Management of Hyperhemolysis in β-thalassemia With Multiple Immunosuppressives, Including Complement Blockade. Issue 8 (November 2021)

• Record Type: Journal Article
• Authors: Zanetti, Richard C.; Vasta, Lauren M.; Romanelli, Kristen; Newton, Thomas C.
• Is Part Of: Journal of pediatric hematology/oncology. Volume 43:Issue 8(2021)
• Publication Date: 2021-11
• Subjects: hyperhemolysis -- β-thalassemia -- eculizumab; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000002059
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : Hyperhemolysis is a life-threatening condition of exaggerated hemolysis of red blood cells which occurs in patients receiving chronic transfusion therapy. We present a 19-year-old male with the β-thalassemia major with an episode of hyperhemolysis. Hemolysis was initially unresponsive to immunosuppression but responded after the addition of eculizumab. Several weeks after stabilization, hemolysis returned; which was also managed with immunosuppression and eculizumab. Hyperhemolysis presents significant challenges in β-thalassemia due to the underlying dysfunctional erythropoiesis and transfusion dependence. Aggressive immunosuppression combined with eculizumab successfully slowed the hemolysis and allowed for the resumption of transfusions.