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Yang, C. Autoimmune Lymphoproliferative Syndrome. Encyclopedia. Available online: https://encyclopedia.pub/entry/4887 (accessed on 27 September 2026).
Yang C. Autoimmune Lymphoproliferative Syndrome. Encyclopedia. Available at: https://encyclopedia.pub/entry/4887. Accessed September 27, 2026.
Yang, Catherine. "Autoimmune Lymphoproliferative Syndrome" Encyclopedia, https://encyclopedia.pub/entry/4887 (accessed September 27, 2026).
Yang, C. (2020, December 24). Autoimmune Lymphoproliferative Syndrome. In Encyclopedia. https://encyclopedia.pub/entry/4887
Yang, Catherine. "Autoimmune Lymphoproliferative Syndrome." Encyclopedia. Web. 24 December, 2020.
Autoimmune Lymphoproliferative Syndrome
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This entry is adapted from the peer-reviewed paper https://medlineplus.gov/genetics/condition/autoimmune-lymphoproliferative-syndrome

Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder in which the body cannot properly regulate the number of immune system cells (lymphocytes). ALPS is characterized by the production of an abnormally large number of lymphocytes (lymphoproliferation). Accumulation of excess lymphocytes results in enlargement of the lymph nodes (lymphadenopathy), the liver (hepatomegaly), and the spleen (splenomegaly).

genetic conditions

References

  1. Bleesing JJH, Nagaraj CB, Zhang K. Autoimmune Lymphoproliferative Syndrome.2006 Sep 14 [updated 2017 Aug 24]. In: Adam MP, Ardinger HH, Pagon RA, WallaceSE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle(WA): University of Washington, Seattle; 1993-2020. Available fromhttp://www.ncbi.nlm.nih.gov/books/NBK1108/
  2. Dowdell KC, Niemela JE, Price S, Davis J, Hornung RL, Oliveira JB, Puck JM,Jaffe ES, Pittaluga S, Cohen JI, Fleisher TA, Rao VK. Somatic FAS mutations arecommon in patients with genetically undefined autoimmune lymphoproliferativesyndrome. Blood. 2010 Jun 24;115(25):5164-9. doi: 10.1182/blood-2010-01-263145.
  3. Fleisher TA. The autoimmune lymphoproliferative syndrome: an experiment ofnature involving lymphocyte apoptosis. Immunol Res. 2008;40(1):87-92. doi:10.1007/s12026-007-8001-1.
  4. Lenardo MJ, Oliveira JB, Zheng L, Rao VK. ALPS-ten lessons from aninternational workshop on a genetic disease of apoptosis. Immunity. 2010 Mar26;32(3):291-5. doi: 10.1016/j.immuni.2010.03.013.
  5. Madkaikar M, Mhatre S, Gupta M, Ghosh K. Advances in autoimmunelymphoproliferative syndromes. Eur J Haematol. 2011 Jul;87(1):1-9. doi:10.1111/j.1600-0609.2011.01617.x. Review.
  6. Neven B, Magerus-Chatinet A, Florkin B, Gobert D, Lambotte O, De Somer L,Lanzarotti N, Stolzenberg MC, Bader-Meunier B, Aladjidi N, Chantrain C, Bertrand Y, Jeziorski E, Leverger G, Michel G, Suarez F, Oksenhendler E, Hermine O,Blanche S, Picard C, Fischer A, Rieux-Laucat F. A survey of 90 patients withautoimmune lymphoproliferative syndrome related to TNFRSF6 mutation. Blood. 2011 Nov 3;118(18):4798-807. doi: 10.1182/blood-2011-04-347641.
  7. Oliveira JB, Bidère N, Niemela JE, Zheng L, Sakai K, Nix CP, Danner RL, BarbJ, Munson PJ, Puck JM, Dale J, Straus SE, Fleisher TA, Lenardo MJ. NRAS mutation causes a human autoimmune lymphoproliferative syndrome. Proc Natl Acad Sci U S A.2007 May 22;104(21):8953-8.
  8. Oliveira JB, Bleesing JJ, Dianzani U, Fleisher TA, Jaffe ES, Lenardo MJ,Rieux-Laucat F, Siegel RM, Su HC, Teachey DT, Rao VK. Revised diagnostic criteriaand classification for the autoimmune lymphoproliferative syndrome (ALPS): reportfrom the 2009 NIH International Workshop. Blood. 2010 Oct 7;116(14):e35-40. doi: 10.1182/blood-2010-04-280347.
  9. Price S, Shaw PA, Seitz A, Joshi G, Davis J, Niemela JE, Perkins K, HornungRL, Folio L, Rosenberg PS, Puck JM, Hsu AP, Lo B, Pittaluga S, Jaffe ES, FleisherTA, Rao VK, Lenardo MJ. Natural history of autoimmune lymphoproliferativesyndrome associated with FAS gene mutations. Blood. 2014 Mar 27;123(13):1989-99. doi: 10.1182/blood-2013-10-535393.
  10. Rao VK. Approaches to Managing Autoimmune Cytopenias in Novel ImmunologicalDisorders with Genetic Underpinnings Like Autoimmune LymphoproliferativeSyndrome. Front Pediatr. 2015 Jul 21;3:65. doi: 10.3389/fped.2015.00065.
  11. Takagi M, Shinoda K, Piao J, Mitsuiki N, Takagi M, Matsuda K, Muramatsu H,Doisaki S, Nagasawa M, Morio T, Kasahara Y, Koike K, Kojima S, Takao A, Mizutani S. Autoimmune lymphoproliferative syndrome-like disease with somatic KRASmutation. Blood. 2011 Mar 10;117(10):2887-90. doi: 10.1182/blood-2010-08-301515.
  12. Teachey DT, Seif AE, Grupp SA. Advances in the management and understanding ofautoimmune lymphoproliferative syndrome (ALPS). Br J Haematol. 2010Jan;148(2):205-16. doi: 10.1111/j.1365-2141.2009.07991.x.Review.
  13. Turbyville JC, Rao VK. The autoimmune lymphoproliferative syndrome: A raredisorder providing clues about normal tolerance. Autoimmun Rev. 2010May;9(7):488-93. doi: 10.1016/j.autrev.2010.02.007.
  14. Worth A, Thrasher AJ, Gaspar HB. Autoimmune lymphoproliferative syndrome:molecular basis of disease and clinical phenotype. Br J Haematol. 2006Apr;133(2):124-40. Review.
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Subjects: Genetics & Heredity
Contributor MDPI registered users' name will be linked to their SciProfiles pages. To register with us, please refer to https://encyclopedia.pub/register : Catherine Yang
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Update Date: 24 Dec 2020
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