Language

English

Publication Date

8-1-2024

Journal

Pediatric Blood & Cancer

DOI

10.1002/pbc.31061

PMID

38840429

PMCID

PMC11648260

PubMedCentral® Posted Date

12-16-2024

PubMedCentral® Full Text Version

Author MSS

Abstract

Chronic hemolytic anemia and vascular occlusion are hallmarks of sickle cell disease (SCD). Blood transfusions are critical for supportive and preventive management of SCD complications. Patients with SCD are at risk for hyperhemolysis syndrome (HHS), a subtype of delayed hemolytic transfusion reactions. HHS management includes intravenous immunoglobulin, corticosteroids, and avoidance of further transfusions. Not all patients respond to first-line agents. Eculizumab, which blocks terminal complement activation, has been proposed as second-line management of HHS. We describe two patients who received eculizumab for refractory HHS. In our experience, eculizumab is a safe and effective option for refractory pediatric HHS.

Keywords

Humans, Antibodies, Monoclonal, Humanized, Anemia, Sickle Cell, Male, Female, Child, Hemolysis, Adolescent, Child, Preschool, Transfusion Reaction, delayed hemolytic transfusion reactions, eculizumab, hyperhemolysis syndrome, sickle cell disease

Published Open-Access

yes

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