Abnormalities of cellular immunity and natural killer cells in Gaucher's disease

Gaucher's disease (GD) which is a congenital defect characterized by the accumulation of glucocerebroside in cells of the reticulo endothelial system, is associated with malignancies of the lymphoid system such as multiple myeloma, chronic lymphatic leukemia, as well as acute leukemia of childhood. We report the immunological profile of the T-cell system in GD patients and in GD carriers. We found a variable response to lectins stimulation (PHA, Con A, PWM in various concentrations) which supports the hypothesis that a persistent antigenic stimulation in GD affects all components of the immune system of those patients. A highly significant decrease in the number of NK cells was also found, which might be a predisposing factor to the increase in malignant B and other lymphoid cell proliferations described in patients with GD.