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 constitutional symptoms of weight loss, fatigue, and night sweats for patients with Janus kinase 2 (JAK2)-positive or Janus kinase 2 (JAK2)-negative primary myelofibrosis, post–essential thrombocythemia myelofibrosis, or post–polycythemia vera myelofibrosis. Hydroxyurea, chemotherapy, or radiotherapy 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.[1][2]
- Glucocorticoids: Glucocorticoids can improve the red blood cell (RBC) survival, which can be markedly decreased in some patients.[1][3]
- Androgen therapy: Danazol can be given to stimulate the production of red blood cells (RBCs).
- Erythropoietic growth factors: Erythropoietin and darbepoetin can help if patients are not dependent on transfusion.
- Thalidomide, lenalidomide, or pomalidomide: These may help in improving the red blood cell counts, thus improving anemia and help in the reversal of splenomegaly. However, patients on these drugs require prophylaxis for avoiding thrombosis and careful monitoring for hematologic toxicity.[1]
Janus kinase (JAK) Inhibitor Therapy
- Ruxolitinib, an inhibitor of JAK1 and JAK2, can reduce the splenomegaly and the symptoms of weight loss, fatigue, and night sweats in patients with Janus kinase 2 (JAK2)-positive or Janus kinase 2 (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 1.2 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
- ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
- ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.