Autoimmune lymphoproliferative syndrome classification: Difference between revisions
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; David Teachey, MD [2]
Overview
Classification of Autoimmune lymphoproliferative syndrome is done by following the revised diagnostic criteria and classification guidelines came from an international workshop held in NIH in September 2009.
Classification
There is no established system for the staging of Autoimmune Lymphoproliferative Syndrome. Old nomenclature[1]
- IA - Fas
- IB - Fas ligand
- IIA - Caspase 10
- IIB - Caspase 8
- III - unknown
- IV - Neuroblastoma RAS viral oncogene homolog
Revised nomenclature (2010)[2]
- ALPS-FAS: Fas. Germline FAS mutations. 70% of patients. Autosomal dominant. Dominant negative and haploinsufficient mutations described.[3]
- ALPS-sFAS: Fas. Somatic FAS mutations in DNT compartment.[4] 10% of patients
- ALPS-FASL: Fas ligand. Germline FASL mutations. 3 reported cases
- ALPS-CASP10: Caspase 10. Germline CASP10 mutation. 2% of patients
- ALPS-U: Undefined. 20% of patients
- CEDS: Caspase 8 deficiency state. No longer considered a subtype of ALPS but a distinct disorder
- RALD: NRAS , KRAS. Somatic mutations in NRAS and KRAS in the lymphocyte compartment. No longer considered a subtype of ALPS but a distinct disease.
- Revised classification of ALPS
References
- ↑ Sneller MC, Dale JK, Straus SE (2003). "Autoimmune lymphoproliferative syndrome". Curr Opin Rheumatol. 15 (4): 417–21. PMID 12819469.
- ↑ Oliveira JB, Bleesing JJ, Dianzani U, Fleisher TA, Jaffe ES, Lenardo MJ; et al. (2010). "Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop". Blood. 116 (14): e35–40. doi:10.1182/blood-2010-04-280347. PMC 2953894. PMID 20538792.
- ↑ Kuehn HS, Caminha I, Niemela JE, Rao VK, Davis J, Fleisher TA; et al. (2011). "FAS haploinsufficiency is a common disease mechanism in the human autoimmune lymphoproliferative syndrome". J Immunol. 186 (10): 6035–43. doi:10.4049/jimmunol.1100021. PMID 21490157.
- ↑ Holzelova E, Vonarbourg C, Stolzenberg MC, Arkwright PD, Selz F, Prieur AM; et al. (2004). "Autoimmune lymphoproliferative syndrome with somatic Fas mutations". N Engl J Med. 351 (14): 1409–18. doi:10.1056/NEJMoa040036. PMID 15459302.