Current treatment algorithm for the management of patients with myelofibrosis, JAK inhibitors, and beyond

Abstract

Myelofibrosis (MF) is a heterogeneous disorder characterized by splenomegaly, constitutional symptoms, ineffective hematopoiesis, and an inherent risk of leukemic transformation. The past decade has seen a massive shift in available therapeutic options for our patients and we are learning how and when to use novel agents, either alone or in combination, during the disease course. This has translated into improved management of splenomegaly, significant amelioration in disease-related symptom burden for many, and may lead to improved survival. But limitations in the therapeutic options remain. Moreover, enhanced delineation of the mutational landscape of MF has offered both increasingly complex prognostic algorithms and yielded further potential therapeutic avenues. In this review, we will focus on stratifying both standard and experimental therapeutic management in 2017 and 2018 and postulate on the emerging treatments that will likely become part of our increasingly complex treatment algorithm.