Evans syndrome overview

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Overview
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Epidemiology and Demographics
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Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Physical Examination
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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". 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.

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.