Managing and Treating Myelofibrosis (MF) — Animation

This animation helps you understand how myelofibrosis (MF) is managed and treated. You'll learn about the goals of treatment, including relieving symptoms such as fatigue and pain, reducing spleen size if your spleen becomes enlarged, improving blood cell counts, and helping you feel as well as possible. It also explains how doctors assess your risk of complications and disease progression, and when approaches such as active surveillance, medications called JAK inhibitors (including ruxolitinib, fedratinib, pacritinib, and momelotinib), or stem cell transplant may be used. The animation also reviews supportive care, anemia treatments, clinical trials, and living well with MF.

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Managing and Treating Myelofibrosis (MF)

When you have myelofibrosis, your bone marrow – the factory that makes blood cells – becomes scarred and can't work properly. This affects how your body makes red blood cells, white blood cells, and platelets

The main goals of treating myelofibrosis are to improve symptoms like fatigue and pain, reduce spleen size in people with enlarged spleens, improve blood cell counts, and help you feel better overall.¹ Most treatments help manage symptoms and improve how you feel every day. While a stem cell transplant can cure MF, it is not right for everyone who has MF.

Stem cell transplant for myelofibrosis  

Stem cell transplants are usually considered for people with higher-risk MF who are healthy enough to undergo the procedure. Your doctor will take your overall health into account and may use risk-scoring tools to help determine whether a transplant is a recommended option for you. If not, other treatments are available to manage your symptoms and help you feel as well as possible. ^1,2

Risk assessment and scoring for myelofibrosis  

Doctors use scoring systems called IPSS, DIPSS, DIPSS-Plus, and MIPSS70+2.0 to learn how likely MF is to transform into leukemia.³ They count factors like age, symptoms, blood counts, and whether you have certain genetic changes. Your risk level – low, intermediate, or high – helps guide your treatment options.

The active surveillance approach

Low-risk people with no symptoms might just have checkups every 3 to 6 months. Doctors call this approach active surveillance.¹ During these visits, your doctor will check your blood counts, symptoms, and general health. If anything changes, you can start treatment as soon as necessary.

JAK inhibitors – The main treatment for myelofibrosis

Many people with MF have mutations such as JAK2, CALR, or MPL. Four approved medications can block their activity: ruxolitinib, pacritinib, momelotinib, and fedratinib. These drugs are called JAK inhibitors. They calm the signals that cause inflammation and abnormal blood cell production. They can shrink an enlarged spleen and reduce symptoms like night sweats, itching, and fatigue.²

Each JAK inhibitor works a little differently. Your doctor will choose the best one for you.

• Ruxolitinib is often used first for people with intermediate and high-risk MF.^1,2
• Pacritinib is designed for people with very low platelet counts.
• Momelotinib may help if you also have anemia, a low red cell count.
• Fedratinib is commonly used after ruxolitinib or to better control spleen symptoms.¹

Managing anemia in myelofibrosis

More than half of people with myelofibrosis have anemia when they are first diagnosed.³ Treatment depends on the cause. After ruling out causes like low iron or vitamin B12, your doctor may prescribe medications to boost red blood cell production.¹

You may receive a blood transfusion or medication to treat anemia symptoms.⁴ Options may include erythropoiesis-stimulating agents, or ESAs, luspatercept, or adjusting your JAK inhibitor therapy.¹

Other helpful medications for myelofibrosis

Hydroxyurea can help lower blood cell counts and reduce spleen size, though it is not specifically approved for myelofibrosis.⁵

Pegylated interferon may also help improve symptoms and manage blood counts.⁵

Managing an enlarged spleen

An enlarged spleen can cause pain and an early feeling of fullness when you eat. JAK inhibitors usually help shrink the spleen.² If medication does not help, low-dose radiation or surgery to remove the spleen may, although these are less common options.

Supportive care for myelofibrosis  

Beyond medication, supportive care is important.⁶ This includes appropriate exercise to help manage fatigue, eating nutritious foods, not smoking, and treating anxiety or depression. You might need regular blood transfusions. You can work with your care team to adjust your treatment as needed.

Clinical trials offer hope for myelofibrosis

Clinical trials study new treatments for myelofibrosis. They may give you access to treatments before they are widely available. Some are testing new medications or combinations of treatments, including drugs used with ruxolitinib, to see if they can improve symptoms or reduce spleen size more effectively. Talk with your doctor about clinical trials you might qualify for. Organizations like MPN Research Foundation can also help you find trials.

Living well with myelofibrosis 

Regular checkups, communicating with your doctor, and staying informed can help you live well with myelofibrosis. Many effective treatments are available to help you control MF symptoms and live as well as possible. Stay informed about your condition, work closely with your healthcare team, and remember that you're not alone on this journey.

Disclaimer: The information provided is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always consult your doctor about any questions you may have regarding a medical condition.

References

1. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Myeloproliferative Neoplasms, Version 3.2024.
   https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1477
2. Harrison CN, Schaap N, Vannucchi AM, et al. Janus kinase-2 inhibitor fedratinib in patients with myelofibrosis previously treated with ruxolitinib (JAKARTA-2): a single-arm, open-label, non-randomised, phase 2, multicentre study. Lancet Haematol. 2017 Jul;4(7):e317-e324.
3. Tefferi A. Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management. Am J Hematol. 2021 Jan;96(1):145-162.
4. Cervantes F, Alvarez-Larrán A, Hernández-Boluda JC, et al. Erythropoietin treatment of the anaemia of myelofibrosis with myeloid metaplasia: results in 20 patients and review of the literature. Br J Haematol. 2004 Nov;127(4):399-403.
5. Kiladjian JJ, Mesa RA, Hoffman R. The renaissance of interferon therapy for the treatment of myeloid malignancies. Blood. 2011 May 5;117(18):4706-4715.
6. Mesa R, Miller CB, Thyne M, , et al. Myeloproliferative neoplasms (MPNs) have a significant impact on patients' overall health and productivity: the MPN Landmark survey. BMC Cancer. 2016 Feb 27;16:167.