Autoimmune lymphoproliferative syndrome classification: Difference between revisions
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==Classification== | ==Classification== | ||
There is no established system for the staging of [[Autoimmune]] [[Lymphoproliferative]] [[Syndrome]]. | |||
Old nomenclature<ref name="pmid12819469">{{cite journal| author=Sneller MC, Dale JK, Straus SE| title=Autoimmune lymphoproliferative syndrome. | journal=Curr Opin Rheumatol | year= 2003 | volume= 15 | issue= 4 | pages= 417-21 | pmid=12819469 | doi= | pmc= | url= }} </ref> | Old nomenclature<ref name="pmid12819469">{{cite journal| author=Sneller MC, Dale JK, Straus SE| title=Autoimmune lymphoproliferative syndrome. | journal=Curr Opin Rheumatol | year= 2003 | volume= 15 | issue= 4 | pages= 417-21 | pmid=12819469 | doi= | pmc= | url= }} </ref> | ||
* IA - [[Fas]] | * IA - [[Fas]] | ||
| Line 58: | Line 21: | ||
* ALPS-CASP10: [[Caspase 10]]. Germline CASP10 mutation. 2% of patients | * ALPS-CASP10: [[Caspase 10]]. Germline CASP10 mutation. 2% of patients | ||
* ALPS-U: Undefined. 20% of patients | * ALPS-U: Undefined. 20% of patients | ||
* CEDS: Caspase 8 deficiency state. No longer considered a subtype of ALPS but distinct disorder | * 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 | * 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== | ==References== | ||
Revision as of 16:21, 24 June 2021
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; David Teachey, MD [2]
Overview
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.