Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Autoimmune lymphoproliferative syndrome epidemiology and demographics
Introduction
Overview
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
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
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.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sharmi Biswas, M.B.B.S