eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

Hypereosinophilic Syndrome: Follow-up

Author: Bruce M Rothschild, MD, Professor of Medicine, The Northeastern Ohio Universities College of Medicine; Director, Arthritis Center of Northeast Ohio; Adjunct Professor, Department of Biomedical Engineering, University of Akron
Contributor Information and Disclosures

Updated: Aug 15, 2008

Follow-up

Further Inpatient Care

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

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.

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 PDGFRA-associated hypereosinophilic syndrome, imatinib, which inhibits the activity of fusion kinase FIP1L1/PDGFRA, is a first-line treatment.

Complications

  • Patients with thrombotic phenomenon require constant monitoring.

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

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.

Miscellaneous

Medicolegal Pitfalls

  • Be sure to eliminate other diagnostic possibilities.
  • Be alert for thrombotic events.

Special Concerns

  • Attention to cardiac involvement and thrombotic phenomenon is essential.
 


More on Hypereosinophilic Syndrome

Overview: Hypereosinophilic Syndrome
Differential Diagnoses & Workup: Hypereosinophilic Syndrome
Treatment & Medication: Hypereosinophilic Syndrome
Follow-up: Hypereosinophilic Syndrome
References
[ CLOSE WINDOW ]

References

  1. Martinelli G, Rondoni M, Ottaviani E, Paolini S, Baccarani M. Hypereosinophilic syndrome and molecularly targeted therapy. Semin Hematol. 2007;44(Suppl 2):S4-S16.

  2. James J. Pediatric hypereosinophilic syndrome (HES) differs from adult HES. Pediatrics. 2006;118:S49-S50. [Full Text].

  3. Carey JP, Burke AC. Transient hypereosinophilia in the infant of a mother with hypereosinophilic syndrome. Arch Intern Med. Sep 1982;142(9):1754-5. [Medline].

  4. Roche-Lestienne C, Lepers S, Soenen-Cornu V, et al. Molecular characterization of the idiopathic hypereosinophilic syndrome (HES) in 35 French patients with normal conventional cytogenetics. Leukemia. May 2005;19(5):792-8. [Medline].

  5. Schoch C, Reiter A, Bursch S, et al. Chromosome binding analysis, FISH and RT-PCR performed in parallel in hyperesosinophilic syndrome establishes the diagnosis of chronic eosinophilic leukemia in 22% of cases: A study of 40 patients. Blood. 2004;104:2444.

  6. Miyazawa K, Kakazu N, Ohyashiki K. Clinical features of hypereosinophilic syndrome: FIP1L1-PDGFRA fusion gene-positive disease is a distinct clinical entity with myeloproliferative features and a poor response to corticosteroid. Int J Hematol. Jan 2007;85(1):5-10. [Medline].

  7. [Best Evidence] Rothenberg ME, Klion AD, Roufosse FE, et al. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. N Engl J Med. Mar 20 2008;358(12):1215-28. [Medline].

  8. Adame J, Cohen PR. Eosinophilic panniculitis: diagnostic considerations and evaluation. J Am Acad Dermatol. Feb 1996;34(2 Pt 1):229-34. [Medline].

  9. Adams HW, Mainz DL. Eosinophilic ascites. A case report and review of the literature. Am J Dig Dis. Jan 1977;22(1):40-2. [Medline].

  10. Alfaham MA, Ferguson SD, Sihra B, Davies J. The idiopathic hypereosinophilic syndrome. Arch Dis Child. Jun 1987;62(6):601-13. [Medline].

  11. Anders HJ, Schattenkirchner M. Destructive joint lesions and bursitis in idiopathic hypereosinophilic syndrome. Rheumatology (Oxford). Feb 1999;38(2):185-6. [Medline].

  12. Bain BJ. Eosinophilic leukaemias and the idiopathic hypereosinophilic syndrome. Br J Haematol. Oct 1996;95(1):2-9. [Medline].

  13. Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). Jan 1975;54(1):1-27. [Medline].

  14. Cryer PE, Kissane J. Hypereosinophilic syndrome with pulmonary hypertension. 1976;60:239-247.

  15. Davies J, Spry CJ, Sapsford R, et al. Cardiovascular features of 11 patients with eosinophilic endomyocardial disease. Q J Med. Winter 1983;52(205):23-39. [Medline].

  16. Flaum MA, Schooley RT, Fauci AS, Gralnick HR. A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. I. Hematologic manifestations. Blood. Nov 1981;58(5):1012-20. [Medline].

  17. Kang EY, Shim JJ, Kim JS, Kim KI. Pulmonary involvement of idiopathic hypereosinophilic syndrome: CT findings in five patients. J Comput Assist Tomogr. Jul-Aug 1997;21(4):612-5. [Medline].

  18. Katz HT, Haque SJ, Hsieh FH. Pediatric hypereosinophilic syndrome (HES) differs from adult HES. J Pediatr. Jan 2005;146(1):134-6. [Medline].

  19. Layzer RB, Shearn MA, Satya-Murti S. Eosinophilic polymyositis. Ann Neurol. Jan 1977;1(1):65-71. [Medline].

  20. Lierman E, Folens C, Stover EH, et al. Sorafenib is a potent inhibitor of FIP1L1-PDGFRalpha and the imatinib-resistant FIP1L1-PDGFRalpha T674I mutant. Blood. Aug 15 2006;108(4):1374-6. [Medline].

  21. Parrillo JE, Borer JS, Henry WL, et al. The cardiovascular manifestations of the hypereosinophilic syndrome. Prospective study of 26 patients, with review of the literature. Am J Med. Oct 1979;67(4):572-82. [Medline].

  22. Postovsky S, Daitzchman M, Dale A, Elhasid R, Ben Arush MW. Unusual presentation of mastoid eosinophilic granuloma in a young patient. Pediatr Hematol Oncol. Jun 2001;18(4):283-9. [Medline].

  23. Spark RP, Gleason DM, DeBenedetti CD, Gigax JH. Is eosinophilic ureteritis an entity? 2 case reports and review. J Urol. Jun 1991;145(6):1256-60. [Medline].

  24. Tefferi A. Blood eosinophilia: a new paradigm in disease classification, diagnosis, and treatment. Mayo Clin Proc. Jan 2005;80(1):75-83. [Medline].

  25. van den Hoogenband HM. Skin lesions as the first manifestation of the hypereosinophilic syndrome. Clin Exp Dermatol. May 1982;7(3):267-71. [Medline].

  26. Wardlaw AJ, Moqbel R, Kay AB. Eosinophils: biology and role in disease. Adv Immunol. 1995;60:151-266. [Medline].

  27. Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. May 15 1994;83(10):2759-79. [Medline].

  28. White WL, Wahner HW, Brown ML, James EM. Sequential liver imaging in the hypereosinophilic syndrome: discordant images with scintigraphy, ultrasound, and computed tomography. Clin Nucl Med. Feb 1981;6(2):75-7. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

disseminated eosinophilic collagen disease, endomyocardial disease, eosinophilia, eosinophilic leukocytosis, myeloproliferative, lymphocytic, idiopathic, hypereosinophilic syndrome, endomyocardial fibrosis, platelet-derived growth factor receptor alpha, PDGFRA, secondary endocarditis, anemia, thrombocytopenia, ascites, hepatic thrombosis, Budd-Chiari syndrome, Raynaud phenomenon, thrombotic phenomenon, mastitis, dementia, endomyocardial fibrosis, myocarditis, arrhythmia, congestive heart failure, valvular incompetence, mitral regurgitation

tricuspid regurgitation, aortic valve disease, vesiculobullous rash, papulonodular rash, livido reticularis, angioedema, cellulitis, erythroderma, erythema annulare, subungual petechiae, digital necrosis, multifocal bursitis, pauciarticular arthritis, small-bowel necrosis, sclerosing cholangitis, chronic active hepatitis, enterocolitis, pancreatitis, hepatosplenomegaly, pleuritis, pulmonary hypertension, encephalopathy, pupillotonia, keratoconjunctivitis sicca, episcleritis, retinal vein thrombosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Bruce M Rothschild, MD, Professor of Medicine, The Northeastern Ohio Universities College of Medicine; Director, Arthritis Center of Northeast Ohio; Adjunct Professor, Department of Biomedical Engineering, University of Akron
Bruce M Rothschild, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Rheumatology, American Federation for Clinical Research, American Heart Association, American Society for Clinical Pharmacology and Therapeutics, International Skeletal Society, New York Academy of Sciences, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

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: "no financial interest" None None

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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.

CME Editor

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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.