Autoimmune lymphoproliferative syndrome pathophysiology: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
Normally, after infectious insult, the immune system down-regulates by increasing Fas expression on activated B and T [[lymphocytes]] and [[Fas-ligand]] on activated T lymphocytes. Fas and Fas-ligand interact to trigger the caspase cascade, leading to cell [[apoptosis]]. Patients with [[ALPS]] have a defect in this apoptotic pathway, leading to chronic non-malignant lymphoproliferation, [[autoimmune disease]], and secondary cancers.<ref name="pmid19930184">{{cite journal| author=Teachey DT, Seif AE, Grupp SA| title=Advances in the management and understanding of autoimmune lymphoproliferative syndrome (ALPS). | journal=Br J Haematol | year= 2010 | volume= 148 | issue= 2 | pages= 205-16 | pmid=19930184 | doi=10.1111/j.1365-2141.2009.07991.x | pmc=PMC2929682 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19930184 }} </ref> | Normally, after infectious insult, the immune system down-regulates by increasing Fas expression on activated B and T [[lymphocytes]] and [[Fas-ligand]] on activated T lymphocytes. Fas and Fas-ligand interact to trigger the caspase cascade, leading to cell [[apoptosis]]. Patients with [[ALPS]] have a defect in this apoptotic pathway, leading to chronic non-malignant lymphoproliferation, [[autoimmune disease]], and secondary cancers.<ref name="pmid19930184">{{cite journal| author=Teachey DT, Seif AE, Grupp SA| title=Advances in the management and understanding of autoimmune lymphoproliferative syndrome (ALPS). | journal=Br J Haematol | year= 2010 | volume= 148 | issue= 2 | pages= 205-16 | pmid=19930184 | doi=10.1111/j.1365-2141.2009.07991.x | pmc=PMC2929682 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19930184 }} </ref> | ||
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[[File:ALPS.png|center|thumb|306x306px|Activation induced cell pathway by Fast apoptosis signal (FAS) receptor.<ref name="MatsonYang2019">{{cite journal|last1=Matson|first1=Daniel R.|last2=Yang|first2=David T.|title=Autoimmune Lymphoproliferative Syndrome: An Overview|journal=Archives of Pathology & Laboratory Medicine|volume=144|issue=2|year=2019|pages=245–251|issn=0003-9985|doi=10.5858/arpa.2018-0190-RS}}</ref>]] | |||
===Associated Conditions=== | ===Associated Conditions=== | ||
* Autoimmune cytopenias: Most common. Can be mild to very severe. Can be intermittent or chronic.<ref name="pmid15542578">{{cite journal| author=Teachey DT, Manno CS, Axsom KM, Andrews T, Choi JK, Greenbaum BH et al.| title=Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). | journal=Blood | year= 2005 | volume= 105 | issue= 6 | pages= 2443-8 | pmid=15542578 | doi=10.1182/blood-2004-09-3542 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15542578 }} </ref> | |||
** Autoimmune [[Hemolytic Anemia]] | *Autoimmune cytopenias: Most common. Can be mild to very severe. Can be intermittent or chronic.<ref name="pmid15542578">{{cite journal| author=Teachey DT, Manno CS, Axsom KM, Andrews T, Choi JK, Greenbaum BH et al.| title=Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). | journal=Blood | year= 2005 | volume= 105 | issue= 6 | pages= 2443-8 | pmid=15542578 | doi=10.1182/blood-2004-09-3542 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15542578 }} </ref> | ||
** Autoimmune [[Neutropenia]] | **Autoimmune [[Hemolytic Anemia]] | ||
** Autoimmune [[Thrombocytopenia]] | **Autoimmune [[Neutropenia]] | ||
* Other: Can affect any organ system similar to [[systemic lupus erythematosis]] (most rare affecting <5% of patients) | **Autoimmune [[Thrombocytopenia]] | ||
** Nervous: Autoimmune cerebellar [[ataxia]]; [[Guillain-Barre]]; [[Transverse myelitis]] | *Other: Can affect any organ system similar to [[systemic lupus erythematosis]] (most rare affecting <5% of patients) | ||
** GI: Autoimmune [[esophagitis]], [[gastritis]], [[colitis]], [[hepatitis]], [[pancreatitis]] | **Nervous: Autoimmune cerebellar [[ataxia]]; [[Guillain-Barre]]; [[Transverse myelitis]] | ||
** Derm: [[Urticaria]] | **GI: Autoimmune [[esophagitis]], [[gastritis]], [[colitis]], [[hepatitis]], [[pancreatitis]] | ||
** Pulmonary: [[Bronchiolitis obliterans]] | **Derm: [[Urticaria]] | ||
** Renal: Autoimmune [[glomerulonephritis]], [[nephrotic syndrome]] | **Pulmonary: [[Bronchiolitis obliterans]] | ||
* Cancer: Secondary neoplasms affect approximately 10% of patients. True prevalence unknown as <20 reported cases of [[cancer]]. Most common EBER+ Non-Hodgkin's and [[Hodgkin's]] lymphoma | **Renal: Autoimmune [[glomerulonephritis]], [[nephrotic syndrome]] | ||
*Cancer: Secondary neoplasms affect approximately 10% of patients. True prevalence unknown as <20 reported cases of [[cancer]]. Most common EBER+ Non-Hodgkin's and [[Hodgkin's]] lymphoma | |||
==References== | ==References== | ||
Revision as of 02:35, 29 June 2021
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Editor-In-Chief: David Teachey, MD [1]
Overview
Pathophysiology
Normally, after infectious insult, the immune system down-regulates by increasing Fas expression on activated B and T lymphocytes and Fas-ligand on activated T lymphocytes. Fas and Fas-ligand interact to trigger the caspase cascade, leading to cell apoptosis. Patients with ALPS have a defect in this apoptotic pathway, leading to chronic non-malignant lymphoproliferation, autoimmune disease, and secondary cancers.[1]
Associated Conditions
- Autoimmune cytopenias: Most common. Can be mild to very severe. Can be intermittent or chronic.[3]
- Autoimmune Hemolytic Anemia
- Autoimmune Neutropenia
- Autoimmune Thrombocytopenia
- Other: Can affect any organ system similar to systemic lupus erythematosis (most rare affecting <5% of patients)
- Nervous: Autoimmune cerebellar ataxia; Guillain-Barre; Transverse myelitis
- GI: Autoimmune esophagitis, gastritis, colitis, hepatitis, pancreatitis
- Derm: Urticaria
- Pulmonary: Bronchiolitis obliterans
- Renal: Autoimmune glomerulonephritis, nephrotic syndrome
- Cancer: Secondary neoplasms affect approximately 10% of patients. True prevalence unknown as <20 reported cases of cancer. Most common EBER+ Non-Hodgkin's and Hodgkin's lymphoma
References
- ↑ Teachey DT, Seif AE, Grupp SA (2010). "Advances in the management and understanding of autoimmune lymphoproliferative syndrome (ALPS)". Br J Haematol. 148 (2): 205–16. doi:10.1111/j.1365-2141.2009.07991.x. PMC 2929682. PMID 19930184.
- ↑ Matson, Daniel R.; Yang, David T. (2019). "Autoimmune Lymphoproliferative Syndrome: An Overview". Archives of Pathology & Laboratory Medicine. 144 (2): 245–251. doi:10.5858/arpa.2018-0190-RS. ISSN 0003-9985.
- ↑ 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.