ABSTRACT
Introduction: Hematologic abnormalities affect many patients with systemic lupus erythematosus (SLE). Despite their clinical relevance, standardized treatment guidelines remain limited, and management strategies rely heavily on observational evidence. This systematic review synthesizes current evidence on treatment strategies for hematologic cytopenias in adults with SLE and identifies key gaps in the literature. Methods: A systematic review was conducted in accordance with PRISMA guidelines. Searches of Embase, MEDLINE, and the Cochrane Library (from database inception to March 31, 2025) identified 15,197 records. Following screening and eligibility assessment, 37 studies evaluating anemia, leukopenia, thrombocytopenia, or bi-/pancytopenia in SLE were included. Treatment strategies were analyzed by cytopenia subtype and categorized according to line of therapy. Results: Management strategies varied according to cytopenia subtype. Anemia was treated with corticosteroids for autoimmune hemolytic anemia and pure red cell aplasia, with rituximab or immunosuppressants for refractory cases. Iron deficiency was managed with oral or intravenous iron; anemia of chronic disease responded to lupus control and erythropoiesis-stimulating agents. Leukopenia was observed if asymptomatic, but corticosteroids, immunosuppressants, or granulocyte-colony stimulating factor were used when clinically indicated. Thrombocytopenia treatment was tiered by severity and bleeding risk, using corticosteroids, rituximab, thrombopoietin receptor agonists, or splenectomy. Bi-or pancytopenia required urgent intervention based on underlying etiology (e.g., thrombotic thrombocytopenic purpura, hemophagocytic lymphohistiocytosis, marrow failure), often involving corticosteroids combined with intravenous immunoglobulin, rituximab, or cytotoxic agents; plasma exchange was reserved for conditions such as thrombotic thrombocytopenic purpura. Conclusion: Cytopenias in SLE require subtype-specific management strategies tailored to the underlying mechanism and disease severity. This review summarizes current therapeutic approaches and highlights the need for prospective studies to establish standardized treatment strategies.