Evans syndrome
| Evans syndrome | |
| ICD-9 | 287.32 |
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| DiseasesDB | 29724 |
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Evans syndrome Microchapters |
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Evans syndrome On the Web |
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American Roentgen Ray Society Images of Evans syndrome |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Those Affected
Signs and Symptoms
The signs and symptoms of Evan’s Syndrome will be a combination of the signs and symptoms of the two underlying conditions. In idiopathic thrombocytopenic purpura the following may be found: Bleeding of skin or mucus lined areas of the body. This may show up as bleeding in the mouth, or purpuric rashes (look almost like bruises), or tiny red dots on the skin called petechiae. Laboratory results will show low levels of platelets
In autoimmune hemolytic anemia the following may be found: Fatigue, Pale skin color, Shortness of breath, Rapid heartbeat,Jaundice colored skin, yellow/jaundice colored eyes, and/or Dark urine
Possible Causes
Diagnosis
The diagnosis of Evan’s Syndrome is based primarily on laboratory findings, as well as the corresponding physical signs and symptoms. A complete blood count (CBC) will confirm the presence of anemia and low platelets. Additional studies may include a peripheral smear and a Coombs test. A peripheral smear of the blood may reveal evidence of red blood cell destruction or reticulocytosis. Reticulocytes are immature red blood cells and are usually abundant in Evan’s syndrome where there is a need to replace ongoing losses. There are also distinct shapes to certain cells that may be found when a sample of the patient’s blood is viewed under a microscope. In patients with Evan’s syndrome the red blood cells may appear small and globular shaped (then called spherocytes) but will not be fragmented. A Coombs test is used to detect the presence of antibodies against the RBC and is usually positive in immune mediated haemolysis.
Treatment
Prognosis
The Future
External links
References
- http://www.madisonsfoundation.org/content/3/1/display.asp?did=81
- http://www.rarediseases.org
- http://autoimmune.pathology.jhmi.edu/diseases.cfm?systemid=5
- Oyama Y, Papadopoulos EB, Miranda M, Traynor AE, Burt RK (2001). "Allogeneic stem cell transplantation for Evans syndrome". Bone Marrow Transplant. 28 (9): 903–5. doi:10.1038/sj.bmt.1703237. PMID 11781654.
- 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.A. archives of internal medicine. 87 (1): 48–65. PMID 14782741.
- Mathew P, Chen G, Wang W (1997). "Evans syndrome: results of a national survey". J. Pediatr. Hematol. Oncol. 19 (5): 433–7. PMID 9329465. Unknown parameter
|month=ignored (help) - Shanafelt TD, Madueme HL, Wolf RC, Tefferi A (2003). "Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome". Mayo Clin. Proc. 78 (11): 1340–6. PMID 14601692.
- Shanafelt TD, Madueme HL, Wolf RC, Tefferi A (2003). "Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome" (pdf). Mayo Clin. Proc. 78 (11): 1340–6. PMID 14601692.