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

Abstract

Patient: Female, 39-year-old Final Diagnosis: Hemolytic transfusion reaction Symptoms: Hemolysis • severe anemia Medication: — Clinical Procedure: — Specialty: Hematology 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 β-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 µg/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.

Complement biology for hematologists

“…Anecdotal cases reports treated with Eculizumab as a salvage therapy after an ABO incompatible transfusion 147,148 …”

“…145 DHTR is more frequent in SCD patients. 146 " sC5b-9 in plasma in some patients with DHTR treated with eculizumab 134,135 Anecdotal cases reports treated with Eculizumab as a salvage therapy after an ABO incompatible transfusion 147,148 to 30-50% of aHUS patients do not have markers of systemic complement activation. 53,54 3.3 | Hematopoietic stem cell transplantation associated thrombotic microangiopathy: A multisystem endothelial injury…”

Epidemiological and clinical features, therapeutic strategies and outcomes in patients with hyperhaemolysis: A systematic review

“…Hyperhaemolysis syndrome (HHS) has been defined as destruction of both transfused red blood cells (RBCs) and the patient's endogenous RBCs, resulting in a haemoglobin value below the pretransfusion baseline, evidence of haemolysis and reticulocytopenia and/or an inappropriately low reticulocyte response 4,5 . While HHS is usually observed in patients with sickle cell disease (SCD), it has also been described in a myriad of other conditions, including lymphoma, 6 thalassaemia 7 and human immunodeficiency virus infection 8 . HHS may be mediated by a previously undetected and/or new RBC alloantibody, though no alloantibodies are identified in some cases 4 …”