We present a rare case of newly diagnosed evans syndrome associated with lung papillary adenocarcinoma in which the patient showed prompt restoration of blood cell count and long. Pdf the fisher evans syndrome fes, also known as evans syndrome es is an immune hemopathy characterized by bicytopenia or pancytopenia in the. Evan s syndrome was first described in 19512 by evan s and associates. Brain magnetic resonance imaging was suggestive for right middle cerebral artery ischemic stroke. Evans syndrome is the coexistence of simultaneous or sequential direct coombspositive autoimmune hemolytic anemia aiha with immunemediated thrombocytopenia. In five patients, an increase in lymph node andor spleen size was observed during the exacerbations of cytopenias. Because evans syndrome is a rare disease, little is known about the relationship between pregnancy and evans syndrome. Six patients had either persistent hepatosplenomegaly or generalised lymphadenopathy, or both. Discussion evans syndrome was first described in 1951 by robert evans. Evans syndrome is an autoimmune disease in which an individuals immune system attacks their own red blood cells and platelets, the syndrome can include immune neutropenia. Symptoms include fatigue, shortness of breath and rashes and discoloration of skin due to bleeding from the capillary blood vessels beneath the surface of the skin. The most frequent symptoms on presentation are weight loss, weakness and abdominal pain. Clinicians must have it in mind when evaluating a patient with a positive direct antiglobulin test, anemia and thrombocytopenia, since prognosis depends on its early recognition.
These include immune thrombocytopenia itp, autoimmune hemolytic anemia aiha, andor autoimmune neutropenia ain. All content on this website, including dictionary, thesaurus, literature, geography, and other reference data is for informational purposes only. In the initial description by evans et al in 1951, the anemia and thrombocytopenia varied with respect to time of onset, course, and duration. Patients aged less than 18 years at the initial presentation of autoimmune cytopenia have been prospectively included into. Here we report a case of pregnancy complicated with evans syndrome. In france, a collaborative pediatric network set up via the national rare disease plan now provides comprehensive clinical data in children with this disease. There is some evidence of genetic predisposition in cocker spaniels and miniature schnauzers. Combination cytopenias are also referred to as evans syndrome. Evans syndrome nord national organization for rare. The presence of evans syndrome without significant lymphoproliferation can be consistent with alps, especially. A tenyearold neutered female mixed breed dog was referred for pale mucous membrane and acute onset of right prosencephalic clinical signs. Is there a diet which improves the quality of life of people with evans syndrome.
Are you aware of a diet that can improve the quality of life of people with evans syndrome. Its overall phenotype resembles a combination of autoimmune hemolytic anemia and imm une thrombocytopenic pur pura. Is there a diet that is suggested to avoid when having evans syndrome. Imha can affect both dogs and cats, but typically middleaged dogs are more susceptible. Evans syndrome is a rare autoimmune disorder in which the body makes antibodies that destroy the red blood cells, platelets and white blood cells. Presumptive ischemic brain infarction in a dog with evans.
In this case, aiha developed during pregnancy and itp developed long after delivery. 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. Diagnosis of diffuse hepatic angiosarcoma can be challenging. We report an original case of diffuse liver angiosarcoma revealed by haematological abnormalities initially diagnosed as an evans. The spectrum of evans syndrome archives of disease in. Evans syndrome es is an uncommon autoimmune disease that was defined by robert evans in 1951 when he studied the relationship between autoimmune hemolytic anemia aiha and immune thrombocytopenic purpura itp. Fisher evans syndrome is an uncommon hematologic condition rarely diagnosed and not widely studied. Fever since 2 weeks yellowish discoloration of eyes 1 weeks shortness of breath since 5 days.
Affected people often experience thrombocytopenia too few platelets and coombs positive hemolytic anemia premature destruction of red blood cells. The etiology is unknown and immune dysregulation may be involved in the pathogenesis. The diagnosis was evans syndrome in coexistence with a possible vitamin b12 deficiency. Pdf evans syndrome es is a rare autoimmune disorder whose longterm followup characteristics are unknown. This leads to abnormally low levels of these blood cells in the body cytopenia.
Evans, syndrome, hemolytic anemia, thrombocytopenia, neutropenia. Evans syndrome is an uncommon condition defined by the combination either simultaneously or sequentially of immune thrombocytopenia itp and autoimmune haemolytic anaemia aiha with a positive direct antiglobulin test dat in the absence of known underlying aetiology. Indhuprakash reddy post graduate dept of general medicine. Evans syndro me is an autoimmune disease in which an individuals immune system attacks their own red blood cells and platelets, the syndr ome can include immune neutropenia. The immune system is a network of cells, tissues, and organs that work together to defend the body against germs foreign substances. Evans syndrome symptoms, diagnosis, treatments and causes. Evans syndrome es is a rare autoimmune disorder whose longterm outcome is not well known. A case of pregnancy complicated with evans syndrome with. A rare condition where circulating antibodies red blood cells and a low level of blood platelets causing low iron levels and reduced blood clotting ability. In the initial description by evans et al in 1951,the anemia and thrombocytopenia varied with respect to time of onset, course, and duration.
It is rare diaease, a diagnosis of exclusion, and requires a high index of suspicion. Evans syndrome es is a rare hematological disease commonly defined by the combination of simultaneous or sequential autoimmune hemolytic anemia aiha and immune thrombocytopenia itp sometimes associated with neutropenia in the absence of known underlying etiology. More detailed information about the symptoms, causes, and treatments of evans syndrome is available below. These immune cytopenias may occur simultaneously or sequentially. Important links where can i find evans syndrome information. Evans syndrome is a chronic haematological condition associated with simultaneous or sequential occurrence of coombs positive autoimmune haemolytic anaemia and autoimmune thrombocytopenia. 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. 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. Evans syndrome genetic and rare diseases information. Evans syndrome is an autoimmune disease in which the bodys own antibodies attack the red blood cells that carry oxygen and carbon dioxide. Based on cell blood count, serum biochemistry and serologic tests and flow cytometric detection of antiplatelets.
In dogs that have secondary imha due to disease, spayed females are more commonly affected. Patients are diagnosed with thrombocytopenia and coombs positive hemolytic anemia and have no other known underlying etiology. The immune system normally responds to foreign substances by producing specialized proteins. Abstract fisherevans syndrome is the manifestation of an immune hemolytic anemia that may be simultaneous or subsequent to an immune thrombocytopenia sometimes attached with immune neutropenia. Evans syndrome can be categorized into primary idiopathic or secondary associated with underlying disease 1.
Roughly 60% of dogs with imha will also experience itp evans syndrome. Hepatic angiosarcoma is a rare malignant vascular tumor, which accounts for up to 2% of all primary liver tumors. Detailed investigation led to a diagnosis of evans syndrome. Evans syndrome is a rare disorder in which the bodys immune system produces antibodies that mistakenly destroy red blood cells, platelets and sometimes certain white blood cell known as neutrophils. To better describe the characteristics and outcome of es in adults, a survey was initiated in 2005.
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