Evans syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
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. Patients with Evans syndrome usually appear normal. Physical examination of patients with Evans syndrome is usually remarkable for jaundice, hepatosplenomegaly, and lymphadenopathy. Laboratory findings consistent with the diagnosis of Evans syndrome include anemia, direct coombs test positive, and antineutrophil antibody positive. Pharmacologic medical therapies for Evans syndrome include prednisone, intravenous immunoglobulin, and rituximab. Surgery is not the firstline treatment option for patients with Evans syndrome. Splenectomy is usually reserved for patients who are unresponsive to treatment.
Historical Perspective
Evans syndrome was first described by Dr. Robert S. Evans, an American physician, in 1951.
Pathophysiology
The exact pathogenesis of Evans syndrome is not fully understood. It is thought that Evans syndrome is an autoimmune disorder in which autoantibodies are produced against red blood cells and platelets mainly, but also antibodies can be found against neutrophils and lymphocytes.It is also called "immune pancytopenia".[1] On gross pathology, circumscribed mass with microscopic infiltration is a characteristic finding of Evans syndrome. On microscopic histopathological analysis, alternating fibrous and myxoid stroma of low-grade/low malignant potential, and small tumor cells with scanty eosinophilic cytoplasm with a round to oval nuclei and no nucleoli are characteristic findings of Evans syndrome.
Causes
The cause of Evans syndrome has not been identified.
Differential Evans Syndrome from Other Diseases
Evans syndrome must be differentiated from acquired thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, Kasabach-Merritt syndrome, fibromatosis, fibrosarcoma, myxofibrosarcoma, nodular fasciitis and myxoid neurofibroma.
Epidemiology and Demographics
The incidence of Evan’s Syndrome is not precisely known. Evan’s Syndrome affects male and female equally. Evan’s Syndrome is a rare disease that tends to affect children.
Risk Factors
Natural history, Complications and Prognosis
If left untreated, patients with Evans syndrome have periods of exacerbation. Common complications of Evans syndrome include thrombocytopenia and autoimmune hemolytic anemia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.
Diagnosis
History and Symptoms
Symptoms of Evans syndrome include breathlessness, fatigue, jaundice, and dark urine.
Physical Examination
Patients with Evans syndrome usually appear normal. Physical examination of patients with Evans syndrome is usually remarkable for jaundice, hepatosplenomegaly, and lymphadenopathy.
Laboratory Findings
Laboratory findings consistent with the diagnosis of Evans syndrome include anemia, direct coombs test positive, and antineutrophil antibody positive.
Treatment
Medical Therapy
Pharmacologic medical therapies for Evans syndrome include prednisone, intravenous immunoglobulin, and rituximab.
Surgery
Surgery is not the firstline treatment option for patients with Evans syndrome. Splenectomy is usually reserved for patients who are unresponsive to treatment.
References
- ↑ Pui CH, Wilimas J, Wang W (1980). "Evans syndrome in childhood". J Pediatr. 97 (5): 754–8. doi:10.1016/s0022-3476(80)80258-7. PMID 7191890.