Brian E Nolan1, Yunfei Wang2, Philippe P Pary3, Naomi L C Luban1,2,3,4,5, Edward C C Wong1,2,3,4,5, Tova Ronis1,2,6. 1. Department of Pediatrics, Washington, DC. 2. Center for Translational Science, Washington, DC. 3. Division of Laboratory Medicine, Center for Cancer and Blood Disorders, George Washington School of Medicine and Health Sciences, Children's National Health System, Washington, DC. 4. Division of Hematology, Center for Cancer and Blood Disorders, George Washington School of Medicine and Health Sciences, Children's National Health System, Washington, DC. 5. Department of Pathology, Center for Cancer and Blood Disorders, George Washington School of Medicine and Health Sciences, Children's National Health System, Washington, DC. 6. Division of Rheumatology, Center for Cancer and Blood Disorders, George Washington School of Medicine and Health Sciences, Children's National Health System, Washington, DC.
Abstract
BACKGROUND: Hemolysis is a reported side effect of intravenous immunoglobulin (IVIG) therapy in adults, but pediatric data are scarce. We determined the frequency of IVIG-associated hemolysis in patients with Kawasaki disease (KD) and characterized risk factors for hemolysis. We hypothesized that hemolysis is more common in children with KD than adults with other disorders, and hemolysis risk is related to IVIG dose and degree of inflammation. STUDY DESIGN AND METHODS: This was an 8-year, single-center, retrospective cohort study. A total of 419 KD patients were identified; 123 had pre- and post-treatment complete blood counts allowing for assessment of anemia. Hemolytic anemia was defined as decrease in hemoglobin after IVIG greater than 1 g/dL with immunohematologic or biochemical studies supporting hemolysis. RESULTS: 123 patients were stratified as having hemolysis (n = 18, 15%) or nonhemolysis (n = 105, 85%). Patients with hemolysis were more likely to have complete versus incomplete KD (65% vs. 39%, p = 0.04) and refractory versus nonrefractory course (78% vs. 16%, p < 0.001). Patients receiving 4 g/kg versus 2 g/kg IVIG were more likely to hemolyze (89% vs. 34%, p < 0.001). Patients with hemolysis had mostly non-O blood group (94%), positive direct antiglobulin tests (89%), and positive eluates (72%). Two-thirds of patients with hemolysis required RBC transfusion. CONCLUSIONS: Hemolysis occurred in 15% of KD patients evaluated for anemia and is strongly associated with high-dose (4 g/kg) IVIG. KD patients receiving high-dose IVIG should have close hematologic monitoring to identify hemolysis.
BACKGROUND:Hemolysis is a reported side effect of intravenous immunoglobulin (IVIG) therapy in adults, but pediatric data are scarce. We determined the frequency of IVIG-associated hemolysis in patients with Kawasaki disease (KD) and characterized risk factors for hemolysis. We hypothesized that hemolysis is more common in children with KD than adults with other disorders, and hemolysis risk is related to IVIG dose and degree of inflammation. STUDY DESIGN AND METHODS: This was an 8-year, single-center, retrospective cohort study. A total of 419 KDpatients were identified; 123 had pre- and post-treatment complete blood counts allowing for assessment of anemia. Hemolytic anemia was defined as decrease in hemoglobin after IVIG greater than 1 g/dL with immunohematologic or biochemical studies supporting hemolysis. RESULTS: 123 patients were stratified as having hemolysis (n = 18, 15%) or nonhemolysis (n = 105, 85%). Patients with hemolysis were more likely to have complete versus incomplete KD (65% vs. 39%, p = 0.04) and refractory versus nonrefractory course (78% vs. 16%, p < 0.001). Patients receiving 4 g/kg versus 2 g/kg IVIG were more likely to hemolyze (89% vs. 34%, p < 0.001). Patients with hemolysis had mostly non-O blood group (94%), positive direct antiglobulin tests (89%), and positive eluates (72%). Two-thirds of patients with hemolysis required RBC transfusion. CONCLUSIONS:Hemolysis occurred in 15% of KDpatients evaluated for anemia and is strongly associated with high-dose (4 g/kg) IVIG. KDpatients receiving high-dose IVIG should have close hematologic monitoring to identify hemolysis.
Authors: Christine W Bruggeman; Sietse Q Nagelkerke; Wendy Lau; Cedric Manlhiot; Masja de Haas; Robin van Bruggen; Brian W McCrindle; Rae S M Yeung; Taco W Kuijpers Journal: Blood Adv Date: 2020-07-28