Evaluation of immunoregulatory cells in Duchenne muscular dystrophy and spinal muscular atrophy among African and Indian patients

Abstract

Suppressor cells were assayed by numerical and functional tests in Duchenne muscular dystrophy (DMD) and spinal muscular atrophy (SMA) among African and Indian children in order to contribute to an understanding of the pathogenesis of these neurological disorders. Peripheral blood mononuclears (PBM) were classified as total T cells and T cell subsets by the OKT series of monoclonal antibodies and as B cells by the presence of surface immunoglobulin. The suppressive effects of PBM pretreated with concanavalin A (Con A) on normal homologous phytohaemagglutinin (PHA) transformation of mononuclear cells was determined. PBM stimulation by PHA was also assessed. Patients with DMD had a significant increase ( P = 0.0353) in the number of T suppressor/cytotoxic cells (1218 ± 142 cells/mm 3, mean ± SE) as compared to controls (815 ± 95 cells/mm 3) and a significant reduction ( P = 0.0282) in OKT4 + cells expressed as a percentage of OKT3 +, 50% ± 3 compared to 61% ± 3. No differences were detected in any of the numerical assays employed in SMA as compared to controls, or within SMA patients according to severity of disease. Suppressor function and PHA transformation were normal in both groups of patients. No significant correlations were detected between numerical and functional assays of suppression. The implication of the results obtained for the role of immunoregulatory cells in the pathogenesis of DMD in these children is discussed.