Evans syndrome pathophysiology
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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]
- 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
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.