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X-linked lymphoproliferative syndrome.

J L Sullivan1, B A Woda

  • 1Department of Pediatrics, University of Massachusetts Medical School, Worcester 01655.

Immunodeficiency Reviews
|January 1, 1989
PubMed
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X-linked lymphoproliferative (XLP) syndrome causes severe Epstein-Barr virus (EBV) infections due to uncontrolled T-cell responses. Genetic testing aids in detecting carriers and presymptomatic males for potential prophylaxis.

Area of Science:

  • Immunology
  • Virology
  • Genetics

Background:

  • X-linked lymphoproliferative (XLP) syndrome is a primary immunodeficiency characterized by severe or fatal Epstein-Barr virus (EBV) infections.
  • Individuals with XLP exhibit a selective susceptibility to EBV, leading to infectious mononucleosis and acquired immunodeficiency.

Observation:

  • Males with XLP demonstrate robust cytotoxic T-cell responses prior to EBV infection, including polyclonally activated alloreactive cytotoxic T cells.
  • Fatal EBV infections in XLP males are often associated with extensive liver necrosis.
  • Survivors of acute EBV infection in XLP present with global cellular immune defects affecting T-cell, B-cell, and NK-cell functions.

Findings:

  • A hypothesis suggests that uncontrolled alloreactive T-cell responses, triggered by EBV-transformed B cells, underlie the immunopathology of XLP.

Related Experiment Videos

  • Genetic linkage studies identified XLP with restriction fragment length polymorphisms using DXS42 and DXS37 probes (Xq26-q27).

    • Implications:

    • Genetic probes enable the detection of carrier females and presymptomatic EBV-seronegative males with XLP.
    • Current treatment strategies for acute EBV infection in XLP males have been unsuccessful, shifting focus towards prophylactic measures like intravenous gammaglobulin.