Abstract
Leaky SCID presents with reduced but not absent numbers of CD3+ T-cells and lymphocyte proliferation to phytohemagglutinin (PHA) in the absence of maternal T-cells. Despite antimicrobial pharmacologic prophylaxis patients remain susceptible to a variety of opportunistic infections. T-cells without thymic maturation in leaky SCID can undergo oligoclonal expansion, particularly in response to viral infections, leading to the development of Omenn Syndrome.
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