Myelofibrosis medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
Red blood cell transfusion, danazol therapy, or thalidomide are recommended for patients who develop anemia. Ruxolitinib, an inhibitor of Janus kinase 1 (JAK1) and Janus kinase 2 (JAK2), can reduce the splenomegaly and the symptoms of weight loss, fatigue, and night sweats for patients with JAK2-positive or JAK2-negative primary myelofibrosis, post–essential thrombocythemia myelofibrosis, or post–polycythemia vera myelofibrosis. Hydroxyurea, chemotherapy, radiotherapy, or splenectomy are recommended for patients who develop splenomegaly.
Medical Therapy
Treatment for Anemia
- Red blood cell transfusion:The profound anemia that develops in this disease usually requires red blood cell transfusion. Red blood cell survival is markedly decreased in some patients; this can sometimes be treated with glucocorticoids.[1]
- Androgen therapy: Danazol may stimulate the production of red blood cells and decrease the anemia.
- Erythropoietic growth factors: Erythropoietin and darbepoetin are less likely to help when patients are transfusion dependent or manifest a serum erythropoietin level greater than 125 U/L.
- Thalidomide, lenalidomide, or pomalidomide: These drugs may help in elevating the red blood cell counts, thus improving anemia, and reverse the splenomegaly. However, patients on thalidomide, lenalidomide, or pomalidomide require prophylaxis for avoiding thrombosis and careful monitoring for hematologic toxicity.[1]
JAK2 Inhibitor Therapy
- Ruxolitinib, an inhibitor of JAK1 and JAK2, can reduce the splenomegaly and the debilitating symptoms of weight loss, fatigue, and night sweats for patients with JAK2-positive or JAK2-negative primary myelofibrosis, post–essential thrombocythemia myelofibrosis, or post–polycythemia vera myelofibrosis.
Treatment for Splenomegaly
- JAK2 inhibitor therapy: Painful splenomegaly can be treated temporarily with ruxolitinib.
- Hydroxyurea: Hydroxyurea can reduce the splenomegaly but may have a potential leukemogenic effect.
- Chemotherapy: Drugs like thalidomide, lenalidomide, or cladribine may reduce the spleen size, and relieve the pain symptoms associated with it.
- Radiation therapy: Radiation may be used to kill the cells and reduce the size of the spleen, when splenectomy is contraindicated.
- Splenectomy: Surgical removal of the spleen.
References
- ↑ 1.0 1.1 Treatment overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016