Autoimmune lymphoproliferative syndrome natural history, complications and prognosis: Difference between revisions
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===Prognosis=== | ===Prognosis=== | ||
*Prognosis is generally | *[[Prognosis]] is generally good, and the [[survival rate]] of [[patients]] with [[Autoimmune lymphoproliferative syndrome]](ALPS) is approximately 85% by age 50.<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> | ||
*[[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]].<ref name="JacksonFischer1999">{{cite journal|last1=Jackson|first1=Christine E.|last2=Fischer|first2=Roxanne E.|last3=Hsu|first3=Amy P.|last4=Anderson|first4=Stacie M.|last5=Choi|first5=Youngnim|last6=Wang|first6=Jin|last7=Dale|first7=Janet K.|last8=Fleisher|first8=Thomas A.|last9=Middelton|first9=Lindsay A.|last10=Sneller|first10=Michael C.|last11=Lenardo|first11=Michael J.|last12=Straus|first12=Stephen E.|last13=Puck|first13=Jennifer M.|title=Autoimmune Lymphoproliferative Syndrome with Defective Fas: Genotype Influences Penetrance|journal=The American Journal of Human Genetics|volume=64|issue=4|year=1999|pages=1002–1014|issn=00029297|doi=10.1086/302333}}</ref> | ||
==References== | ==References== | ||
Revision as of 05:36, 26 June 2021
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
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.