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Hypereosinophilic Syndrome Follow-up

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

Complications

Hypereosinophilic syndrome has many and varied complications. Their development depends entirely on which organ systems are involved in the disease process (see Presentation and Pathophysiology). The most serious complication of hypereosinophilic syndrome is cardiac involvement, which can lead to myocardial fibrosis, chronic heart failure, and death (see Pathophysiology).

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Prognosis

Hypereosinophilic syndrome carries a variable prognosis. It is a chronic and progressive disorder that is potentially fatal if left untreated. Blast transformation may occur after many years.

Although initial studies of hypereosinophilic syndrome showed a very poor prognosis (a 3-y survival rate of 12%),[4] management of cardiovascular disease by early echocardiographic monitoring and advances in medical and surgical therapies have improved the overall survival. A study of 40 cases by Lefebcve et al showed a 5-year survival of 80% and a 15-year survival of 42%.[19]

The availability of tyrosine kinase inhibitors such as imatinib, which prevent progression of cardiac disease and other organ damage—particularly in FIP1L1/PGDFRA–positive cases—will likely further improve the prognosis of hypereosinophilic syndrome. However, FIP1L1/PGDFRA– negative cases of hypereosinophilic syndrome that are resistant to corticosteroids have not been shown to have a durable response to imatinib.

Lastly, additional insight into the molecular pathogenesis of such cases of hypereosinophilic syndrome is required to develop effective targeted therapies.

Features that indicate a favorable prognosis in hypereosinophilic syndrome include the following:

  • Angioedema
  • Urticaria
  • Elevated serum IgE level
  • Sustained response to corticosteroids
  • Early diagnosis and intensive management

The presence of features that are suggestive of myeloproliferative disorder (MPD) and leukocytosis greater than 90,000/μ L carry a worse prognosis in hypereosinophilic syndrome.

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Long-term Monitoring

Periodically observe patients with hypereosinophilic syndrome to confirm that the eosinophilia is controlled and that no evidence of new or worsening organ involvement occurs. Patients should be advised to report any new or worsening symptoms.

Follow up patients with hypereosinophilic syndrome with serum troponin levels every 3 months, as well as with echocardiograms and pulmonary function tests every 6 months.

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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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