Autoimmune lymphoproliferative syndrome epidemiology and demographics
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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.