A better understanding of the control of the extracellular matrix (ECM) within the bone marrow niche may lead to combined therapeutic options in primary myelofibrosis (PMF), according to a recent review.

While cellular mutations involved in the development of PMF have been heavily investigated, noteworthy is the important role the ECM plays in the progression of bone marrow fibrosis.

Treatment of myelofibrosis (MF), which is found not only in PMF but also secondary to many hematological diseases, is still a challenge, and options are scarce. Specific targeting of ECM dysregulation to prevent and diminish MF may prove the frontline of research and therapy development in PMF with the greatest promise of relieving symptoms and extending life expectancy of patients.