This site is intended for healthcare professionals

Go to /sign-in page

You can view 5 more pages before signing in

Treatment

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Treatment is palliative, and may include:

  • for anaemia
    • blood transfusions for anaemia
    • administration of recombinant erythropoietin may be beneficial
    • androgens - e.g. oxymetholone, nandrolone or danazol - to reduce transfusion requirement; but poorly tolerated by women
  • for splenomegaly
    • therapy should be considered only in patients who are truly symptomatic (1)
    • hydroxyurea, - most commonly
      • most commonly used initial medical therapy in splnomegaly
      • to reduce hypermetabolism, leucocytosis and thrombocytosis
      • adverse effects include myelosuppression which may exacerbate the underlying anaemia (1)
    • alkylating agents - busulphan, melphalan or chlorambucil
      • can be used in some patients with splenomegaly although there is a risk of potential myelosuppression and increased risk of eventual blastic transformation (1)
  • for both anaemia and splenomegaly
    • agents which improves splenomegaly do so by non specific myelosuppression effects
    • immunomodulatory drugs (IMiDs) have shown that it has the ability to improve both the conditions e.g. - thalidomide, lenalidomide and pomalidomide (investigational agent) (1)
  • splenectomy is indicated if:
    • the spleen becomes very large and painful
    • transfusion requirement are high
    • thrombocytopenia is severe
  • newer drugs
    • immunomodulatory drugs (IMiDs)
    • hypomethylating agents - azacitidine and decitabine
    • JAK2 Inhibitors (1,2)
      • the identification of driver mutations in JAK2, CALR, and MPL has contributed to a better understanding of disease pathogenesis, implicating near-universal upregulation of JAK-STAT signaling, and has led to the development of the sole targeted therapy for MF, the JAK2 inhibitor ruxolitinib

Reference:


Create an account to add page annotations

Add information to this page that would be handy to have on hand during a consultation, such as a web address or phone number. This information will always be displayed when you visit this page

The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

Connect

Copyright 2024 Oxbridge Solutions Limited, a subsidiary of OmniaMed Communications Limited. All rights reserved. Any distribution or duplication of the information contained herein is strictly prohibited. Oxbridge Solutions receives funding from advertising but maintains editorial independence.