Evans syndrome overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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.

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. 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 eiosinophilic cytoplasm wiht round to oval nuclei and no nucleoli are characteristic findings of Evans syndrome.

Causes

The cause of Evans syndrome has not been identified.