Myelofibrosis (MF)

Myelofibrosis (MF) is a rare, chronic blood cancer characterized by progressive scarring or fibrosis of the bone marrow, which affects the production of healthy blood cells.¹ 

Over the last few decades, researchers have discovered that many patients with MF present with acquired mutations in specific genes (JAK2, CALR, and MPL) that are considered drivers of MF development.²

Diet and Nutrition 

Eating a healthy, balanced diet may improve a patient’s quality of life and prevent exacerbation of MF symptoms related to systemic inflammation. This means enjoying a diet rich in fruits, vegetables, whole grains, fish, oils, and nuts. It also means minimizing or avoiding foods and beverages that increase inflammation, including alcohol, sugary food and drinks, heavily refined or processed foods, and fried foods.³ 

For patients with MF who have significant enlargement of the spleen, eating smaller, more frequent meals throughout the day may prevent feelings of early fullness during meals, malnutrition due to decreased appetite or inability to eat enough because of early satiety, and abdominal discomfort.³ 

Read more about MF diet and nutrition 

Physical Activity

Patients with MF may question whether it is safe to exercise, given the disease-related complications of high or low platelet levels, which increase the risk for developing blood clots and bleeding, respectively.⁴ Additionally, enlargement of the spleen and symptoms related to anemia (shortness of breath and fatigue) may decrease exercise tolerance and capacity, quality of life, social interactions, and the ability to work in patients with MF.^5,6 

It may be advisable for patients to pace their activities with frequent rest breaks and plan for exercise during peak levels of energy. Modifications to activities and exercise may be required to manage fatigue.^4,7

Pre- and Post-Stem Cell Transplantation

Studies on exercise and stem cell transplantation (SCT) have shown that beginning an exercise regimen before transplantation can help reduce the risk of post-transplant atrophy and the long-lasting fatigue commonly associated with SCT. When started before transplantation, exercise was shown to have a positive effect on lower and upper body strength, quality of life, and fatigue levels. However, all exercise should be planned and carried out under the supervision of the guiding physician and physical therapist. If patients have time to learn and incorporate these routines before SCT, the interventions may be more successful.⁸

Read more about MF surgical management

Rest

Adhering to regular bedtimes, taking naps when needed, and avoiding caffeine are all recommended to get sufficient, quality sleep and to manage energy levels, especially when dealing with anemic fatigue as a symptom of MF.⁷

Read more about MF signs and symptoms

Infection Prevention

Certain disease-related complications and treatments increase the risk of infections in patients with MF, including enlargement of the spleen, decreased number of white blood cells (neutropenia),⁹ allogeneic SCT based on the degree of immunosuppression and exposures,¹⁰ splenectomy,⁹ and other treatments that reduce blood cell counts, such as hydroxyurea, thalidomide, prolonged use of corticosteroids, and Jakafi^® (ruxolitinib).¹¹ Bacterial infections, especially those of the respiratory and urogenital tracts, are the most common type of infection.¹¹ 

Patients should report any signs or symptoms of infection to their healthcare provider immediately, such as fevers over 100.4 °F, sore throat or cold, shortness of breath, cough, and burning or pain with urination.¹² 

Tips to prevent infection include¹²:

• Washing hands frequently and asking others around you to wash their hands
• Showering or bathing daily and performing routine oral hygiene
• Avoiding cutting ingrown nails or cuticles and wearing fake nails
• Avoiding large crowds and people who are sick
• Avoiding handling pet waste
• Wearing protective clothing, such as gloves and long pants, when gardening or doing yard work
• Cleaning all scratches and cuts immediately
• Staying current on all vaccinations
• Informing the oncology team prior to any dental appointments or medical procedures, as they may recommend prophylactic antibiotics

Read more about MF complications

Mental Health and Emotional Support

Recommendations to reduce stress and maintain a positive outlook include deep-breathing and relaxation techniques, walking, meditation, prayer, taking long baths, and reading a good book. These techniques may decrease the systemic inflammation that may aggravate MF symptoms and cause disease progression.¹³

Patients may also find relief from the physical support of family and friends, such as asking for help with household chores and accepting rides to and from medical appointments. This support can decrease the stress and physical burdens that may aggravate symptoms. Joining MF support groups to connect with individuals experiencing similar situations may help patients cope with the diagnosis of MF and learn more about the condition and beneficial treatments.¹³

Read more about MF diagnosis

References

1. Primary myelofibrosis. MedlinePlus. Updated September 1, 2014. Accessed December 28, 2022.
2. Vainchenker W, Kralovics R. Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms. Blood. 2017;129(6):667-679. doi:10.1182/blood-2016-10-695940
3. Scherber R, Mesa R, Eckert R. Nutrition recommendations for MPN patients. MPN Research Foundation. Accessed December 28, 2022.
4. Advice for MPN patients: exercising safely. Patient Power. March 8, 2018. Updated September 11, 2019. Accessed December 28, 2022.
5. Song MK, Park BB, Uhm JE. Understanding splenomegaly in myelofibrosis: association with molecular pathogenesis. Int J Mol Sci. 2018;19(3):898. doi:10.3390/ijms19030898
6. Tefferi A, Hudgens S, Mesa R, et al. Use of the functional assessment of cancer therapy−anemia in persons with myeloproliferative neoplasm-associated myelofibrosis and anemia. Clin Ther. 2014;36(4):560-566. doi:10.1016/j.clinthera.2014.02.016
7. Three ways to manage your energy levels when you have myelofibrosis. Voices of MPN. Accessed December 28, 2022.
8. Liang Y, Zhou M, Wang F, Wu Z. Exercise for physical fitness, fatigue and quality of life of patients undergoing hematopoietic stem cell transplantation: a meta-analysis of randomized controlled trials. Jpn J Clin Oncol. 2018;48(12):1046-1057. doi:10.1093/jjco/hyy144
9. Myelofibrosis facts. Leukemia & Lymphoma Society. Updated April 2012. Accessed December 28, 2022.
10. Wingard JR. Prevention of infections in hematopoietic cell transplant recipients. UpToDate. Updated January 24, 2022. Accessed December 28, 2022.
11. Polverelli N, Breccia M, Benevolo G, et al. Risk factors for infections in myelofibrosis: role of disease status and treatment. A multicenter study of 507 patients. Am J Hematol. 2017;92(1):37-41. doi:10.1002/ajh.24572
12. Bach C. Ruxolitinib (Jakafi^®). OncoLink. Accessed December 28, 2022.
13. Coping with myelofibrosis. CancerCare. Accessed December 28, 2022.

Reviewed by Kyle Habet, MD, on 12/31/2022.

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Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT

Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT is a freelance medical writer and Doctor of Physical Therapy from Maryland. She has expertise in the therapeutic areas of orthopedics, neurology, chronic pain, gastrointestinal dysfunctions, and rare diseases especially Ehlers Danlos Syndrome.