Autoimmune lymphoproliferative syndrome pathophysiology

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Autoimmune lymphoproliferative syndrome Microchapters

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Editor-In-Chief: David Teachey, MD [1] Sharmi Biswas, M.B.B.S

Overview

Autoimmune lymphoproliferative syndrome(ALPS) is the first disease in human beings which are caused by apoptosis defect. Defect in FAS signaling cause benign chronic lymphoproliferation followed by accumulation of TCRαβ(+) CD4(-) CD8(-) double-negative T (DNT) cells. FAS also prevent the mailgnant proliferation of lymphocytes , so ALPS patients are at higher risk of developing lymphoma.There is an identified genetic defect in 2/3 rd of the total cases of ALPS. Germline mutation is the most common type. Somatic mutation, mutations in the proteins of FAS ligand(FASL) and caspase are also responsible in some cases.The pathogenesis of ALPS is still not very clear and research is ongoing.

Pathophysiology

Associated Conditions

Microscopic Pathology

Histopathological study findings can be helpful to diagnose Autoimmune lymphoproliferative syndrome. The findings are

Histopathology of spleen in ALPS.[3]
Histopathology of lymph nodes in ALPS.[3]


References

  1. Teachey DT, Manno CS, Axsom KM, Andrews T, Choi JK, Greenbaum BH; et al. (2005). "Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS)". Blood. 105 (6): 2443–8. doi:10.1182/blood-2004-09-3542. PMID 15542578.
  2. Li, Pu; Huang, Ping; Yang, Ye; Hao, Mu; Peng, Hongwei; Li, Fei (2015). "Updated Understanding of Autoimmune Lymphoproliferative Syndrome (ALPS)". Clinical Reviews in Allergy & Immunology. 50 (1): 55–63. doi:10.1007/s12016-015-8466-y. ISSN 1080-0549.
  3. 3.0 3.1 Lim, Megan S.; Straus, Stephen E.; Dale, Janet K.; Fleisher, Thomas A.; Stetler-Stevenson, Maryalice; Strober, Warren; Sneller, Michael C.; Puck, Jennifer M.; Lenardo, Michael J.; Elenitoba-Johnson, Kojo S.J.; Lin, Albert Y.; Raffeld, Mark; Jaffe, Elaine S. (1998). "Pathological Findings in Human Autoimmune Lymphoproliferative Syndrome". The American Journal of Pathology. 153 (5): 1541–1550. doi:10.1016/S0002-9440(10)65742-2. ISSN 0002-9440.