Successful splenectomy for refractory systemic lupus erythematosus-associated autoimmune hemolytic anemia: a case report and mechanistic insight.

Systemic lupus erythematosus (SLE) frequently involves the hematologic system, and autoimmune hemolytic anemia (AIHA) is a severe and potentially life-threatening complication. Splenectomy has been regarded as a third-line treatment option for refractory AIHA; however, its role in SLE-associated AIHA, particularly in patients with atypical immunologic phenotypes, remains to be further clarified. Here, we report a patient with refractory SLE-associated AIHA who failed multiple lines of therapy and subsequently underwent splenectomy, after which the hemoglobin level gradually increased and sustained hematologic improvement was achieved during follow-up. Splenic pathology suggested that the spleen was not only a major site of erythrocyte destruction but also a key pathologic organ in sustaining abnormal immune responses and ongoing hemolysis. In addition, positive IFI44L methylation provided supplementary supportive evidence for an SLE-related immune background. This case suggests that splenectomy may still represent a therapeutic option worthy of careful consideration in patients with refractory SLE-associated AIHA who fail multiple lines of therapy.