Histopathologic Abnormalities of the Lymphoreticular Tissues in Organic Cation Transporter 2 Deficiency: Evidence for Impaired B Cell Maturation

Immunohistology of lymphoreticular tissues of a fatal case of organic cation transporter 2 deficiency revealed inhibited proliferation with increased apoptosis in the germinal centers, resulting in “burned out” follicles. This is indicative of impaired antigen driven B cell affinity maturation. Defective humoral immune response might explain the recurrent infections in untreated organic cation transporter 2 deficiency.