IMMUNOLOGICAL STUDY OF THE ASPLENIA SYNDROME

Asplenia syndrome is usually associated with complex congenital cardiovascular defects. These patients often succumb to overwhelming infections. A complete immunological evaluation was therefore conducted on 13 consecutive patients with asplenia syndrome and 12 age-matched patients with congenital heart disease but without evident splenic problems. IgG, IgA, IgM and C3 and C4 values were normal for age in all subjects studied. T cell subsets, including the percentage of CD3, and CD4 cells and the CD4/CD8 ratio were significantly different in patients and controls (P < 0.01, respectively). Lymphoproliferative responses to mitogens (ConA, concanavalin A, phytohemagglutinin and pokeweed mitogen) were also decreased in patients. Fc-mediated clearance of sensitized autologous erythrocytes was significantly impaired in patients (n = 13) when compared with age-matched controls (n = 5) (clearance t1/2 59.0 +/- 9.6 minutes vs. 12.5 +/- 1.6 minutes, P < 0.001). Thus profoundly impaired reticuloendothelial clearance and decreased T cell function might account for the life-threatening infections frequently seen in patients with congenital asplenia syndrome.