Pure Red Cell Aplasia Associated with Systemic Lupus Erythematosus in Children Response to Rituximab– A Case Report and Literature Review

Abstract

Purpose: This study analyzes the clinical presentation of SLE-associated PRCA in a pediatric patient at our institution and evaluates rituximab's therapeutic efficacy. Methods: To summarize the clinical characteristics of SLE related PRCA in a child diagnosed in our hospital by literature review, and discuss the efficacy of rituximab in treating this disease. Results: A 12-year-old girl from Guangdong, China, was admitted to our hospital with the chief complaint of "3 years of SLE history and anemia persisting for 16 days." She received her initial SLE diagnosis at 8 years and 5 months of age. Intravenous cyclophosphamide pulse and methylprednisolone (MP) pulse therapy were administered, followed by maintenance treatment with mycophenolate mofetil (MMF). During her follow-up, she maintained an SLE Disease Activity Index (SLEDAI) score of "0." At 12 years of age, the patient developed progressive anemia, A bone marrow biopsy confirmed the diagnosis of pure red cell aplastic anemia. The anemia did not respond to immunoglobulin, corticosteroids, or cyclosporine A. Subsequently, rituximab was administered, leading to a gradual normalization of her hemoglobin and reticulocyte count. She was subsequently treated with MMF for maintenance, with an SLEDAI score of "0" during the follow-up period. Conclusion: Corticosteroids have traditionally been the mainstay of treatment for SLE-related PRCA, and the combination of cyclosporine and corticosteroids has shown promise in improving remission rates and reducing recurrence rates. The administration of rituximab, followed by sequential MMF use in the presented case, demonstrated significant efficacy.