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* Although Evans syndrome seems to be a disorder of immune regulation, the exact pathophysiology is unknown. | * Although Evans syndrome seems to be a disorder of immune regulation, the exact pathophysiology is unknown. | ||
* Evans syndrome is an [[autoimmune disease]] in which an individual's [[antibodies]] attack their own [[red blood cell]]s and [[platelet]]s.<ref name="pmid14782741">{{cite journal |author=Evans RS, Takahashi K, Duane RT, Payne R, Liu C |title=Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology |journal=A.M.AARRAYrchives of internal medicine |volume=87 |issue=1 |pages=48–65 |year=1951 |pmid=14782741 |doi=}}</ref> Both of these events may occur simultaneously or one may follow on from the other.<ref name="pmid16398647">{{cite journal |author=Norton A, Roberts I |title=Management of Evans syndrome |journal=Br. J. Haematol. |volume=132 |issue=2 |pages=125–37 |year=2006 |pmid=16398647 |doi=10.1111/j.1365-2141.2005.05809.x}}</ref> | * Evans syndrome is an [[autoimmune disease]] in which an individual's [[antibodies]] attack their own [[red blood cell]]s and [[platelet]]s.<ref name="pmid14782741">{{cite journal |author=Evans RS, Takahashi K, Duane RT, Payne R, Liu C |title=Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology |journal=A.M.AARRAYrchives of internal medicine |volume=87 |issue=1 |pages=48–65 |year=1951 |pmid=14782741 |doi=}}</ref> Both of these events may occur simultaneously or one may follow on from the other.<ref name="pmid16398647">{{cite journal |author=Norton A, Roberts I |title=Management of Evans syndrome |journal=Br. J. Haematol. |volume=132 |issue=2 |pages=125–37 |year=2006 |pmid=16398647 |doi=10.1111/j.1365-2141.2005.05809.x}}</ref> | ||
* Pathophysiology of this disease involves decreased cluster of differentiation (CD)4+ T-helper cell counts, increased CD8+ T-suppressor cell counts, a decreased CD4/CD8 ratio, and reduced serum immunoglobulin G, M and A levels - indicating a complex immune dysregulation. | |||
* Its overall pathology resembles a combination of [[autoimmune hemolytic anemia]] and [[idiopathic thrombocytopenic purpura]].<ref name="pmid14782741"/> Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry [[oxygen]] and [[carbon dioxide]] are destroyed by an autoimmune process. Idiopathic thrombocytopenic purpura is a condition in which platelets are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of [[blood clot]]s in the body to prevent bleeding. | * Its overall pathology resembles a combination of [[autoimmune hemolytic anemia]] and [[idiopathic thrombocytopenic purpura]].<ref name="pmid14782741"/> Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry [[oxygen]] and [[carbon dioxide]] are destroyed by an autoimmune process. Idiopathic thrombocytopenic purpura is a condition in which platelets are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of [[blood clot]]s in the body to prevent bleeding. | ||
* It has been variously reported that between 10%<ref>{{GPnotebook|-919273445|Evan's syndrome}}</ref> and 23%<ref name="pmid2632179">{{cite journal |author=Cai JR, Yu QZ, Zhang FQ |title=[Autoimmune hemolytic anemia: clinical analysis of 100 cases] |language=Chinese |journal=Zhonghua Nei Ke Za Zhi |volume=28 |issue=11 |pages=670–3, 701–2 |year=1989 |pmid=2632179 |doi=}}</ref> of patients who have [[autoimmune hemolytic anemia]], will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.<ref name="pmid1451398">{{cite journal |author=Ng SC |title=Evans syndrome: a report on 12 patients |journal=Clinical and laboratory haematology |volume=14 |issue=3 |pages=189–93 |year=1992 |pmid=1451398 |doi=10.1111/j.1365-2257.1992.tb00364.x}}</ref> | * It has been variously reported that between 10%<ref>{{GPnotebook|-919273445|Evan's syndrome}}</ref> and 23%<ref name="pmid2632179">{{cite journal |author=Cai JR, Yu QZ, Zhang FQ |title=[Autoimmune hemolytic anemia: clinical analysis of 100 cases] |language=Chinese |journal=Zhonghua Nei Ke Za Zhi |volume=28 |issue=11 |pages=670–3, 701–2 |year=1989 |pmid=2632179 |doi=}}</ref> of patients who have [[autoimmune hemolytic anemia]], will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.<ref name="pmid1451398">{{cite journal |author=Ng SC |title=Evans syndrome: a report on 12 patients |journal=Clinical and laboratory haematology |volume=14 |issue=3 |pages=189–93 |year=1992 |pmid=1451398 |doi=10.1111/j.1365-2257.1992.tb00364.x}}</ref> | ||
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* [[Autoimmune hemolytic anemia]] | * [[Autoimmune hemolytic anemia]] | ||
* Idiopathic thrombocytopenia purpura | * Idiopathic thrombocytopenia purpura | ||
* Viral infection | |||
* Systemic lupus erythematosus | |||
* Hashimoto’s thyroiditis | |||
* Dermatomyositis | |||
* Chronic inflammatory demyelinating polyneuropathy | |||
* Autoimmune hepatitis | |||
==Gross Pathology== | ==Gross Pathology== | ||
Revision as of 17:52, 11 January 2016
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Overview
Pathogenesis
- Although Evans syndrome seems to be a disorder of immune regulation, the exact pathophysiology is unknown.
- Evans syndrome is an autoimmune disease in which an individual's antibodies attack their own red blood cells and platelets.[1] Both of these events may occur simultaneously or one may follow on from the other.[2]
- Pathophysiology of this disease involves decreased cluster of differentiation (CD)4+ T-helper cell counts, increased CD8+ T-suppressor cell counts, a decreased CD4/CD8 ratio, and reduced serum immunoglobulin G, M and A levels - indicating a complex immune dysregulation.
- Its overall pathology resembles a combination of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura.[1] Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry oxygen and carbon dioxide are destroyed by an autoimmune process. Idiopathic thrombocytopenic purpura is a condition in which platelets are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of blood clots in the body to prevent bleeding.
- It has been variously reported that between 10%[3] and 23%[4] of patients who have autoimmune hemolytic anemia, will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.[5]
- Depending on the pathophysiology, Evans syndrome is classified as primary and secondary.
Genetics
Associated Conditions
- Autoimmune hemolytic anemia
- Idiopathic thrombocytopenia purpura
- Viral infection
- Systemic lupus erythematosus
- Hashimoto’s thyroiditis
- Dermatomyositis
- Chronic inflammatory demyelinating polyneuropathy
- Autoimmune hepatitis
Gross Pathology
Microscopic Pathology
References
- ↑ 1.0 1.1 Evans RS, Takahashi K, Duane RT, Payne R, Liu C (1951). "Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology". A.M.AARRAYrchives of internal medicine. 87 (1): 48–65. PMID 14782741.
- ↑ Norton A, Roberts I (2006). "Management of Evans syndrome". Br. J. Haematol. 132 (2): 125–37. doi:10.1111/j.1365-2141.2005.05809.x. PMID 16398647.
- ↑ Template:GPnotebook
- ↑ Cai JR, Yu QZ, Zhang FQ (1989). "[Autoimmune hemolytic anemia: clinical analysis of 100 cases]". Zhonghua Nei Ke Za Zhi (in Chinese). 28 (11): 670–3, 701–2. PMID 2632179.
- ↑ Ng SC (1992). "Evans syndrome: a report on 12 patients". Clinical and laboratory haematology. 14 (3): 189–93. doi:10.1111/j.1365-2257.1992.tb00364.x. PMID 1451398.