Augmented availability of arginine could cause hyperproduction of NO in LPI, which could result in CD8+T lymphocyte activation, excessive cytokine production and ectopic migration of leukocytes leading to the immune phenotypes seen in LPI patients, such as hepatosplenomegaly, HLH and autoimmunity (18, 76). The gene discussed is CD8A; the disease is lysinuric protein intolerance.