Janus Kinase Inhibitors and Stem Cell Transplantation in Myelofibrosis

Clin Lymphoma Myeloma Leuk. 2015 Jun:15 Suppl:S34-42. doi: 10.1016/j.clml.2015.02.034.

Abstract

Myelofibrosis (MF) is characterized by splenomegaly, blood count abnormalities, particularly cytopenias, and a propensity for transformation to acute leukemia. The current treatment approach is to ameliorate symptoms due to these abnormalities. Treatment with Janus kinase 2 inhibitors reduces spleen size and improves symptoms in patients with MF, but most of the patients eventually have disease progression and stop responding. Allogeneic stem cell transplantation remains the only curative option. However, its efficacy must be balanced against the risk of treatment-related death and long-term sequelae of transplant like chronic graft versus host disease. The challenge is to integrate treatment with Janus kinase inhibitors with allogeneic stem cell transplantation.

Keywords: Myelofibrosis; Myeloproliferative neoplasm; Stem cell transplant.

Publication types

  • Review

MeSH terms

  • Hematopoietic Stem Cell Transplantation / methods*
  • Humans
  • Janus Kinase 2 / genetics*
  • Janus Kinase 2 / metabolism*
  • Primary Myelofibrosis / drug therapy*
  • Primary Myelofibrosis / pathology
  • Prognosis
  • Protein Kinase Inhibitors / therapeutic use*
  • Transplantation Conditioning / methods*
  • Transplantation, Homologous / methods*

Substances

  • Protein Kinase Inhibitors
  • JAK2 protein, human
  • Janus Kinase 2