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Pediatric Hypereosinophilic Syndrome Treatment & Management

  • Author: Bruce M Rothschild, MD; Chief Editor: Harumi Jyonouchi, MD  more...
 
Updated: Aug 31, 2015
 

Medical Care

No therapy is indicated in hypereosinophilic syndrome with absence of organ damage. Presence of angioedema and urticaria suggest a benign course (see the image below). However, therapy to prevent the risk of thrombosis may be reasonable for all patients suffering from hypereosinophilic syndrome.[11]

Urticarial and erythematous rash. Urticarial and erythematous rash.

Corticosteroids have been used as first-line treatment. However, mucosal ulcers do not respond to corticosteroids. Other immunosuppresants and immunomodulating agents have been used as detailed in the medication section. These include hydroxyurea, vincristine, cyclophosphamide, busulfan, methotrexate, chlambucil, etoposide, cyclosproin, and alemtuzumab.

Rapid intervention for cardiac disease is essential.

Reducing the eosinophil load is the major goal of treatment.[10] Although seemingly important, therapeutic benefits of aggressive control of eosinophilia are hypothetical, because all reports about treatment approaches are essentially anecdotal for this rare disorder. In the absence of organ disease, any indication for treatment is unclear, except for preventive measures for thrombosis.

A small experimental study found that alemtuzumab (humanized anti-CD52 murine monoclonal antibody) was helpful in patients with advanced hypereosinophilic syndrome refractory to other standard therapies. Escalating doses of 5 mg, 10 mg, and 30 mg IV on days 1-3, and then at tolerated dose 3 times per week for 3 weeks, were suggested. If a full response is seen, weekly administration is indicated by authors. The authors also suggest clinical evaluation of alemtuzumab in a larger clinical trial is warranted.[12]

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Surgical Care

Cardiac surgery is indicated for annuloplasty, valve replacement, thrombectomy, and aortic prostheses. Because patients with mechanical valve replacements are especially prone to thrombosis, bioprostheses are recommended. Splenectomy may ameliorate platelet sequestration and is indicated for splenic infarction and pain due to splenic distention.

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Consultations

Obtaining subspecialty input from a rheumatologist and hematologist is essential.

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Contributor Information and Disclosures
Author

Bruce M Rothschild, MD Professor of Medicine, Northeast Ohio Medical University; Adjunct Professor, Department of Biomedical Engineering, University of Akron; Research Associate, University of Kansas Museum of Natural History; Research Associate, Carnegie Museum

Bruce M Rothschild, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Rheumatology, International Skeletal Society, New York Academy of Sciences, Sigma Xi, Society of Skeletal Radiology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David J Valacer, MD 

David J Valacer, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American Thoracic Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD Faculty, Division of Allergy/Immunology and Infectious Diseases, Department of Pediatrics, Saint Peter's University Hospital

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Pediatric Research, Society for Mucosal Immunology

Disclosure: Nothing to disclose.

Additional Contributors

James M Oleske, MD, MPH François-Xavier Bagnoud Professor of Pediatrics, Director, Division of Pulmonary, Allergy, Immunology and Infectious Diseases, Department of Pediatrics, Rutgers New Jersey Medical School; Professor, Department of Quantitative Methods, Rutgers New Jersey Medical School

James M Oleske, MD, MPH is a member of the following medical societies: Academy of Medicine of New Jersey, American Academy of Allergy Asthma and Immunology, American Academy of Hospice and Palliative Medicine, American Association of Public Health Physicians, American College of Preventive Medicine, American Pain Society, Infectious Diseases Society of America, Infectious Diseases Society of New Jersey, Medical Society of New Jersey, Pediatric Infectious Diseases Society, Arab Board of Family Medicine, American Academy of Pain Management, National Association of Pediatric Nurse Practitioners, Association of Clinical Researchers and Educators, American Academy of HIV Medicine, American Thoracic Society, American Academy of Pediatrics, American Public Health Association, American Society for Microbiology, Infectious Diseases Society of America, Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

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Urticarial and erythematous rash.
 
 
 
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