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Pediatric Hypereosinophilic Syndrome Workup

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

Laboratory Studies

Hypereosinophilic syndrome is characterized by an eosinophil count of more than 1500/μL (usually many more).

It is necessary to evaluate for a 4q12 microdeletion (800 kb) that produces recombination fusion, forming the FIP1L1/PDGFRA fusion gene.[7, 8] The formation of the FIP1L1/PDGFRA fusion gene results in increased tyrosine kinase activity of PDGFRA, rendering poor response to steroids but sensitivity to imatinib.[9]

The erythrocyte sedimentation rate (ESR) is usually elevated but can be normal. Leukocyte alkaline phosphatase levels may be high or low. Hypergammaglobulinemia in the form of extremely high immunoglobulin E (IgE) levels is noted in one third of patients. Immunoglobulin G (IgG), immunoglobulin M (IgM), and immunoglobulin A (IgA) levels are only rarely elevated.

Rheumatoid factor is only rarely present. Coombs test may be positive. Results of rapid plasma reagin (RPR) tests are rarely positive. Hematuria, proteinuria, and azotemia may be present. Synovial fluid eosinophilia is prominent.

Chromosomal analysis rarely reveals abnormalities. When abnormal chromosomes are present, a workup for eosinophilic leukemia is recommended. Eosinophilic leukemia is an unrelated disorder. The presence of chromosome abnormalities or eosinophil blast cells in the circulating blood or marrow may allow the leukemia to be recognized.

A laboratory workup is also indicated to rule out disorders that can mimic the hypereosinophilic syndrome (see Differentials).

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Imaging Studies

Chest radiography may reveal nonspecific focal or diffuse, interstitial, or alveolar infiltrates. CT scanning may reveal pulmonary nodules with a halo of ground-glass attenuation, as well as nonspecific focal or diffuse, interstitial, or alveolar infiltrates. Ultrasonography findings of the affected liver may be normal. Technetium-99m sulfur colloid scanning may reveal nonhomogenous uptake in the presence of liver disease.

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Other Tests

Electrocardiography may reveal atrial fibrillation, ST-segment depression, T-wave changes, conduction abnormalities, left atrial atrophy, or left ventricular hypertrophy. Electrocardiography reveals cardiac involvement in 90% of patients with hypereosinophilic syndrome.

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Procedures

Bone marrow biopsy may reveal myelofibrosis. The presence of eosinophil blast cells suggests the alternative diagnosis of eosinophilic leukemia, an unrelated disorder.

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Histologic Findings

Activated eosinophil levels are noted in the blood and involved structures; this finding is associated with fibrosis.

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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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