Delayed Severe Hemolytic Transfusion Reaction During Pregnancy in a Woman with β-Thalassemia Intermediate: Successful Outcome After Eculizumab Administration

Objective: Rare co-existance of disease or pathology Background: Delayed hemolytic transfusion reactions (DHTR) are life-threatening complications mostly triggered by red blood cell (RBC) transfusions in patients with hemoglobinopathy. Case Report: We present a case of DHTR and hyperhemolysis syndrome in a 39-year-old pregnant woman with a history of beta-thalassemia intermediate in whom the hemoglobin (Hb) level fell to 27 g/L after transfusion of 2 units of crossmatch-compatible packed RBCs. No allo- or auto-antibody formation was detected. Administration of intravenous immunoglobulins and methylprednisolone followed by anti-CD20 rituximab was tried, but was unsuccessful. Infusions of eculizumab (900 mg twice at a 7-day interval) followed by another course of intravenous immunoglobulins (2 g/kg/day for 5 days) and combined with repeated erythropoietin injections (darbepoetin alpha 300 mu g/week) finally allowed biological and clinical improvement. Blood counts remained controlled until delivery. Despite signs of intrauterine growth retardation, she gave birth by cesarean section at 31 weeks of pregnancy to a 1.15-kg infant. Conclusions: Eculizumab seems to be of benefit in DHTR associated with hyperhemolysis and should be used early in the treatment of this pathology. Despite premature birth, our case report showed an acceptable outcome for the infant when eculizumab treatment was used during pregnancy.