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Chronic Active Epstein-Barr Virus (CAEBV) Infection

Rare lymphoproliferative disorder with persistent EBV infection of T or NK cells, recurrent or sustained mononucleosis-like illness, organ involvement, hemophagocytic syndrome risk, and progression to lymphoma; allogeneic hematopoietic stem cell transplant is curative.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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What is Chronic Active Epstein-Barr Virus (CAEBV) Infection?

Chronic active Epstein-Barr virus (CAEBV) infection is a rare T-cell or NK-cell lymphoproliferative disorder characterized by persistent EBV viremia, clonal or oligoclonal proliferation of EBV-infected T or NK lymphocytes, recurrent or sustained infectious mononucleosis-like symptoms lasting more than 3 months, and organ infiltration leading to a high risk of fatal complications.

Pathophysiology involves dysregulated immune control of EBV with abnormal expansion of EBV-positive T or NK cells, ongoing inflammation, hemophagocytic lymphohistiocytosis, vasculitis, organ damage, and eventual progression to overt EBV-positive lymphoma in many patients.

Diagnosis requires demonstration of high EBV DNA in peripheral blood (especially in T or NK cell fractions), exclusion of B-cell EBV disease and immunodeficiency-associated lymphoproliferation, organ biopsy showing EBER-positive lymphocytes, and a multidisciplinary assessment by hematology and infectious disease specialists.

Symptoms

Persistent or recurrent fevers, fatigue, and malaise lasting beyond 3 months
Lymphadenopathy and hepatosplenomegaly often massive and progressive
Cytopenias from bone marrow infiltration or hemophagocytic syndrome
Hepatitis with elevated transaminases, sometimes acute liver failure
Skin rash, especially hypersensitivity to mosquito bites or hydroa vacciniforme-like lymphoproliferation
Coronary aneurysms, interstitial pneumonia, central nervous system involvement, uveitis

Risk Factors

East Asian and Latin American descent, with higher incidence in Japan, China, Korea, and Mexico
Children and young adults are most commonly affected, although adult-onset cases occur
Genetic background with predisposition to dysregulated EBV immune control
History of severe primary EBV infection or hypersensitivity to mosquito bites
Coexistent immune dysregulation conditions, but most cases lack identified primary immunodeficiency
Higher risk of progression in patients with systemic CAEBV, T-cell type, or hemophagocytic complications

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent fever and lymphadenopathy beyond several weeks despite initial workup — referral to infectious disease and hematology specialists for EBV viral load and lymphocyte subset analysis
  • Suspected hemophagocytic lymphohistiocytosis with cytopenias, hepatosplenomegaly, and high ferritin — emergency hematology and intensive care assessment
  • Acute liver failure or massive hepatosplenomegaly with cytopenias — hospitalization with consideration of urgent immunosuppressive therapy
  • Coronary aneurysm, neurologic symptoms, severe pulmonary involvement — multidisciplinary cardiology, neurology, pulmonology referral
  • Diagnosed CAEBV patient — long-term hematology follow-up at a transplant center experienced in CAEBV management

Treatment Methods

01
Inflammation control with corticosteroids, etoposide-based regimens, or HLH-94/HLH-2004 protocols in patients with hemophagocytic syndrome
02
Combination immunochemotherapy regimens (e.g., cooperative therapy with steroids, etoposide, cyclosporine) to bridge to transplant
03
Allogeneic hematopoietic stem cell transplantation as the only curative therapy, ideally performed during remission or stable disease before lymphoma transformation
04
Antiviral agents (e.g., ganciclovir, foscarnet) generally have limited efficacy alone but may be used in selected scenarios
05
Long-term post-transplant monitoring for graft-versus-host disease, EBV recurrence, secondary malignancy, and organ function in survivors

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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