Laboratory parameters in relation to anticomplement treatment and splenectomy. ↓: Two units of packed red cells transfused. Eculizumab (Soliris, Alexion Pharmaceuticals) was started in September 2005 (gray arrow), according to the standard schedule (600 mg weekly ×4, then 900 mg fortnightly). Splenectomy was performed in May 2006 (black arrow), supported by vaccinations and antibiotics. No infections or other clinical complications have been recorded to date. The need for transfusion (17 units in the previous 12 months) remained stable during the TRIUMPH study (placebo arm), and was significantly reduced after introduction of eculizumab, which resolved intravascular hemolysis with LDH normalization; however, transfusion independence with hemoglobin stabilization was achieved only after splenectomy. Bilirubinemia was persistently elevated (almost entirely unconjugated) with some fluctuations, in part due to associated Gilbert syndrome (genetically confirmed by the presence of homozygous (TA)7 polymorphism of the UGT1A1 gene).