Background: Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) with a positive direct anti-human globulin test. Evan?s syndrome is a rare disorder because it is diagnosed in only 0.8% to 3.7% of all patients with either ITP or AIHA at onset. The clinical presentation can include fatigue, pallor, jaundice and mucosal bleeding, with remissions and exacerbations during the person?s lifetime, and acute manifestations as catastrophic bleeding and massive hemolysis. Case Description: A 20-year-old female was referred from Mardi Waluyo Hospital with complaints of weakness since 1 week before admission. She also complained of pallor, headache and gum bleeding. Physical examination: temperature 36.2? C, heart rate 90 x/minute, respiratory rate 18 x/minute and blood pressure 120/70 mmHg, without organ enlargement. Laboratory result: Hemoglobin 5.6 g/dL, WBC 5.03 x 103/?L, Platelets 62.0 x 103/?L,C3 59.5, C4 16.8, positive direct and indirect Coomb?s test, direct bilirubin 0.46 mg/dL, total bilirubin 3.61 mg/dL. Blood Smear: normochromic anisopoikilocytosis anemia and thrombocytopenia. BONE MARROW ASPIRATION: Increase in erythropoietic activity, increase in thrombopoietic activity. Conclusion: Based on the physical examination, laboratory results especially positive direct Coomb?s test and increase in marrow?s erythropoietic and thrombopoietic activity, the cytopenia in this patient was due to peripheral destruction factor, possibly an autoimmune disease. This patient fulfilled the diagnosis of Evans Syndrome, although it is needed to be confirmed by antiplatelet antibody.

Evans Syndrome

AIHA

ITP