Autoimmune lymphoproliferative syndrome: a human disorder of abnormal lymphocyte survival

JJH Bleesing, SE Straus, TA Fleisher - Pediatric Clinics of North America, 2000 - Elsevier
It has become apparent that in addition to developmental and functional lymphocyte
abnormalities, nonmalignant disorders exist that are characterized by alterations in the
survival of lymphocytes. The immune response to infectious agents results in the expansion
of antigen-specific lymphocytes with potent effector functions, some of which could become
harmful to the host. The maintenance of proper homeostasis requires that lymphocyte
expansion be appropriately balanced by lymphocyte elimination or death (reviewed in …