Autoimmune Lymphoproliferative Syndrome: Types I, II and Beyond

  • Hyung J. Chun
  • Michael J. Lenardo
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 490)


An essential element in the maintenance of homeostasis of the immune system is the capacity for apoptosis, or programmed cell death. Apoptosis limits the accumulation of lymphocytes, and minimizes reactions against self-antigen that can lead to autoimmunity1,2. Lymphocyte apoptosis occurs in at least two major forms: antigen receptor engagement and lymphokine withdrawal. These forms of death are controlled in a negative feedback mechanism termed propriocidal regulation1,3. Antigen-mediated death of lymphocytes is regulated by Fas (CD95/APO-1), tumor necrosis factor receptor (TNFR), and related molecules4-6. Passive apoptosis via lymphokine withdrawal may result from the cytoplasmic activation of caspases that is closely regulated by the mitochondria and the members of the Bcl-2 family of proteins. Apoptosis of other immune cells such as dendritic cells may also contribute to immune homeostasis7.


Lymphocyte Apoptosis Autoimmune Lymphoproliferative Syndrome Research Scholar Program Antigen Receptor Engagement Dendritic Cell Death 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Science+Business Media New York 2001

Authors and Affiliations

  • Hyung J. Chun
    • 1
    • 2
  • Michael J. Lenardo
    • 1
  1. 1.Laboratory of Immunology, National Institute of Allergy and Infectious DiseasesNational Institutes of HealthBethesdaUSA
  2. 2.Howard Hughes Medical Institute—National Institutes of Health Research Scholars ProgramUSA

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