Abstract
Recent studies have indicated that autoimmune thrombocytopenic purpura (ATP) patients show immune system alterations that are not restricted to the B-cell compartment, but that also affect T lymphocytes. This report studies the phenotypic characteristics of natural killer (NK) cells in the peripheral blood of ATP patients, as well as their clinical significance in 33 ATP patients with active disease. Ten patients had stable disease (sustained platelet counts > 50,000/microL without the need for treatment), whereas 23 patients had therapy- dependent disease (platelet counts < 50,000/microL). A significant increase in both CD56+ CD3- NK cells and CD56+ CD3+ cytotoxic T lymphocytes was observed in peripheral blood mononuclear cells and in purified CD2+ cells from therapy-dependent ATP patients as compared with ATP patients with stable disease and healthy controls. Moreover, there were more major histocompatibility complex (MHC) class II molecules in the CD56+ CD3- cells from the therapy-dependent patients' peripheral blood preparations than there were in the stable ATP patients' and healthy controls' peripheral blood preparations. This growth in the number of CD56+ CD3- NK cells was statistically higher in patients whose disease was refractory to conventional therapy (corticosteroids and splenectomy). In addition to the CD56+ CD3- NK cells, the percentage of CD3+ T lymphocytes and their proliferative response to phytohemagglutinin (PHA) stimulation were studied in fresh CD2+ preparations from nine patients with stable disease, 22 patients with therapy-dependent disease, and 26 healthy controls. The proliferative response of CD2+ lymphocytes from both groups was similar and significantly defective with respect to that found in healthy controls. In conclusion, clinically severe ATP (therapy-dependent disease) is associated with a significant increase of CD56+ CD3- NK cells, which is particularly marked in patients whose disease is refractory to therapy.