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Familial chronic mononucleosis

M Ballow, J Seeley, D T Purtilo

    Annals of Internal Medicine
    |December 1, 1982
    PubMed
    Summary
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    A family experienced chronic mononucleosis with persistent Epstein-Barr virus (EBV) antibodies and immune abnormalities. These findings suggest EBV can cause chronic active infection in immunodeficient states.

    Area of Science:

    • Immunology
    • Virology
    • Genetics

    Background:

    • Infectious mononucleosis, typically caused by Epstein-Barr virus (EBV), is usually self-limiting.
    • Chronic active EBV infection is a rare but serious complication.
    • Understanding the serological and immunological profiles in chronic EBV is crucial for diagnosis and management.

    Observation:

    • Two family members presented with a chronic mononucleosis syndrome characterized by persistent fatigue, recurrent infections, and immune abnormalities.
    • Serological testing revealed intermittently positive heterophil titers for over two years post-acute infection.
    • Abnormal Epstein-Barr virus-specific antibodies, including persistent R component of the early antigen complex and absent or low Epstein-Barr nuclear antigen titers, were noted.

    Findings:

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    • The proband showed persistent R component of the early antigen complex for 3 years and failed to develop normal Epstein-Barr nuclear antigen antibodies.
    • Her brother exhibited low to absent Epstein-Barr nuclear antigen titers and antibodies to both R and D components of the early antigen complex.
    • These distinct serological patterns in affected family members highlight complex EBV immune responses.

    Implications:

    • Abnormal EBV-specific serological findings in primary and acquired immunodeficiency states may indicate host attempts to control virus spread.
    • Alterations in the EBV latent state due to deficient immune responses can lead to chronic active infection.
    • This study underscores the potential for EBV to cause a chronic mononucleosis syndrome, particularly in individuals with underlying immune deficiencies.