HSCs). This notion is supported by genomic studies on MPN patients, in which recurrent mutations on genes such as JAK2, CALR, and MPL have been identified. Interestingly, recent studies using MPN mouse models have demonstrated that the HSC niche, where HSCs are located and maintained in the bone marrow (BM), plays an important role in MPN initiation and progression. Changes in the BM microenvironment alone can serve as a driver for altered hematopoiesis and hematological malignancies. Additionally, during MPN progression mutant HSCs remodel the HSC niche into a hospitable microenvironment for themselves that accelerates disease progression, creating a positive feedback loop where mutant HSCs thrive, and continue to alter the BM microenvironment to their advantage. In an attempt to provide a fresh perspective in understanding MPN pathogenesis, this review highlights the studies conducted that explore the role of the HSC niche in MPN pathogenesis and progression.