Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect many organs, including the bone marrow. Aplastic anemia (AA) is a rare but serious complication of this condition. Patients with AA secondary to SLE are usually treated with immunosuppression but less is known about hematopoietic stem cell transplantation (HSCT) as a therapeutic option. We present the case of a 22-year-old Latin-American woman with a known diagnosis of SLE referred to our center due to pancytopenia. She presented joint pain, fatigue, oral ulcers, a non-scarring alopecia, and anemic syndrome. A diagnostic workup revealed severe aplastic anemia (sAA). She received immunosuppressive therapy with corticosteroids, cyclosporine, danazol, and rituximab, which led to a transient partial response. An outpatient HLA-matched related allogeneic HSCT was performed. She received 5.9 x 106 CD34+ cells/kg after conditioning with high-dose cyclophosphamide plus fludarabine and anti-thymocyte globulin. She achieved a complete response and continues in remission with no signs of graft-versus-host disease (GVHD) or SLE activity at 38 months of follow-up. Even though allogeneic hematopoietic stem cell transplantation is not defined as a first-line treatment for severe AA in SLE, the procedure resulted in the complete remission of both related autoimmune diseases in this patient.

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