Activation and Function of Eosinophils in Conditions With Blood or Tissue Eosinophilia

NCT00001406 | Recruiting

• 2 other identifiers: 94007994-I-0079
• Study Type: observational
• Target: 800
• Locations: 1 country
• Sites: 1

Brief Summary

This study will investigate how, why and under what conditions eosinophils (a type of white blood cell) become activated and will examine their function in immune reactions. Eosinophil counts often rise in response to allergies, asthma, and parasitic worm infections. They can also go up in uncommon autoimmune conditions and, rarely, in association with tumors. Elevated levels of these cells is called eosinophilia. Usually, eosinophilia causes no apparent symptoms, but in rare cases there may be local swelling and itching, allergic lung problems, heart disease or nerve damage caused by the release of toxic substances in these cells into body tissues. Patients 1 to 100 years of age with eosinophil counts greater than 750/ml or an abnormal accumulation of eosinophils in the skin or body tissues may be eligible for this study. All participants will have a thorough medical history, physical examination and blood tests. Depending on the person's age and symptoms, other diagnostic tests may be done, including specialized studies of the eye, lungs, skin, bone marrow, nerves or heart. This is not a treatment study, and no experimental treatments will be offered. Patients who require treatment will receive standard medical care. Certain other procedures may be requested solely for research purposes. All participants will be asked to donate extra blood for laboratory studies investigating how immune cells and other immune substances in the blood act to stimulate a rise in eosinophils. In addition, some participants may undergo one or more of the following:

• Annual Follow-up evaluations - Physical examinations and blood tests to evaluate changes in the patient's condition and eosinophil counts over time.
• Bone marrow biopsy and aspiration will be recommended during the initial evaluation, and in certain patients at other times when it is important to look directly at the newly developing cells in the bone marrow. For this procedure an area of skin and bone is anesthetized with xylocaine (an anesthetic similar to that used by dentists), and a very sharp needle is used to sample the bone marrow for evaluation. Bone marrow biopsy and aspiration can have side effects of pain and/or bleeding into the skin and soft tissues at the site of the procedure. Rarely the area at the biopsy site can become infected, and is treated with antibiotics.
• Genetic testing: Some of the blood drawn from you as part of this study will be used for genetic tests. Genetic tests can help researchers study how health or illness is passed on to you by your parents or from you to your children. Any genetic information collected or discovered about you or your family will be confidential.
• Leukapheresis (only patients 18 years and older) to collect large numbers of certain cells - In this procedure, whole blood is collected through a needle placed in an arm vein. The blood circulates through a machine that separates it into its components. The white cells are then removed and the rest of the blood is returned to the body, either through the same needle used to draw the blood or through a second needle placed in the other arm.

Conditions

Eosinophilia; Helminthiasis; Hypersensitivity; Parasitic Disease; Immune System Diseases

Keywords

Eosinophils; Helminth Parasites; Hypereosinophilia; Allergy; Asthma; Natural History

Outcome Measures

Primary Outcomes (1)

• To understand the mechanisms driving eosinophilia and disease pathogenesis in patients with a wide range of eosinophilic disorders

  Identification and characterization of clinical and genetic variants of hypereosinophilic syndromes.

  Ongoing assessment

Secondary Outcomes (4)

• To assess the signs and symptoms experienced by patients with HES

  Ongoing

• To understand the mechanisms of action of therapeutic agents used or in development for the treatment of HES

  Ongoing

• To determine the mechanisms underlying eosinophil activation and recruitment to the blood and tissues

  Ongoing

• To develop a diagnostic algorithm that accurately classifies eosinophilic patients by underlying etiology

  Ongoing

Study Arms (1)

1

Volunteers with elevated eosinophil counts in the peripheral blood or tissues; or a relative of a volunteer with eosinophilia

Eligibility Criteria

• Age: 1 Year - 100 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Participants with marked eosinophilia, eosinophilia in tissues or suspected eosinophilic end organ involvement will be seen on this protocol. Evaluation of family members may be of interest when a genetic cause of eosinophilia in suspected in a study participant.

You may qualify if:

• To be eligible to participate in this study, an individual must meet all of the following criteria:
• Stated willingness to comply with all study procedures and availability for the duration of the study
• Male or female, aged 1-100
• Ability of subject (or Legally Authorized Representative (LAR)) to understand and sign a written informed consent document
• Eosinophilic Patients only:
• Documented peripheral blood count \>1500/mm3, tissue eosinophilia (abnormal accumulation of eosinophils in the skin or other body tissues) or suspected eosinophilic end organ involvement
• Primary (non-NIH) physician for routine medical care
• Relatives only:
• Extended family member of an eosinophilic participant on this protocol

You may not qualify if:

• An individual who meets any of the following criteria will be excluded from participation in this study:
• Any condition(s) or diagnosis, physical and/or psychological, that the investigator feels precludes the patient from participation in the study.
• Relatives only:
• Females must not be pregnant

Sponsors & Collaborators

• National Institute of Allergy and Infectious Diseases (NIAID): lead

Study Sites (1)

National Institutes of Health Clinical Center

Bethesda, Maryland, 20892, United States

RECRUITING

Related Publications (6)

• Ezekwe EAD Jr, Khoury P, Nutman TB. Anaphylaxis. J Allergy Clin Immunol Pract. 2024 Jan;12(1):262-263.e12. doi: 10.1016/j.jaip.2023.09.028. No abstract available.

  PMID: 38185496 DERIVED

• Stokes K, Yoon P, Makiya M, Gebreegziabher M, Holland-Thomas N, Ware J, Wetzler L, Khoury P, Klion AD. Mechanisms of glucocorticoid resistance in hypereosinophilic syndromes. Clin Exp Allergy. 2019 Dec;49(12):1598-1604. doi: 10.1111/cea.13509. Epub 2019 Oct 27.

  PMID: 31657082 DERIVED

• Kuang FL, Legrand F, Makiya M, Ware J, Wetzler L, Brown T, Magee T, Piligian B, Yoon P, Ellis JH, Sun X, Panch SR, Powers A, Alao H, Kumar S, Quezado M, Yan L, Lee N, Kolbeck R, Newbold P, Goldman M, Fay MP, Khoury P, Maric I, Klion AD. Benralizumab for PDGFRA-Negative Hypereosinophilic Syndrome. N Engl J Med. 2019 Apr 4;380(14):1336-1346. doi: 10.1056/NEJMoa1812185.

  PMID: 30943337 DERIVED

• Khoury P, Desmond R, Pabon A, Holland-Thomas N, Ware JM, Arthur DC, Kurlander R, Fay MP, Maric I, Klion AD. Clinical features predict responsiveness to imatinib in platelet-derived growth factor receptor-alpha-negative hypereosinophilic syndrome. Allergy. 2016 Jun;71(6):803-10. doi: 10.1111/all.12843. Epub 2016 Mar 2.

  PMID: 26797802 DERIVED

• Khoury P, Herold J, Alpaugh A, Dinerman E, Holland-Thomas N, Stoddard J, Gurprasad S, Maric I, Simakova O, Schwartz LB, Fong J, Lee CC, Xi L, Wang Z, Raffeld M, Klion AD. Episodic angioedema with eosinophilia (Gleich syndrome) is a multilineage cell cycling disorder. Haematologica. 2015 Mar;100(3):300-7. doi: 10.3324/haematol.2013.091264. Epub 2014 Dec 19.

  PMID: 25527564 DERIVED

• Khoury P, Zagallo P, Talar-Williams C, Santos CS, Dinerman E, Holland NC, Klion AD. Serum biomarkers are similar in Churg-Strauss syndrome and hypereosinophilic syndrome. Allergy. 2012 Sep;67(9):1149-56. doi: 10.1111/j.1398-9995.2012.02873.x. Epub 2012 Jul 9.

  PMID: 22775568 DERIVED

Related Links

• NIH Clinical Center Detailed Web Page

MeSH Terms

Conditions

Immune System Diseases; Eosinophilia; Helminthiasis; Hypersensitivity; Parasitic Diseases; Asthma

Condition Hierarchy (Ancestors)

Leukocyte Disorders; Hematologic Diseases; Hemic and Lymphatic Diseases; Infections; Bronchial Diseases; Respiratory Tract Diseases; Lung Diseases, Obstructive; Lung Diseases; Respiratory Hypersensitivity; Hypersensitivity, Immediate

Study Officials

• Amy D Klion, M.D.

  National Institute of Allergy and Infectious Diseases (NIAID)

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Lori A Penrod, R.N.

CONTACT

(240) 627-3647; lpenrod@niaid.nih.gov

Amy D Klion, M.D.

CONTACT

(240) 381-6073; amy.klion@nih.gov

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: November 3, 1999
• First Posted: November 4, 1999
• Study Start: April 21, 1997
• Last Updated: April 24, 2026

Record last verified: 2026-04-22

Locations

US (1)