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Hypereosinophilic Syndrome Medication

  • Author: Venkata Anuradha Samavedi, MBBS, MD; Chief Editor: Emmanuel C Besa, MD  more...
 
Updated: Feb 23, 2016
 

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications in patients with hypereosinophilic syndrome. Glucocorticoids are the first-line therapy in patients without the FIP1L1/PDGFRA mutation, while imatinib is the first-line choice in patients with the FIP1L1/PDGFRA mutation. A variety of second-line agents may be used for refractory cases.

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Corticosteroids

Class Summary

Corticosteroids often cause a rapid reduction in level of the eosinophilia. The mechanisms for this are not entirely clear.

Prednisone (Deltasone, Rayos)

 

Initial DOC. Once eosinophils are suppressed, the dose may be slowly tapered. Patients whose condition responds to steroids tend to have a better prognosis.

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Antineoplastic Agents

Class Summary

Chemotherapeutic agents may be used in patients whose conditions are refractory to steroid treatment. As a group, antineoplastic agents interfere with the production of eosinophils, but they may also cause toxicity to normal tissues, especially the bone marrow.

Hydroxyurea (Hydrea, Droxia)

 

Second line of treatment. Goal is to reduce total white blood cell (WBC) count to < 10,000 cells/µL. One week of therapy may be required before a reduction of the eosinophil count is observed. Anemia and thrombocytopenia are common complications associated with this drug.

Vincristine (Vincasar PFS)

 

May be instituted in patients whose condition fails or is only partially responsive to hydroxyurea. A response is often observed within 1-3 d. Marrow suppression is less common than with hydroxyurea, but occurrence of neurologic toxicity may limit treatment and closely resemble the neurologic symptoms of hypereosinophilic syndrome.

Chlorambucil (Leukeran)

 

Primary alkylating agent used in cases in which prednisone fails and in those patients who cannot tolerate hydroxyurea or vincristine. A reasonable alternative for long-term treatment. Bone marrow suppression may be a problem.

Etoposide (Toposar)

 

Etoposide is a glycosidic derivative of podophyllotoxin that exerts a cytotoxic effect by stabilizing the normally transient covalent intermediates formed between the DNA substrate and topoisomerase II. The drug leads to single-stranded and double-stranded DNA breaks that arrest cellular proliferation in the late S or early G2 phase of cell cycle.

Cytarabine

 

An antimetabolite antineoplastic agent, cytarabine is converted intracellularly to the active compound cytarabine-5'-triphosphate, which inhibits DNA polymerase. It is metabolized in the liver, with a half-life of 1-3 h. The agent is widely distributed, including in the central nervous system and tears, after IV administration.

Imatinib (Gleevec)

 

Imatinib is specifically designed to inhibit the tyrosine kinase activity of bcr-abl kinase in Ph1-positive leukemic CML cell lines. It is well absorbed after oral administration, with maximum concentrations achieved within 2-4 hours. Elimination is primarily in feces in the form of metabolites.

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Immunomodulators

Class Summary

Immunomodulators are naturally produced proteins with antiviral, antitumor, and immunomodulatory actions. Alpha, beta, and gamma interferons may be given topically, systemically, and intralesionally. These agents have demonstrated efficacy in small trials.

Peginterferon alfa 2a (Pegasys, Pegasys ProClick)

 

Has been reported to effectively suppress eosinophilia in several different patients using several different doses. Some patients have had progression of disease despite therapy.

Interferon alpha-2b (Intron A)

 

Has been reported to effectively suppress eosinophilia in several different patients using several different doses. Some patients have had progression of disease despite therapy.

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Contributor Information and Disclosures
Author

Venkata Anuradha Samavedi, MBBS, MD Internist in Houston, TX

Disclosure: Nothing to disclose.

Coauthor(s)

Paul Schick, MD Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology

Disclosure: Nothing to disclose.

Ronald A Sacher, MB, BCh, FRCPC, DTM&H Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada, American Clinical and Climatological Association, International Society of Blood Transfusion

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: GSK Pharmaceuticals,Alexion,Johnson & Johnson Talecris,,Grifols<br/>Received honoraria from all the above companies for speaking and teaching.

Vincent E Herrin, MD, FACP Professor of Medicine, Division of Hematology and Medical Oncology, Director, Medicine Residency Program, University of Mississippi School of Medicine

Vincent E Herrin, MD, FACP is a member of the following medical societies: American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Society of Clinical Oncology, Southern Society for Clinical Investigation, American Society of Hematology

Disclosure: Nothing to disclose.

Joe C Files, MD Director, Division of Hematology, Associate Chairman, Professor, Department of Internal Medicine, University of Mississippi Medical Center

Joe C Files, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association for Cancer Education, American College of Physicians, American Federation for Medical Research, American Heart Association, American Medical Association, American Society of Human Genetics, Mississippi State Medical Association, New York Academy of Sciences, Southern Medical Association

Disclosure: Nothing to disclose.

Youwen Zhou, MD, PhD, FRCPC Associate Professor, Department of Dermatology and Skin Science, University of British Columbia Faculty of Medicine; Director, Hyperhidrosis Specialty Clinic, Co-Director, Psoriasis and Phototherapy Centre, Consulting Physician, Department of Dermatology, Vancouver General Hospital; Co-Director, Vitiligo and Pigmentation Clinic, Oncologist Consultant, Skin Tumor Program, BC Cancer Agency

Youwen Zhou, MD, PhD, FRCPC is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Antoni Ribas, MD Assistant Professor of Medicine, Division of Hematology-Oncology, University of California at Los Angeles Medical Center

Disclosure: Nothing to disclose.

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Indurated edematous plaques of hypereosinophilic syndrome on a patient's legs.
Erythroderma in a patient with hypereosinophilic syndrome.
 
 
 
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