Autoimmune lymphoproliferative syndrome natural history, complications and prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sharmi Biswas, M.B.B.S
Overview
The symptoms of Autoimmune lymphoproliferative syndrome usually develop in the first decade of life and start with chronic lymphadenopathy with or without splenomegaly and hepatomegaly or both.The median age of initiation of lymphadenopathy is 11.5 months. Some of the patients present with fatigue, pallor and jaundice which are due to autoimmune hemolytic anemia,symptoms of thrombocytopenia as spontaneous bruises,and mucocutaneous hemorrhages or neutropenia associated infections Common complication of Autoimmune lymphoproliferative syndrome include, lymphoma both Hodgkins and Non Hodgkins lymphoma. 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 chronic lymphadenopathy with or without splenomegaly and hepatomegaly or both. The median age of initiation of lymphadenopathy is 11.5 months. Some of the patients present with fatigue, pallor and jaundice which are due to autoimmune hemolytic anemia,symptoms of thrombocytopenia as spontaneous bruises,and mucocutaneous hemorrhages or neutropenia associated infections.[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.
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