Surgery is not the first­line treatment option for patients with Evans syndrome. Splenectomy is usually reserved for patients who are unresponsive to treatment.

In more severe cases that are unresponsive to treatment, it may become necessary to remove the spleen. The spleen is the organ that is responsible for removing the platelets and red blood cells from circulation in the blood once they have been marked with the antibodies. A person may live a normal life without their spleen, but is at increased risk for certain types of bacterial infections and pneumonia. Current knowledge refutes the efficacy of splenectomy in Evans Syndrome, especially in adults. The median success rate among adults is 1 month. Splenectomy is not a definitive cure.