Oliwia Kubiś, Katarzyna Wojakowicz, Zuzanna Rzepkowska, Kacper Siemek, Alicja Dąbrowska, Magdalena Szmyrka
Pancytopenia is a significant hematological complication in patients with systemic lupus erythematosus (SLE), with an estimated prevalence of 10-40%. It is defined as a simultaneous decrease in red blood cells, white blood cells, and platelets. Although isolated cytopenias are more common, pancytopenia may indicate severe disease activity or secondary complications, such as bone marrow suppression or hemophagocytic syndromes. This review aims to summarize and synthesize current knowledge on the etiology, pathophysiology, diagnostic approach, and treatment strategies for pancytopenia in SLE, as well as its differentiation from other causes of bone marrow failure. The pathogenesis of pancytopenia in SLE is multifactorial and includes drug-induced bone marrow suppression, hypersplenism, myelofibrosis, macrophage activation syndrome (MAS), and autoimmune bone marrow failure. The diagnostic evaluation includes hematologic assessment, bone marrow examination, and exclusion of alternative diagnoses, such as aplastic anemia and paroxysmal nocturnal hemoglobinuria. Therapeutic management depends on the underlying cause. We also summarize published case reports comparing treatment approaches and clinical outcomes. The role of rituximab (RTX) in the management of pancytopenia and isolated cytopenias associated with SLE is discussed in detail. Particular attention is given to its mechanisms of action, safety profile and mixed clinical outcomes, documented in multicenter retrospective cohort studies, meta-analyses, and case series. Early recognition of pancytopenia remains a clinical challenge and requires well-structured diagnostic and therapeutic strategies. A thorough understanding of its underlying mechanisms and clinical manifestations is essential to avoid delays in treatment and prevent complications.