Autoimmune hemolytic anemia (AIHA) is a rare, very heterogeneous, and sometimes life-threatening acquired hematologic disease characterized by increased red blood cell (RBC) destruction by autoantibodies (autoAbs), either with or without complement involvement. Recent studies have shown that the involvement of T- and B-cell dysregulation and an imbalance of T-helper 2 (Th2) and Th17 phenotypes play major roles in the pathogenesis of AIHA. AIHA can be primary (idiopathic) but is more often secondary, triggered by infections or drug use or as a part of other diseases. As the location of origin of autoAbs and the location of autoAb-mediated RBC clearance, as well as the location of extramedullary hematopoiesis, the spleen is crucially involved in all the steps of AIHA pathobiology. Splenectomy, which was the established second-line therapeutic option in corticosteroid-resistant AIHA patients for decades, has become less common due to increasing knowledge of immunopathogenesis and the introduction of targeted therapy. This article provides a comprehensive overview of current knowledge regarding the place of the spleen in the immunological background of AIHA and the rapidly growing spectrum of novel therapeutic approaches. Furthermore, this review emphasizes the still-existing expediency of laparoscopic splenectomy with appropriate perioperative thromboprophylaxis and the prevention of infection as a safe and reliable therapeutic option in the context of the limited availability of rituximab and other novel therapies.
CD47 is a transmembrane protein that possesses the ability to inhibit macrophage phagocytosis, enabling immune system evasion. While CD47 overexpression is associated with a poor prognosis in solid malignancies, data on its prognostic significance in lymphomas are inconsistent. This study aimed to evaluate the clinical and prognostic significance of CD47 expression in patients with large B-cell lymphoma (LBCL). In this study biopsy specimens from 146 patients diagnosed with LBCL and treated with immunochemotherapy were analyzed. CD47 expression was assessed using standard immunohistochemical methods. A high level of CD47 expression was detected in 30 (20.5%) patients. High CD47 expression was more frequently observed in patients with high β2-microglobulin levels and extranodal disease compared to nodal LBCL. No significant difference in CD47 expression was observed between gastrointestinal LBCL and other extranodal localizations. CD47 positivity had no significant influence on progression-free survival (PFS) and overall survival (OS); however, a trend toward shorter PFS and OS was noted (p = 0.099 and p = 0.149, respectively). The median PFS and OS were 27 and 37 months, respectively, in patients with high CD47 expression, while the median PFS and OS were not reached in the group of CD47 negative patients. Although CD47 expression was not an independent predictor of survival, the magnitude and direction of the observed hazard ratios suggest a potentially clinically meaningful effect.