About Myelofibrosis (MF)
MF is a disorder of the bone marrow, in which the marrow is replaced by scar tissue, with approximately 1 in every 133,000 people estimated to be affected by the disease1,2,3. This condition is part of a group of related blood cancers known as myeloproliferative neoplasms (MPNs)1.
Since the abnormal marrow can no longer produce enough normal blood cells, the spleen often becomes enlarged when it takes responsibility for blood cell creation1. More than 80% of patients with MF have mutations that directly or indirectly activate the JAK/STAT signaling pathway, which may explain the development of the disease4. Patients may also suffer debilitating symptoms, including fatigue/weakness, shortness of breath, fever/night sweats, itchy skin, bone/joint pain, abdominal pain and/or weight loss, and have a poor quality of life and shortened survival1,5,6.
Treatment of MF
- For most people, MF treatment does not cure the disease. Rather, the goal of treatment is to reduce symptoms and improve quality of life1. This means that the treatment patients receive for MF will depend on the symptoms. It is, therefore, important that patients and caregivers understand the symptoms to collaborate with a hematologist to create an optimal treatment plan.
- Remember, even mild symptoms can have a significant impact on day-to-day life and if they do, it’s important to address these with a hematologist.
Questions to ask your doctor
- How can I assess my symptoms?
- How will my MF-related symptoms impact my quality of life?
- What are my treatment choices, specifically in terms of medication therapy, splenectomy and transplant?
- What are the expected benefits of each kind of treatment?
- What are the risks of each treatment?
- What are the side effects of each treatment?
- Is there a benefit to starting treatment prior to the development of debilitating symptoms?
- How will we know if the treatment is working?
- How will each treatment affect my daily life?
- Are there new treatments or clinical trials I should consider?
- Leukemia & Lymphoma Society. “Myelofibrosis Facts.” Available at: http://www.lls.org/sites/default/files/file_assets/FS14_Myelofibrosis_Fact%20Sheet_Final9.12.pdf. Accessed August 2015.
- Girodon F, Bonicelli G, Schaeffer C, et al. Significant increase in the apparent incidence of essential thrombocythemia related to new WHO diagnostic criteria: a population-based study. Haematologica. 2009; 94(6):865-869.
- McNally RJQ, Rowland D, Roman E, Cartwright RA. Age and sex distributions of hematological malignancies in the U.K. Hematol Oncol. 1997;15:173–189.
- Cazzola M, Kralovics R. From Janus Kinase 2 to Calreticulin: The Clinically Relevant Genomic Landscape of Myeloproliferative Neoplasms. Blood. 2014 Jun 12;123(24):3714-9.
- Mesa RA, Schwagera S, Radia D, et al. The Myelofibrosis Symptom Assessment Form (MFSAF): An Evidence-Based Brief Inventory to Measure Quality of Life and Symptomatic Response to Treatment in Myelofibrosis. Leuk Res. 2009; 33:1199-1203.
- Cervantes F, Dupriez B, Pereira A, et al. New Prognostic Scoring System for Primary Myeloﬁbrosis Based on a Study of the International Working Group for Myeloﬁbrosis Research and Treatment. Blood. 2009;113:2895–2901.