Evans syndrome

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Evans' Syndrome is an autoimmune disease in which an individual's antibodies attack their own RBCs as well as their platelets. Its overall pathology is therefore effectively a combination of the two autoimmune induced conditions: autoimmune hemolytic anemia and immune thrombocytopenic purpura.

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. Immune thrombocytopenic purpura is a condition in which the platelets in the blood 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.

Those Affected

The incidence of Evan’s Syndrome is not precisely known. The syndrome is reported to be a complication affecting 4-10% of those persons with a particular type of thrombocytopenia known as idiopathic thrombocytopenic purpura. The syndrome is more prevalent in children than in adults. simple symptoms of carriers are breathlessness, feeling faint, shooting pains and a faster heart rate.

evans syndrome is extremely serious and can eventually lead to death

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

The cause of the signs and symptoms of Evan’s Syndrome are directly related to the low levels of red blood cells (RBC) and platelets in the blood. These low levels are a result of circulating antibodies that bind to the blood cells and destroy them. Antibodies are made under normal conditions against foreign substances in the body and are therefore very useful in warding off infection. In conditions that are referred to as “autoimmune” the body makes antibodies against itself. In the case of Evan’s Syndrome, it is not currently known what triggers this reaction to happen.

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

The treatment for Evan’s syndrome will involve close monitoring of hemoglobin and platelet counts. To increase the number of platelets and RBCs in the blood, the patient may be given a corticosteroid such as prednisone. Prednisone will reduce the destruction of the platelets and the RBCs. A certain level of prednisone may need to be taken for the patient's entire life to prevent the levels of platelets and RBCs from dropping. Further treatment may be necessary with immune system suppressing drugs to block the production of the antibodies. To specifically treat the anemia of the syndrome, it may be necessary to provide the child with new RBCs by a blood transfusion. It has not been shown to be effective to transfuse platelets in a similar fashion to treat the thrombocytopenia.

'Note'(((In more severe cases that are unresponsive to treatment, it may become necessary to remove the spleen. The spleen is the organ that is responsible for removing the platelets and RBCs from circulation in the blood once they have been marked with the antibodies. A person can live a normal life without their spleen but, but is at increased risk for certain types of bacterial infections and pneumonia though Current knowledge refutes the effiacy of splenectomy in Evans Syndrome: especially in Adults with a median success rate of 1 month, splenectomy is not really a cure.

A drug, Rituxan, has been giving some good results in acute and refractory Evans syndrome cases. Mayo 2003 study Search the internet for more current studies on this rare "1:1,000,000 Adults" syndrome. CBC and Direct Coombs test before treatment will give proper diagnosis.

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. In more serious cases it can massively reduce life span and is seriously life threatening. Evan's Syndrome is rare, serious, and has a reported mortality rate of just under 18%.

The Future

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.

External links

• http://www.evanssyndrome.net

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 (September/October 1997). "Evans syndrome: results of a national survey". J. Pediatr. Hematol. Oncol. 19 (5): 433-7. PMID 9329465.
• 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.

v • d • e Pathology: hematology, myeloid hematologic disease (primarily D50-D77, 280-289)
RBCs/ hemoglobinopathy	+ Polycythemia - Macrocytosis - Anemia Nutritional Iron deficiency anemia (Plummer-Vinson syndrome), Megaloblastic anemia (Pernicious anemia) Hemolytic Hereditary enzyme: G6PD Deficiency - Pyruvate kinase deficiency - Triosephosphate isomerase deficiency hemoglobin: Thalassemia - Sickle-cell disease/trait membrane: Hereditary spherocytosis - Hereditary elliptocytosis - Hereditary stomatocytosis Acquired Autoimmune (Warm, Cold), HUS, MAHA, PNH, PCH, Myelophthisic Aplastic Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia, Sideroblastic anemia Blood tests Normocytic - Microcytic - Macrocytic - Normochromic - Hypochromic Other Methemoglobinemia
Coagulation/platelets/ coagulopathy/ bleeding diathesis	+ Hypercoagulability primary: Antithrombin III deficiency - Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden - Hyperprothrombinemia acquired: DIC (Congenital afibrinogenemia, Purpura fulminans) - autoimmune (Antiphospholipid) Other Essential thrombocytosis - clotting factor: Hemophilia (A/VIII, B/IX, C/XI) • Von Willebrand disease • Hypoprothrombinemia/II - XIII platelet function: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Gray platelet syndrome - May Hegglin anomaly - Pelger-Huet anomaly Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP Thrombocytopenia (Heparin-induced thrombocytopenia)
Monocytes/ macrophages	+ Histiocytosis WHO-I (Langerhans cell histiocytosis) WHO-II/non-Langerhans-cell (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) WHO-III/malignant (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease) Other Chronic granulomatous disease -cytosis: Monocytosis - -penia: Monocytopenia
Granulocytes	+ -cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia) - -penia: Granulocytopenia/agranulocytosis (Neutropenia/Kostmann syndrome, Eosinopenia, Basopenia)
See also hematological malignancy and immune disorders

v • d • e WikiDoc Research Resources for Evans syndrome
Articles on Evans syndrome	Most recent articles on Evans syndrome • Most cited articles on Evans syndrome • Review articles on Evans syndrome • Articles on Evans syndrome in N Eng J Med, Lancet, BMJ
Media (Slides, Video, Images, MP3) on Evans syndrome	Powerpoint slides on Evans syndrome • Images of Evans syndrome • Photos of Evans syndrome • Podcasts & MP3s on Evans syndrome • Videos on Evans syndrome
Evidence Based Medicine Regarding Evans syndrome	Cochrane Collaboration on Evans syndrome • Bandolier on Evans syndrome • TRIP on Evans syndrome
Cost Effectiveness of Evans syndrome	Cost Effectiveness of Evans syndrome
Clinical Trials Involving Evans syndrome	Ongoing Trials on Evans syndrome at Clinical Trials.gov • Trial results on Evans syndrome • Clinical Trials on Evans syndrome at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Evans syndrome	US National Guidelines Clearinghouse on Evans syndrome • NICE Guidance on Evans syndrome • NHS PRODIGY Guidance • FDA on Evans syndrome • CDC on Evans syndrome
Textbook Information on Evans syndrome	Books and Textbook Information on Evans syndrome
Pharmacology Resources on Evans syndrome	Dosing of Evans syndrome • Drug interactions with Evans syndrome • Side effects of Evans syndrome • Allergic reactions to Evans syndrome • Overdose information on Evans syndrome • Carcinogenicity information on Evans syndrome • Evans syndrome in pregnancy • Pharmacokinetics of Evans syndrome •
Genetics, Pharmacogenomics, and Proteinomics of Evans syndrome	Genetics of Evans syndrome • Pharmacogenomics of Evans syndrome • Proteomics of Evans syndrome
Newstories on Evans syndrome	Evans syndrome in the news • Be alerted to news on Evans syndrome • News trends on Evans syndrome
Commentary on Evans syndrome	Blogs on Evans syndrome
Patient Resources on Evans syndrome	Patient resources on Evans syndrome • Discussion groups on Evans syndrome • Patient Handouts on Evans syndrome • Directions to Hospitals Treating Evans syndrome • Risk calculators and risk factors for Evans syndrome
Healthcare Provider Resources on Evans syndrome	Symptoms of Evans syndrome • Causes & Risk Factors for Evans syndrome • Diagnostic studies for Evans syndrome • Treatment of Evans syndrome
Continuing Medical Education (CME) Programs on Evans syndrome	CME Programs on Evans syndrome
International Resources on Evans syndrome	Evans syndrome en Espanol • Evans syndrome en Francais
Business Resources on Evans syndrome	Evans syndrome in the Marketplace • Patents on Evans syndrome
Informatics Resources on Evans syndrome	List of terms related to Evans syndrome

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .