Abstract | PURPOSE OF REVIEW: The autoimmune lymphoproliferative syndrome is a recently identified human disorder of lymphocyte apoptosis that has provided important information about Fas-mediated lymphocyte apoptosis. In this review we summarize current information regarding the diagnosis, management and underlying molecular basis of the syndrome. RECENT FINDINGS: SUMMARY: Defects in multiple molecules within the Fas apoptotic pathway may result in autoimmune lymphoproliferative syndrome and, despite recent advances, a number of patients remain with unidentified genetic defects. There is also clear need for improved understanding of mechanisms underlying the development of autoimmunity in this disorder and to provide early evidence for development of malignancy. This syndrome is the first human disorder linked to a germline defect in lymphocyte apoptosis and it continues to be an area of productive research and new information regarding this process of lymphocyte homeostasis and its role in human disease.
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Authors | João B Oliveira, Thomas Fleisher |
Journal | Current opinion in allergy and clinical immunology
(Curr Opin Allergy Clin Immunol)
Vol. 4
Issue 6
Pg. 497-503
(Dec 2004)
ISSN: 1528-4050 [Print] United States |
PMID | 15640690
(Publication Type: Journal Article, Review)
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Chemical References |
- FASLG protein, human
- Fas Ligand Protein
- Membrane Glycoproteins
- Tumor Necrosis Factors
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Topics |
- Animals
- Apoptosis
(immunology)
- Autoimmune Diseases
(diagnosis, therapy)
- Fas Ligand Protein
- Genotype
- Germ-Line Mutation
- Humans
- Lymphoproliferative Disorders
(diagnosis, immunology, therapy)
- Membrane Glycoproteins
(metabolism)
- Phenotype
- T-Lymphocytes
(immunology)
- Tumor Necrosis Factors
(metabolism)
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