Pediatric Hypereosinophilic Syndrome Follow-up

  • Author: Bruce M Rothschild, MD; Chief Editor: Harumi Jyonouchi, MD   more...
 
Updated: May 27, 2010
 

Further Inpatient Care

Monitoring for eosinophil count, cardiac disease, adverse effects of treatment, and complications is essential.

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Further Outpatient Care

Monitoring for eosinophil count, cardiac disease, adverse effects of treatment, and complications is also an essential part of outpatient care.

The frequency of monitoring depends on the apparent stability of disease. Initially, weekly monitoring is indicated; motoring is performed monthly if the patient enters a chronic phase.

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Inpatient & Outpatient Medications

In the presence of organ involvement, a steroid trial is indicated. If steroidal trial fails, vincristine is used when immediate reduction of eosinophil levels is imperative. Dapsone is considered for skin involvement.

Because most treatment reports are anecdotal, therapy with alkylating agents is the next consideration. Treatment choices should be individualized for each patient.

Because of the risk of thrombotic phenomenon, antiplatelet therapy with aspirin or a NSAID, but not a COX-2–specific agent, is indicated. In the presence of actual thrombotic activity, warfarin (Coumadin) is indicated. Coumadin may also be considered in significant cardiac involvement.

In platelet-derived growth factor receptor alpha (PDGFRA)-associated hypereosinophilic syndrome, imatinib, which inhibits the activity of fusion kinase FIP1L1/PDGFRA, is a first-line treatment.

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Complications

Patients with thrombotic phenomenon require constant monitoring.

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Prognosis

The prognosis is poor, and treatment reports are anecdotal. The mean survival is 9 months. The 3-year survival rate is reported to be 12%. Survival is prolonged if sequelae of organ damage, especially cardiac, can be controlled.

Poor prognostic indicators include the following:

  • Anemia
  • Thrombocytopenia
  • A WBC count higher than 100,000/µL
  • Circulating basophilic abnormal cells
  • Abnormal bone marrow
  • An elevated vitamin B-12 level
  • Abnormal leukocyte alkaline phosphatase levels
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Patient Education

Patients and their families must be alerted to look for signs of thrombotic disease; any change in pulmonary, cardiac, or neurologic status; bruising; or a sore throat. These are indications for urgent reassessment.

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Contributor Information and Disclosures
Author

Bruce M Rothschild, MD  Professor of Medicine, Northeastern Ohio Universities College of Medicine; Adjunct Professor, Department of Biomedical Engineering, University of Akron; Adjunct Professor, Department of Anthropology, University of Kansas; Director, Arthritis Center

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, and Society of Skeletal Radiology

Disclosure: Nothing to disclose.

Specialty Editor Board

James M Oleske, MD, MPH  François-Xavier Bagnoud Professor of Pediatrics, Director, Division of Pulmonary, Allergy, Immunology and Infectious Diseases, Department of Pediatrics, 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 Pediatrics, American Public Health Association, American Society for Microbiology, Infectious Diseases Society of America, and Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

David J Valacer, MD  Consulting Staff, Hoffman La Roche Pharmaceuticals

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, and New York Academy of Sciences

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD  Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School

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 Mucosal Immunology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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