Idiopathic CD4 Lymphopenia and Its Spectrum of Complications

This rare disease is associated with a variety of opportunistic infections and cancers distinct from that seen in other immunodeficiencies.

Idiopathic CD4 lymphocytopenia (ICL; CD4 T-cell counts <300/µL on repeated tests) is an uncommon disease of unknown origin, not associated with any known primary or secondary immunodeficiency. First identified in 1992, the syndrome is so rare that, for this observational study, the NIH could only enroll 108 cases (median age, 48; 49% female) between January 2009 and March 2020. Participants were followed for a median 3 years.

Of the 91 patients with confirmed ICL who were further analyzed and followed, 64% presented with an atypical infection resulting in the ICL diagnosis at work-up; infectious complications occurred in another 5% during follow-up. In all, 36% had autoimmune conditions. Median CD4 and CD8 counts (80/µL and 130/µL, respectiv…

Reviewing Author

Thomas Glück, MD

Disclosures

Editorial BoardsConsilium Infectiorum by InfectoPharm, Infection

Thomas Glück, MD

Disclosures

Editorial BoardsConsilium Infectiorum by InfectoPharm, Infection

Idiopathic CD4 Lymphopenia and Its Spectrum of Complications

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Reviewing Author