Autoimmune lymphoproliferative syndrome epidemiology and demographics: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Autoimmune lymphoproliferative syndrome}} | {{Autoimmune lymphoproliferative syndrome}} | ||
{{CMG}} | {{CMG}} {{AE}} {{SharmiB}} | ||
==Overview== | ==Overview== | ||
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===Prevalence=== | ===Prevalence=== | ||
*The [[prevalence]] is not known as many cases are left unidentified. | *The [[prevalence]] is not known as many cases are left unidentified. A total of 500 [[patients]] with ALPS have been identified, from more than 300 families.<ref name="ShahWu2014">{{cite journal|last1=Shah|first1=Shaili|last2=Wu|first2=Eveline|last3=Rao|first3=V. Koneti|last4=Tarrant|first4=Teresa K.|title=Autoimmune Lymphoproliferative Syndrome: an Update and Review of the Literature|journal=Current Allergy and Asthma Reports|volume=14|issue=9|year=2014|issn=1529-7322|doi=10.1007/s11882-014-0462-4}}</ref> | ||
===Case-fatality rate/Mortality rate=== | ===Case-fatality rate/Mortality rate=== | ||
*[[ | * The [[mortality rate]] is 15% in [[patients]] with [[ALPS]]-FAS by [[age]] 50.<ref name="ShahWu2014">{{cite journal|last1=Shah|first1=Shaili|last2=Wu|first2=Eveline|last3=Rao|first3=V. Koneti|last4=Tarrant|first4=Teresa K.|title=Autoimmune Lymphoproliferative Syndrome: an Update and Review of the Literature|journal=Current Allergy and Asthma Reports|volume=14|issue=9|year=2014|issn=1529-7322|doi=10.1007/s11882-014-0462-4}}</ref> | ||
===Age=== | ===Age=== | ||
*[[Patients]] of all [[age]] groups may develop [[Autoimmune lymphoproliferative syndrome]].<ref name="PriceShaw2014">{{cite journal|last1=Price|first1=Susan|last2=Shaw|first2=Pamela A.|last3=Seitz|first3=Amy|last4=Joshi|first4=Gyan|last5=Davis|first5=Joie|last6=Niemela|first6=Julie E.|last7=Perkins|first7=Katie|last8=Hornung|first8=Ronald L.|last9=Folio|first9=Les|last10=Rosenberg|first10=Philip S.|last11=Puck|first11=Jennifer M.|last12=Hsu|first12=Amy P.|last13=Lo|first13=Bernice|last14=Pittaluga|first14=Stefania|last15=Jaffe|first15=Elaine S.|last16=Fleisher|first16=Thomas A.|last17=Rao|first17=V. Koneti|last18=Lenardo|first18=Michael J.|title=Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations|journal=Blood|volume=123|issue=13|year=2014|pages=1989–1999|issn=0006-4971|doi=10.1182/blood-2013-10-535393}}</ref> | *[[Patients]] of all [[age]] groups may develop [[Autoimmune lymphoproliferative syndrome]].<ref name="PriceShaw2014">{{cite journal|last1=Price|first1=Susan|last2=Shaw|first2=Pamela A.|last3=Seitz|first3=Amy|last4=Joshi|first4=Gyan|last5=Davis|first5=Joie|last6=Niemela|first6=Julie E.|last7=Perkins|first7=Katie|last8=Hornung|first8=Ronald L.|last9=Folio|first9=Les|last10=Rosenberg|first10=Philip S.|last11=Puck|first11=Jennifer M.|last12=Hsu|first12=Amy P.|last13=Lo|first13=Bernice|last14=Pittaluga|first14=Stefania|last15=Jaffe|first15=Elaine S.|last16=Fleisher|first16=Thomas A.|last17=Rao|first17=V. Koneti|last18=Lenardo|first18=Michael J.|title=Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations|journal=Blood|volume=123|issue=13|year=2014|pages=1989–1999|issn=0006-4971|doi=10.1182/blood-2013-10-535393}}</ref> | ||
*Typically [[ALPS]] | *Typically [[ALPS]] appears in early [[childhood]]. The [[median]] age at [[diagnosis]] is 3 years.<ref name="Rieux-LaucatMagérus-Chatinet2018">{{cite journal|last1=Rieux-Laucat|first1=Frédéric|last2=Magérus-Chatinet|first2=Aude|last3=Neven|first3=Bénédicte|title=The Autoimmune Lymphoproliferative Syndrome with Defective FAS or FAS-Ligand Functions|journal=Journal of Clinical Immunology|volume=38|issue=5|year=2018|pages=558–568|issn=0271-9142|doi=10.1007/s10875-018-0523-x}}</ref> | ||
===Race=== | ===Race=== | ||
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[[Category:Hematology]] | [[Category:Hematology]] | ||
[[Category:Up To Date]] | |||
Latest revision as of 19:34, 17 September 2021
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sharmi Biswas, M.B.B.S
Overview
Autoimmune lymphoproliferative syndrome (ALPS) is a rare disease that mostly affects children of early age. The incidence and prevalence of ALPS are unknown. Male predominance has been found.
Epidemiology and Demographics
Incidence
- The incidence of Autoimmune lymphoproliferative syndrome(ALPS) is unknown as many cases remain undiagnosed or misdiagnosed.[1]
Prevalence
- The prevalence is not known as many cases are left unidentified. A total of 500 patients with ALPS have been identified, from more than 300 families.[2]
Case-fatality rate/Mortality rate
- The mortality rate is 15% in patients with ALPS-FAS by age 50.[2]
Age
- Patients of all age groups may develop Autoimmune lymphoproliferative syndrome.[3]
- Typically ALPS appears in early childhood. The median age at diagnosis is 3 years.[4]
Race
- There is no racial predilection to Autoimmune Lymphoproliferative Syndrome.
Gender
- Males are more commonly affected by Autoimmune lymphoproliferative syndrome than females. The male to female ratio is 14:4.[3]
Region
- There is no information about any specific region where Autoimmune lymphoproliferative syndrome is prevalent.
References
- ↑ Rao, V. Koneti; Oliveira, João Bosco (2011). "How I treat autoimmune lymphoproliferative syndrome". Blood. 118 (22): 5741–5751. doi:10.1182/blood-2011-07-325217. ISSN 0006-4971.
- ↑ 2.0 2.1 Shah, Shaili; Wu, Eveline; Rao, V. Koneti; Tarrant, Teresa K. (2014). "Autoimmune Lymphoproliferative Syndrome: an Update and Review of the Literature". Current Allergy and Asthma Reports. 14 (9). doi:10.1007/s11882-014-0462-4. ISSN 1529-7322.
- ↑ 3.0 3.1 Price, Susan; Shaw, Pamela A.; Seitz, Amy; Joshi, Gyan; Davis, Joie; Niemela, Julie E.; Perkins, Katie; Hornung, Ronald L.; Folio, Les; Rosenberg, Philip S.; Puck, Jennifer M.; Hsu, Amy P.; Lo, Bernice; Pittaluga, Stefania; Jaffe, Elaine S.; Fleisher, Thomas A.; Rao, V. Koneti; Lenardo, Michael J. (2014). "Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations". Blood. 123 (13): 1989–1999. doi:10.1182/blood-2013-10-535393. ISSN 0006-4971.
- ↑ Rieux-Laucat, Frédéric; Magérus-Chatinet, Aude; Neven, Bénédicte (2018). "The Autoimmune Lymphoproliferative Syndrome with Defective FAS or FAS-Ligand Functions". Journal of Clinical Immunology. 38 (5): 558–568. doi:10.1007/s10875-018-0523-x. ISSN 0271-9142.