Abstract

Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by a relapsing-remitting course and marked variability in clinical presentation and disease course. Hematologic involvement in SLE presents with a broad spectrum, including anemia, leukopenia, and thrombocytopenia, reflecting varying degrees of involvement of all three hematopoietic cell lines. These manifestations may occur at any stage of the disease and, in some cases, may represent the initial pattern of organ involvement. In this review, evidence regarding the treatment of SLE-associated autoimmune hemolytic anemia, immune thrombocytopenia, leukopenia, thrombotic microangiopathies (TMA), and hemophagocytic syndrome (HS) was evaluated. The available evidence is largely derived from observational studies, case reports, and expert opinion, with no randomized controlled trials specifically addressing hematological manifestations identified. Although treatment strategies vary according to the type and severity of hematological involvement, glucocorticoids constitute the cornerstone of therapy in most clinical scenarios. In cases of refractory or relapsing autoimmune hemolytic anemia and immune thrombocytopenia, the use of immunosuppressive agents and intravenous immunoglobulins has been reported; however, evidence supporting their early steroid-sparing use remains limited. Conversely, the combination of high-dose corticosteroids and immunosuppressive agents in the acute management of HS, as well as plasma exchange therapy in TMA, has been reported to improve clinical outcomes. Nevertheless, evidence across all hematological manifestations remains limited, underscoring the ongoing need for prospective, controlled studies tailored to specific hematological patterns to establish comprehensive treatment recommendations.