Evans syndrome foundation nord national organization. Ivig is common early on too, especially if platelets are low. We describe the case of a 21yearold male with longlasting and refractory evans syndrome. Successful treatment of evans syndrome with tacrolimus. While imha involves the destruction of red blood cells, in itp there is increased destruction of platelets by the bodys own immune system, at a rate faster than. Evans syndrome is an autoimmune disease in which the bodys own antibodies attack the red blood cells that carry oxygen and carbon dioxide. It means that the patients antibodies attack their own blood products like the red blood cells and platelet.
His blood counts improved, and the facial swelling and rash subsided. In france, a collaborative pediatric network set up via the national rare disease plan now provides comprehensive clinical data in children with this disease. Evans syndrome es is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia aiha and immune thrombocytopenia itp andor immune neutropenia. Evans syndrome is an autoimmune disorder in which a persons own antibodies attack red blood cells rbc, which deliver oxygen to body tissues, platelets which help blood clot, andor neutrophils a type of white blood cell that helps fight infections. The type of aiha that presents in evans syndrome is warm aiha, in which. In the initial description by evans et al in 1951, the anemia and thrombocytopenia varied with respect to time of onset, course, and duration. However, many dogs with evans syndrome can achieve remission with prompt recognition. About 20% of dogs with imtp itp will also have imha. Evans syndrome es is defined by the combination either simultaneous or sequential of immune thrombocytopenia itp and autoimmune haemolytic anaemia aiha. To better describe the characteristics and outcome of es in adults, a survey was initiated in 2005. These two conditions together can be life threatening. Plasmapheresis was stopped but administration of steroids continued. Efficacy of rituximab treatment mixed type evans syndrome is a very rare hematologic disease.
The association of immune thrombocytopenic purpura itp and autoimmune hemolytic anemia aiha, both mediated by autoantibodies and responding to splenectomy, was first described in 1951 in 24 patients aged 378 years by robert evans. In the initial description by evans et al in 1951, the anemia and thrombocytopenia varied with respect to. Evans syndrome is an autoimmune disorder in which noncrossreacting autoantibodies are. There are no established guidelines regarding which tests should be performed in. It is thought that evans syndrome is an autoimmune disorder in which autoantibodies are produced against red blood cells and platelets. Immunemediated thrombocytopenia itp, also known as evans syndrome and by the acronym imp, occurs due to primary idiopathic or secondary e. Evans syndrome treatment danafarberboston childrens. It is the combination of coombspositive idiopathic autoimmune hemolytic anemia iaha and immune thrombocytopenic purpura itp. Evans syndrome when both immune medeiated thrombocytopenia imtp and autoimmune haemolytic anaemia aiha occur concurrently, this is known as evans syndrome. The thymus contains regulatory t cell treg, which plays a pivotal role in maintaining immunological selftolerance.
Evans syndrome es is an autoimmune disorder defined by the simultaneous or sequential development of itp and autoimmune hemolytic anemia aiha with a positive dat andor immune neutropenia in the absence of known underlying etiology evans et al. Other clinical signs, such as vomiting, diarrhea, or respiratory distress, are treated as needed. We describe here a 46yrold female with the steroid. Although almost all patients require therapy at some time during the course of the disease, the.
Although mixed type evans syndrome may initially respond well to steroids, this disease usually runs a chronic course with intermittent exacerbations. Medications like prednisone have been used for years in various autoimmune disorders. Guidelines on the use of intravenous immune globulin for hematologic conditions. Management of evans syndrome university of wisconsin. The life expectancy of evans syndrome depend on each case. Various blood tests, and in some cases a bone marrow aspiration, may be needed to. The immune system is a network of cells, tissues, and organs that work together to defend the body against germs foreign substances. As in other autoimmune cytopenias, there is no established evidencebased treatment and steroids are the firstline therapy, with intravenous. Natural cure for evans syndrome and alternative treatments.
However, both the medical and surgical treatment of refractory evans syndrome have been rather unsuccessful and allogeneic stem cell transplantation has been used for truly refractory disease 3. The remissions of the disease are common, as well as relapses. Rituximab for refractory evans syndrome and other immune. Pdf successful treatment of evans syndrome post liver.
Allogeneic stem cell transplantation for evans syndrome. Blood and platelets transfusion is the treatment given to alleviate symptoms and gain time but its use should be minimized. Pdf evans syndrome is a rare autoimmune disorder in which the body makes antibodies. Affected people often experience thrombocytopenia too few platelets and coombs positive hemolytic anemia premature destruction of red blood cells. However, not all patients maintain a complete remission and often require secondary treatment. Epidemiology evans syndrome is uncommon but not rare. Evans syndrome is a rare syndrome associated with the simultaneous or sequential development of autoimmune hemolytic anemia aiha and idiopathic thrombocytopenic purpura itp.
Secondline treatment for refractory es includes rituximab, mofetil. The treatment protocol for evans syndrome is the same as imtp or aiha, but often the survival rate is lower. Evans syndrome autoimmune hemolytic anemia combined with thrombocytopenia is a rare hematological syndrome in the elderly. The best treatments for evans can be different for every patient. This means that a diagnosis is made in people with coombspositive hemolytic anemia and thrombocytopenia related to an abnormal immune response once other conditions with similar signs and symptoms have been ruled out. Management of evans syndrome uw hematology protocols. Furthermore, acute kidney injury aki is a syndrome characterized by the rapid loss of kidney excretory function and is most often secondary to extrarenal events. Sexual differences in incidence no sexual predilection is known in evans syndrome. Evans syndrome revisited priti dave, kavita krishna, ag diwan. Responses to other agents have been anecdotal and inconclusive.
The dog has not one, but two, separate life threatening conditions to overcome. He was ultimately diagnosed with evans syndrome as a presenting feature of systemic lupus erythematosus. The evans syndrome foundation has been created as an effort to educate evans syndrome patients, their care givers and the general public. First line treatment is usually steroids and they can help many patients. Natural history, pathogenesis, and treatment of evans. The spectrum of evans syndrome archives of disease in.
Pdf successful treatment of evans syndrome with tacrolimus. What is the life expectancy of someone with evans syndrome. Evans syndrome es is a very rare autoimmune disease first described in 1951. Evans syndrome is the coexistence of simultaneous or sequential direct coombspositive autoimmune hemolytic anemia aiha with immunemediated thrombocytopenia. Some cases of evans syndrome es were associated, mainly in adults, with various medical conditions such as systemic. Successful treatment of evans syndrome post liver transplant with splenectomy and switch from tacrolimus to cyclosporine. In a retrospective study of 17 children with evans syndrome, rituximab therapy in combination with prednisone 14 patients. Evans syndrome es is a rare autoimmune disorder whose longterm outcome is not well known. Also, the treatment options differ according to the classification. Agerelated demographics evans syndrome occurs in individuals of all ages. Drugs used to treat evans syndrome the following list of medications are in some way related to, or used in the treatment of this condition.
Evans syndrome is a combination of imtp also itp and imha. Eleven patients 10 boys, one girl with evans syndrome with a median follow up time of 8. Treatment for evans syndrome depends on many factors, including the severity of the condition. Canine immunemediated hemolytic anemia 3924 fernandina road columbia, sc 29210 p. A 94yearold patient was diagnosed with evans syndrome with the secretion. The severity of symptoms and the delay between episodes of aiha andor itp is variable. Although almost all patients require therapy at some time during the course of the disease, the search for a consistent, effective, and nontoxic therapy continues. The syndrome is characterized by a chronic, relapsing, and potentially fatal course requiring longterm immunosuppressive therapy.
Treatment is the same as for aiha imha but the survival rate can be lower. Evans syndrome definition of evans syndrome by medical. Management of evans syndrome request pdf researchgate. Primary evans syndrome es is defined by the concurrent or sequential occurrence of immune thrombocytopenia and autoimmune hemolytic anemia in the absence of an underlying etiology. The immune system normally responds to foreign substances by producing specialized proteins, called antibodies, that. Evans syndrome genetic and rare diseases information. The exact pathogenesis of evans syndrome is not fully understood. Unfortunately, if you have evans syndrome, you may require steroids for long periods of time which may result in other issues like high blood pressure hypertension and elevated blood sugars diabetes. Amber yates, md, is a boardcertified pediatric hematologist and a practicing physician at baylor college of medicine. They are the first line treatment for aiha and are also used in itp. Symptoms include fatigue, shortness of breath and rashes and discoloration of skin due to bleeding from the. All drug classes mitotic inhibitors 1 immune globulins 1 antirheumatics 1 glucocorticoids 6 cd20 monoclonal antibodies 1 antigonadotropic agents 2 calcineurin inhibitors 1. Evans syndrome is a combination of two or more immune hematological disorders where your immune system attacks your white blood cells, red blood cells andor platelets. Six patients had either persistent hepatosplenomegaly or generalised lymphadenopathy, or both.
Evans syndrome is a rare phenomenon that has been reported with thymomas. Evans syndrome secondary to chronic lymphocytic leukaemia. Evans syndrome is a disorder that refers to an autoimmune disease. How i manage evans syndrome and wiley online library. It is commonly cause by the two autoimmune disorders, the autoimmune hemolytic anemia a condition in which the red blood cells are destroyed by an autoimmune process.
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