Myelofibrosis is a myeloproliferative disorder characterized by aberrations in hematopoietic stem cells along with fibrosis of the bone marrow. Myelofibrosis can either be primary or secondary to other diseases such as polycythemia vera and essential thrombocythemia. Along with chronic myelogenous leukemia, polycythemia vera, essential thrombocythemia, primary myelofibrosis is termed as myeloproliferative neoplasms. In addition, primary myelofibrosis, polycythemia vera and essential thrombocythemia are termed as Philadelphia negative classical MPN. Myelofibrosis is characterized by anemia, leukocytosis/leukopenia, thrombocytosis/thrombocytopenia, extramedullary hematopoiesis, splenomegaly, hepatomegaly and symptoms associated with these changes.
Lifestyle Changes For Myelofibrosis
Although, lifestyle changes do not modify the course of myelofibrosis, it is important to eat right for many reasons. Taking proper nutrition helps prevent other systemic diseases, such as diabetes, obesity or atherosclerosis, which in turn reduces the risk of clots. In addition, when normal body weight is maintained along with healthy lifestyle, it helps prevent the risk of clots. Thrombosis, being a complication of myelofibrosis that is associated with increased mortality. Thrombosis can take place in arteries or veins. Arterial thrombosis can lead to heart attacks or strokes, which is associated with increased lipids and cholesterol in the blood. Deep vein thrombosis is an example of venous thrombosis and it is associated with level of activity and body weight. Therefore, to prevent these systemic diseases leading to increased risk of thrombosis, it is important to eat healthy and maintain a healthy lifestyle.
A healthy diet is one that is rich in fresh green vegetables and fruits, whole grains, fish, lean meat, olive oil and plenty of water to maintain hydration. Red meat, foods rich in cholesterol, sugars, processed foods, caffeinated drinks, and alcohol should be avoided. Salt consumption should also be reduced, if blood pressure is elevated. Exercise can also be incorporated to maintain blood circulation, reduce obesity, and deep vein thrombosis, in addition to reducing stress and anxiety related to the disease and maintaining vitality. It is important to avoid losing weight during the phase of active treatment, as it is important to take proper nutrition during chemotherapeutic management of the disease. It is important to consult a health care professional before starting any diet regimen, so that they can guide towards a healthy lifestyle modification. (2)
Management Of Myelofibrosis
The only curative treatment for myelofibrosis is allogeneic stem cell transplantation. Since, it is associated with increased mortality of around 35%; it is reserved for younger patients with intermediate to high risk disease who have life expectancy of less than 5 years.
Ruxolitinib (Jakafi) is a JAK inhibitor, which is the only FDA approved drug till date for the management of myelofibrosis and is known to improve the symptoms related to the disease progression. It has shown reduction in spleen size along with decrease in mortality of myelofibrosis patients. Since it has improved quality of life of patients with myelofibrosis, it can is used for symptomatic myelofibrosis in intermediate to high risk patients who fail the criteria of allogeneic stem cell transplantation.
Erythropoiesis stimulating agents have shown good results in managing anemia in patients with myelofibrosis. In addition, corticosteroids, androgenic steroids (danazol), thalidomide, lenalidomide in various combinations have been shown to increase red blood cell count. Splenomegaly and the related symptoms can be managed with hydroxyurea. Interferon alpha can be used in myelosuppression and thrombocytosis can be managed with anagrelide.
Splenectomy also carries greater complications and high mortality; therefore, it should be reserved for specific patients. These include patients with splenomegaly refractory to chemotherapy, progressive anemia, portal hypertension (e.g., variceal bleeding, ascites), severe splenic infarction, and in patients with symptoms of increased metabolism (e.g., cachexia). Although, splenic irradiation can be used as a substitute for splenectomy, the results are usually temporary lasting only about 1.5-2 months.
The clinical trials for newer drugs for the management of myelofibrosis are still ongoing. These include other JAK inhibitors (fedratinib, momelotinib, pacritinib), longer acting interferons, pomalidomide, everolimus, panobinostat, decitabine, azacitidine, and simtuzumab. (1)
- Choi CW, Bang SM, Jang S, et al. Guidelines for the management of myeloproliferative neoplasms. Korean J Intern Med. 2015;30(6):771–788. https://www.ncbi.nlm.nih.gov/pubmed/26552452