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Interferon-α Treatment of Six Patients with the Idiopathic Hypereosinophilic Syndrome

Joseph H. Butterfield, MD; and Gerald J. Gleich, MD
[+] Article and Author Information

From the Mayo Clinic and Foundation and the Mayo Medical School, Rochester, Minnesota. Requests for Reprints: Joseph H. Butterfield, MD, Division of Allergic Diseases, Mayo Clinic and Foundation, Rochester, MN 55905. Acknowledgments: The authors thank Drs. Ragene R. Rivera, Lawrence Schwartz, Eric Pillemer, Louis Letendre, and Robert Gillham for their referral and follow-up of patients; and Linda H. Arneson and Cheryl Adolphson for assistance in preparation of this manuscript. Grant Support: In part by grants from the National Institutes of Health (AI 15231, AI 09728, AI 31155, and RR 585), the Schering-Plough Corporation (Kenilworth, New Jersey 07033), and the Mayo Foundation.


Copyright ©2004 by the American College of Physicians


Ann Intern Med. 1994;121(9):648-653. doi:10.7326/0003-4819-121-9-199411010-00003
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Objective: To examine the response to interferon-α 2B therapy in six patients with the idiopathic hypereosinophilic syndrome.

Design: Prospective cohort study.

Setting: Tertiary referral center, university hospital inpatient and outpatient clinics, and offices of private practice physicians.

Patients: Six patients satisfying the criteria for the hypereosinophilic syndrome, five of whom were resistant to or intolerant of conventional treatment.

Intervention: Individualized dosages of interferon-α based on clinical response and side effects.

Measurements: Measurements of eosinophilia (peripheral and bone marrow counts), levels of serum eosinophil major basic protein, doses of glucocorticoid and cytotoxic medications, and transfusion requirements.

Results: Various dosages of interferon-α from 1.5 MU/d to 8 MU/d decreased the total eosinophil count to less than 1000/mm3 in five of six patients. All patients were able to taper and discontinue prednisone and hydroxyurea. Both patients with incapacitating mucosal ulcers had resolution and no recurrence of these previously resistant lesions. Interferon-α was generally well tolerated except for dose-limiting side effects, including thrombocytopenia in one patient and in a second patient, temporary worsening of mucosal lesions and constitutional symptoms.

Conclusions: Interferon-α is a valuable agent for patients with the hypereosinophilic syndrome who are resistant to or intolerant of conventional therapy and for patients with this syndrome who have incapacitating mucosal ulcers.

Figures

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Figure 1.
Summary of blood eosinophil levels and interferon-α dosages for all six patients.3

Time 0 is normalized to the beginning of interferon-α treatment for each patient. The solid line shows eosinophils/mm , the hatched area shows interferon-α (MU/d) dosages, and the vertical line at right of hatched area shows end of treatment (death in patient 2).

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Figure 2.
Response of mucosal ulcers to interferon-α. Top.Bottom.

Tongue lesion of patient 4 before interferon-α therapy. Sharply marginated, painful ulcers are present that were unresponsive to therapy with prednisone and hydroxyurea. During therapy with interferon-α, 2.5 MU/d, complete healing of the tongue is evident.

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