Myelofibrosis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]Sujit Routray, M.D. [3]

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 (PMF), 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

Glucocorticoids

Androgen therapy

  • Danazol can be given to stimulate the production of red blood cells (RBCs).
  • Preferred regimen: 600-800 mg/day till normalization of cell counts and then reduced to 200-400 mg/day

Erythropoietic growth factors

Thalidomide, lenalidomide, or pomalidomide

Janus kinase (JAK) Inhibitor Therapy

  • Platelets >200,000/mm3: 20 mg twice daily
  • Platelets 100,000 to 200,000/mm3: 15 mg twice daily
  • Platelets 50,000 to <100,000/mm3: 5 mg twice daily
  • Momelotinib is a JAK inhibitor that is currently in clinical trials.
  • Pacritinib is a JAK inhibitor that is currently in clinical trials.

Treatment for Splenomegaly

JAK2 inhibitor therapy

  • Ruxolitinib can provide temporary relief.[1][7] This medication is FDA-approved for intermediate- and high-risk myelofibrosis.

Hydroxyurea

  • Hydroxyurea can reduce the splenomegaly but may have a potential leukemogenic effect.
  • Preferred regimen: Starting dose of 500 mg/day and then subsequently adjusted to the individual efficacy.

Chemotherapy

Radiation therapy

Splenectomy

Interferon therapy

  • Peg-interferon alpha 2a and peg-interferon alpha 2b have been shown to reduce spleen size, improve constitutional symptoms, and improve blood counts.

References

  1. 1.0 1.1 1.2 1.3 1.4 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
  2. Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  3. Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  4. Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  5. Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  6. Gupta V, Kosiorek HE, Mead A, Klisovic RB, Galvin JP, Berenzon D, Yacoub A, Viswabandya A, Mesa RA, Goldberg J, Price L, Salama ME, Weinberg RS, Rampal R, Farnoud N, Dueck AC, Mascarenhas JO, Hoffman R (September 2018). "Ruxolitinib Therapy Followed by Reduced-Intensity Conditioning for Hematopoietic Cell Transplantation for Myelofibrosis: Myeloproliferative Disorders Research Consortium 114 Study". Biol. Blood Marrow Transplant. doi:10.1016/j.bbmt.2018.09.001. PMID 30205231.
  7. Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

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