Genetic analysis of Chronic Active Epstein-Barr Virus

Genetic Studies of Chronic Active Epstein-Barr Virus Infection

Observational National Institutes of Health Clinical Center (CC) · NCT00032513

This study is trying to find genetic changes in children and young adults with Chronic Active Epstein-Barr Virus to better understand the disease and how the immune system responds to it.

Quick facts

Study typeObservational
Enrollment300 (estimated)
Ages1 Year to 120 Years
SexAll
SponsorNational Institutes of Health Clinical Center (CC) NIH
Locations1 site (Bethesda, Maryland)
Trial IDNCT00032513 on ClinicalTrials.gov

What this trial studies

This study focuses on identifying genetic mutations associated with Chronic Active Epstein-Barr Virus (CAEBV), a rare and severe condition primarily affecting children and young adults. It will analyze blood and tissue samples from up to 50 CAEBV patients and their relatives, as well as additional anonymous blood samples for comparison. The study aims to enhance understanding of the disease's natural history and the immunologic control of EBV infections. Participants will undergo evaluations and medically indicated studies at the NIH Clinical Center.

Who should consider this trial

Good fit: Ideal candidates include individuals aged 1 year and older with known or suspected Chronic Active Epstein-Barr Virus.

Not a fit: Patients under 1 year old or those without a confirmed diagnosis of CAEBV may not benefit from this study.

Why it matters

Potential benefit: If successful, this study could lead to better understanding and potential treatments for Chronic Active Epstein-Barr Virus.

How similar studies have performed: While studies on EBV have been conducted, this specific genetic approach to CAEBV is relatively novel and untested.

Eligibility criteria

Show full inclusion / exclusion criteria
* ELIGIBILITY CRITERIA

CAEBV PATIENTS

Study size: up to 150 patients

Sex distribution: male and female

Age range: 1 year old and above

Patients with known or suspected CAEBV and their relatives will be recruited by referrals from health care providers. The rarity of this disease will markedly limit the number of patients available to study; therefore, we will enroll patients who are 1 year of age and older. However, children under the age of 3 years old will be enrolled as samples- only participants and not be evaluated at the Clinical Center.

Patient recruitment is irrespective of racial and ethnic group or sex. NIAID investigators will be available to answer questions and discuss general aspects of CAEBV and identified genes or modifier genes with participants or their providers.

Eligibility criteria: To be considered having CAEBV, a patient must have:

1. a severe progressive illness that began as a primary EBV infection, or associated with positive EBV-specific antibody titers (IgG-VCA, ; EA or EBNA) not otherwise explained AND
2. evidence of major organ involvement such as lymphadenitis, hemophagocytosis, meningoencephalitis, persistent hepatitis, splenomegaly, interstitial pneumonitis, bone marrow hypoplasia, uveitis AND
3. detection of elevated levels of EBV DNA, RNA or proteins in affected tissues OR elevated levels of EBV DNA in the peripheral blood

Exclusion Criteria:

(a) Any other condition that, in the opinion of the investigator, would make the patient unsuitable for enrollment or could interfere with the patient participating in the study.

RELATIVES OF CAEBV PATIENTS:

Study size: up to 150 relatives of CAEBV patients

Age range: 3 years and above

To determine the genetic basis for this syndrome, the evaluation of blood, saliva and/or previously obtained tissue specimens from healthy relatives of CAEBV patients will serve as controls. No tissue biopsies will be performed on healthy relatives. We will

perform some of the studies listed on samples from normal relatives.

UNRELATED VOLUNTEERS

Study size: up to 300 persons

Age range: 18 years and above

Panels of anonymous blood samples will be obtained through NIH Clinical Center Blood Transfusion Medicine to determine if identified genetic changes are mutations or polymorphisms.

Where this trial is running

Bethesda, Maryland

Study contacts

How to participate

  1. Review the eligibility criteria above with your treating physician.
  2. Visit the official trial page on ClinicalTrials.gov for the most current contact information and recruitment status.
  3. Contact the listed study coordinator or principal investigator to request pre-screening. Pre-screening is free and never obligates you to enroll.
Conditions Chronic Active Epstein-Barr VirusHerpesHemophagocytosisEpstein-Barr VirusLymphoproliferative DiseaseMononucleosisNatural HistoryEpstein-Barr
Last reviewed 2026-06-13 by the Find a Trial editorial team. Information on this page is for educational purposes and is not medical advice. Always consult qualified healthcare professionals about clinical trial participation.