Evans syndrome

This code represents Evans syndrome, a rare autoimmune disorder characterized by the simultaneous or sequential development of at least two cytopenias, most commonly autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). It results from the immune system mistakenly attacking and destroying healthy blood cells.

Use this code when documentation explicitly states a diagnosis of Evans syndrome. This typically involves a patient presenting with both AIHA and ITP, or other combinations of autoimmune cytopenias, confirmed through laboratory findings and clinical assessment. Supporting documentation should clearly identify the presence of multiple autoimmune cytopenias.

• Ensure documentation specifies "Evans syndrome" rather than just multiple cytopenias, as not all concurrent cytopenias constitute Evans syndrome.
• Code any associated autoimmune hemolytic anemia (D59.1) and immune thrombocytopenia (D69.3) as secondary diagnoses, if not inherently captured by the Evans syndrome diagnosis.
• Review for any underlying conditions that may be contributing to the Evans syndrome, such as systemic lupus erythematosus, and code those accordingly.