Treatment options for patients with primary myelofibrosis

Abstract

Background Primary myelofibrosis (PMF) is a myeloproliferative disorder resulting in progressive bone marrow fibrosis. In some PMF patients, a mutation in the gene coding for janus-associated kinase 2 (JAK2) results in cell proliferation and evasion of apoptosis. PMF typically occurs in older patients, and is associated with poor prognosis and low survival, with death occurring secondary to cardiovascular disease.(1) Investigations include full blood counts, blood film analysis, and bone marrow aspiration and trephine. Treatment is largely palliative; the single curative intervention of stem-cell transplant being available only to low-risk younger patients. Potential supportive treatments include the use of JAK2 inhibitors.

Method This case report studies a patient who was diagnosed with PMF at the age of 60.

Results The patient’s condition remained stable for up to 11 years before her symptoms deteriorated and her treatment was altered. The report focuses on current and upcoming treatments including JAK inhibitors, and evaluates their possible future implications for the management of patients with PMF.(2,3)

Conclusion Breakthroughs in the understanding of the underlying PMF molecular mechanisms have led to improved treatments. There is a future role for JAK2 inhibitors in the management of palliative care. Ruxolitinib, which is now available for use in clinical practice,