Eosinophilia medical therapy: Difference between revisions

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**0.5-1mg/kg/day of prednisone (or the equivalent dose of another steroids) is the recommended starting dose.  Patients with non-severe disease manifestations can start with lower doses <ref name=":0" />
**0.5-1mg/kg/day of prednisone (or the equivalent dose of another steroids) is the recommended starting dose.  Patients with non-severe disease manifestations can start with lower doses <ref name=":0" />
**Steroids are tapered over time, with second line agents added if needed to maintain control.
**Steroids are tapered over time, with second line agents added if needed to maintain control.
**In one study, corticosteroids were able to induce remission in 85% of patients at 1 month <ref name=":1" />
**In one study, corticosteroids were able to induce remission in 85% of patients at 1 month
*Hydroxyurea is the most commonly used second-line agent for hypereosinophilic syndrome.  Typical dose range is 500mg-2g per day.  Hydroxyurea acts at the level of the bone marrow to reduce production of new eosinophilis. <ref name=":0" /> As such it is not suitable if a rapid lowering in eosinophil levels is needed.  Hydroxyurea is not usually used alone, it is most often used as an additional steroid-sparing agents.
*Hydroxyurea is the most commonly used second-line agent for hypereosinophilic syndrome.  Typical dose range is 500mg-2g per day.  Hydroxyurea acts at the level of the bone marrow to reduce production of new eosinophilis. <ref name=":0" /> As such it is not suitable if a rapid lowering in eosinophil levels is needed.  Hydroxyurea is not usually used alone, it is most often used as an additional steroid-sparing agents.
*[[Interferon-alpha]] is also a second-line, steroid sparing agent.  Use of this agent is limited by its toxicities.  Interferon-alpha also has activity on lymphocytes, so it may be particularly effective in eosinophilia secondary to lymphocytic malignancies. <ref name=":0" />
*[[Interferon-alpha]] is also a second-line, steroid sparing agent.  Use of this agent is limited by its toxicities.  Interferon-alpha also has activity on lymphocytes, so it may be particularly effective in eosinophilia secondary to lymphocytic malignancies. <ref name=":0" />
*[[Imatinib]] and related TKIs have shown promise in treating [[chronic eosinophilic leukemia]] due to similar molecular re-arrangements as CML. <ref name=":0" /><ref>Pardanani et al. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders.  Blood 2003 101:3391-3397.</ref> This family of TKIs is very well tolerated.  These drugs are most effective in patients who have the FIP1L1-PDGFRA rearrangement driving a clonal expansion in the bone marrow. <ref name=":1" />
*[[Imatinib]] and related TKIs have shown promise in treating [[chronic eosinophilic leukemia]] due to similar molecular re-arrangements as CML. <ref>Pardanani et al. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders.  Blood 2003 101:3391-3397.</ref> This family of TKIs is very well tolerated.  These drugs are most effective in patients who have the FIP1L1-PDGFRA rearrangement driving a clonal expansion in the bone marrow.  
*Anti-IL5 antibody [[mepolizumab]] has shown potential to rapidly reduce eosinophil counts in trials and reduce the need for corticosteroids, however this agent is still investigational. <ref>Rothenberg et al. Treatment of Patients with the Hypereosinophilic Syndrome with Mepolizumab.  NEJM 2008; 358:1215-1228</ref>
*Anti-IL5 antibody [[mepolizumab]] has shown potential to rapidly reduce eosinophil counts in trials and reduce the need for corticosteroids, however this agent is still investigational. <ref>Rothenberg et al. Treatment of Patients with the Hypereosinophilic Syndrome with Mepolizumab.  NEJM 2008; 358:1215-1228</ref>
*Other cytotoxic agents have been used in isolated cases, but lack strong evidence
*Other cytotoxic agents have been used in isolated cases, but lack strong evidence

Revision as of 22:12, 29 August 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

  • Medical treatment for eosinophilia should be directed at the underlying cause (for example treating parasitic infection or malignancy).
  • Eosinophilia without evidence of organ dysfunction may be observed with monitoring for evidence of organ involvement.
  • Treatment of hypereosinophilic syndrome has a number of options including corticosteroids, hydroxyurea, interferon-alpha, imatinib and other tyrosine kinase inhibitors, IL-5 antibodies, and other cytotoxic and immunomodulatory agents.

Medical Therapy for Hypereosinophilia Syndromes

  • Corticosteroids are most commonly used as first line therapy. They are able to produce a rapid decrease in eosinophil counts, which is valuable if there is already evidence of organ dysfunction related to high eosinophil levels. [1]
    • 0.5-1mg/kg/day of prednisone (or the equivalent dose of another steroids) is the recommended starting dose. Patients with non-severe disease manifestations can start with lower doses [2]
    • Steroids are tapered over time, with second line agents added if needed to maintain control.
    • In one study, corticosteroids were able to induce remission in 85% of patients at 1 month
  • Hydroxyurea is the most commonly used second-line agent for hypereosinophilic syndrome. Typical dose range is 500mg-2g per day. Hydroxyurea acts at the level of the bone marrow to reduce production of new eosinophilis. [2] As such it is not suitable if a rapid lowering in eosinophil levels is needed. Hydroxyurea is not usually used alone, it is most often used as an additional steroid-sparing agents.
  • Interferon-alpha is also a second-line, steroid sparing agent. Use of this agent is limited by its toxicities. Interferon-alpha also has activity on lymphocytes, so it may be particularly effective in eosinophilia secondary to lymphocytic malignancies. [2]
  • Imatinib and related TKIs have shown promise in treating chronic eosinophilic leukemia due to similar molecular re-arrangements as CML. [3] This family of TKIs is very well tolerated. These drugs are most effective in patients who have the FIP1L1-PDGFRA rearrangement driving a clonal expansion in the bone marrow.
  • Anti-IL5 antibody mepolizumab has shown potential to rapidly reduce eosinophil counts in trials and reduce the need for corticosteroids, however this agent is still investigational. [4]
  • Other cytotoxic agents have been used in isolated cases, but lack strong evidence
  • In severe cases refractory to other therapy, stem cell transplant may be considered if the cause of the eosinophilia is a primary bone marrow process.

References

  1. Ogbogu et al. Hypereosinophilic syndrome: A multicenter, retrospective analysis of clinical characteristics and response to therapy. Journal of Allergy and Clinical Immunology (2009) 124(6):1319–1325
  2. 2.0 2.1 2.2
  3. Pardanani et al. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders. Blood 2003 101:3391-3397.
  4. Rothenberg et al. Treatment of Patients with the Hypereosinophilic Syndrome with Mepolizumab. NEJM 2008; 358:1215-1228

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