Diagnostic and Therapeutic Strategies in Evans Syndrome: A Case Report and Literature Review

Abstract

Evans syndrome (ES) is characterized by a combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Immune dysregulation, which results in the development of antibodies against blood cells, is its defining feature. ES being a diagnosis of exclusion requires a thorough workup to rule out other probable illnesses like lymphoproliferative diseases and systemic lupus erythematosus (SLE). We present the case of a 38-year-old male who experienced shortness of breath, chest discomfort, and generalized weakness. His medical history included recurrent anemia, thrombocytopenia, and pulmonary tuberculosis in remission. Hemolysis, thrombocytopenia, and a large pericardial effusion were discovered during the physical examination and investigations. An initial treatment strategy that included pericardiocentesis was performed. In combination with AIHA and ITP, the clinical and laboratory findings strongly suggested ES, which improved with prednisolone therapy. First-line treatments consist of corticosteroids and intravenous immunoglobulin; refractory cases may also require rituximab, thrombopoietin receptor antagonists, and sirolimus. Achieving remission and lowering relapse rates need careful patient monitoring and customized treatment programs.

Keywords: acute pericardial effusion; autoimmune hemolytic anemia (aiha); coombs positive hemolysis; direct anti-globulin test; evans’ syndrome; immune thrombocytopenia (itp); immune thrombocytopenia purpura; immune-mediated hemolysis; intravenous immunoglobulins (ivig); prednisolone acetate.

Figure 1. ECG findings suggestive of sinus tachycardia and low voltage in limb leads

Figure 2. Two-dimensional echocardiogram showing features of pericardial effusion: (A) parasternal short axis view at the level of the left ventricle; (B) parasternal long axis view; and (C) parasternal long axis view motion mode at the level of the left ventricle