Autoimmune lymphoproliferative syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Prognosis is generally good, and the survival rate of patients with Autoimmune lymphoproliferative syndrome(ALPS) is approximately 85% by age 50.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of Autoimmune lymphoproliferative syndrome usually develop in the first decade of life and start with symptoms such as splenomegaly, lymphadenopathy and cytopenia.[1][2]
Complications
- Common complication of Autoimmune lymphoproliferative syndrome include:
- Lymphoma both Hodgkins and Non Hodgkins lymphoma.[2]
- Sepsis follwed by splenectomy
Prognosis
- Prognosis is generally good, and the survival rate of patients with Autoimmune lymphoproliferative syndrome(ALPS) is approximately 85% by age 50.[3]
- Cytopenia related to ALPS improve with age and respond to immunosuppressive treatment.
- Chronic lymphoproliferation regress with age and does not need any treatment.
- Patients with mutation in the extracellular domain of FAS usually cause milder disease.
- Mutations in the intracellular domain of FAS cause a worse prognosis.[4]
References
- ↑ Bride, Karen; Teachey, David (2017). "Autoimmune lymphoproliferative syndrome: more than a FAScinating disease". F1000Research. 6: 1928. doi:10.12688/f1000research.11545.1. ISSN 2046-1402.
- ↑ 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.
- ↑ 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.
- ↑ Jackson, Christine E.; Fischer, Roxanne E.; Hsu, Amy P.; Anderson, Stacie M.; Choi, Youngnim; Wang, Jin; Dale, Janet K.; Fleisher, Thomas A.; Middelton, Lindsay A.; Sneller, Michael C.; Lenardo, Michael J.; Straus, Stephen E.; Puck, Jennifer M. (1999). "Autoimmune Lymphoproliferative Syndrome with Defective Fas: Genotype Influences Penetrance". The American Journal of Human Genetics. 64 (4): 1002–1014. doi:10.1086/302333. ISSN 0002-9297.