An Inherited Disorder of Lymphocyte Apoptosis: The Autoimmune Lymphoproliferative Syndrome

Figure 6. In normal persons, B-cell ( ) and T-cell ( ) precursors undergo development under conditions that lead to the elimination of self-reactive cells. In the context of antigen stimulation, the mature B and T cells that emerge intact from this process interact through CD40/CD40L, and the B cells differentiate into antibody-producing cells ( ). As a further safeguard against the development of self-reactive cells, the latter are susceptible to Fas-mediated apoptosis unless they are co-stimulated by specific antigen. Interleukin ( )-10 overproduction induces intracellular antiapoptotic proteins (Bcl-2 family proteins); this increases the risk that self-reactive cells will persist during B-cell and T-cell development. This problem is compounded by defective Fas-mediated apoptosis of mature cells. The result is the expansion of self-reactive cells that mediate autoimmunity.
Figure 6. In normal persons, B-cell ( ) and T-cell ( ) precursors undergo development under conditions that lead to the elimination of self-reactive cells. In the context of antigen stimulation, the mature B and T cells that emerge intact from this process interact through CD40/CD40L, and the B cells differentiate into antibody-producing cells ( ). As a further safeguard against the development of self-reactive cells, the latter are susceptible to Fas-mediated apoptosis unless they are co-stimulated by specific antigen. Interleukin ( )-10 overproduction induces intracellular antiapoptotic proteins (Bcl-2 family proteins); this increases the risk that self-reactive cells will persist during B-cell and T-cell development. This problem is compounded by defective Fas-mediated apoptosis of mature cells. The result is the expansion of self-reactive cells that mediate autoimmunity. Proposed mechanism of autoimmunity in the autoimmune lymphoproliferative syndrome. Top.BTAPCBottom.IL

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  1. Ann Intern Med April 6, 1999 vol. 130 no. 7 591-601
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