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| {{Infobox_Disease |
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
| Name = {{PAGENAME}} |
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| DiseasesDB = 29724 |
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| ICD9 = {{ICD9|287.32}}|
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| {{Evans syndrome}} | | {{Evans syndrome}} |
| {{CMG}} | | {{CMG}}; {{AE}} {{Ammu}} {{F.Z}} |
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| ==Overview== | | {{SK}} Low grade fibromyxoid sarcoma; Evan's syndrome; Evans' syndrome; AHA/Immune TP; Coombs positive hemolytic anemia; IGG autoimmune hemolytic anemia |
| | ==[[Evans syndrome overview|Overview]]== |
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| ==Those Affected== | | ==[[Evans syndrome historical perspective|Historical Perspective]]== |
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| ==Signs and Symptoms== | | ==[[Evans syndrome classification|Classification]]== |
| The signs and symptoms of Evan’s Syndrome will be a combination of the signs and symptoms of the two underlying conditions.
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| In idiopathic thrombocytopenic purpura the following may be found:
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| 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.
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| Laboratory results will show low levels of platelets
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| In autoimmune hemolytic anemia the following may be found:
| | ==[[Evans syndrome pathophysiology|Pathophysiology]]== |
| Fatigue, Pale skin color, Shortness of breath, Rapid heartbeat,Jaundice colored skin, yellow/jaundice colored eyes, and/or Dark urine
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| ==Possible Causes== | | ==[[Evans syndrome causes|Causes]]== |
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| ==Diagnosis== | | ==[[Differentiating Evans syndrome from other diseases|Differentiating Evans syndrome from other Diseases]]== |
| 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.
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| | ==[[Evans syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
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| | ==[[Evans syndrome risk factors|Risk Factors]]== |
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| ==Treatment== | | ==[[Evans syndrome screening|Screening]]== |
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| ==Prognosis== | | ==[[Evans syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| Given the fact that the signs and symptoms of this disease are related directly to the abnormal laboratory findings, the prognosis depends on the patient’s response to treatment. Spontaneous remissions of each of the individual component conditions have been reported. If the child responds well to the treatment and the levels of platelets and red blood cells increase, the child can expect to live a normal life. Medications will be needed life long, and laboratory tests will need to be constantly monitored to detect any abnormal changes so that treatment can be adjusted.
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| In more serious cases it can massively reduce life span and is seriously life threatening.
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| Evan's Syndrome is rare, serious, and has a reported mortality rate of just under 18%.
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| ==The Future== | | ==Diagnosis== |
| In a patient with refractory disease, an allogeneic hematopoietic stem cell transplant (HSCT) resulted in complete clinical and serologic remission for more than 30 months. Allogeneic HSCT may be the only current curative therapy for Evans syndrome, but may also be complicated by significant toxicities. In more serious cases no cure exists.
| | [[Evans syndrome history and symptoms|History and Symptoms]] | [[Evans syndrome physical examination|Physical Examination]] | [[Evans syndrome laboratory findings|Laboratory Findings]] | [[Evans syndrome other imaging findings|Other Imaging Findings]] | [[Evans syndrome other diagnostic studies|Other Diagnostic Studies]] |
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| ==External links== | | ==Treatment== |
| *http://www.evanssyndrome.net
| | [[Evans syndrome medical therapy|Medical Therapy]] | [[Evans syndrome surgery|Surgery]] | [[Evans syndrome primary prevention|Primary Prevention]] | [[Evans syndrome secondary prevention|Secondary Prevention]] | [[Evans syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Evans syndrome future or investigational therapies|Future or Investigational Therapies]] |
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| ==References== | | ==Case Studies== |
| *http://www.madisonsfoundation.org/content/3/1/display.asp?did=81
| | [[Evans syndrome case study one|Case #1]] |
| *http://www.rarediseases.org
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| *http://autoimmune.pathology.jhmi.edu/diseases.cfm?systemid=5
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| * {{cite journal |author=Oyama Y, Papadopoulos EB, Miranda M, Traynor AE, Burt RK |title=Allogeneic stem cell transplantation for Evans syndrome |journal=Bone Marrow Transplant. |volume=28 |issue=9 |pages=903-5 |year=2001 |pmid=11781654 |doi=10.1038/sj.bmt.1703237}}
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| * {{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.A. archives of internal medicine |volume=87 |issue=1 |pages=48-65 |year=1951 |pmid=14782741 |doi=}}
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| * {{cite journal |author=Mathew P, Chen G, Wang W |title=Evans syndrome: results of a national survey |journal=J. Pediatr. Hematol. Oncol. |volume=19 |issue=5 |pages=433-7 |year=1997 |month=September/October |pmid=9329465 |doi=}}
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| * {{cite journal |author=Shanafelt TD, Madueme HL, Wolf RC, Tefferi A |title=Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome |journal=Mayo Clin. Proc. |volume=78 |issue=11 |pages=1340-6 |year=2003 |pmid=14601692 |doi=}}
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| *{{cite journal |author=Shanafelt TD, Madueme HL, Wolf RC, Tefferi A |title=Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome |journal=Mayo Clin. Proc. |volume=78 |issue=11 |pages=1340-6 |year=2003 |pmid=14601692 |doi= |url=http://www.mayoclinicproceedings.com/pdf/7811/7811a3.pdf |format=pdf}}
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| {{Hematology}} | | {{Hematology}} |