Abatacept improves posttransplant survival and reduces endothelial injury syndromes in β-thalassemia major.

ABSTRACT:

Abstract

Iron overload in transfusion-dependent β-thalassemia (TDT) generates reactive oxygen species, predisposing to post-hematopoietic stem cell transplant (HSCT) endothelial activation. Abatacept prevents acute graft-versus-host disease (GVHD) by inhibiting CD80/CD86 on T cells, but CD80 is also expressed on neutrophils. Elevated neutrophil extracellular traps (NETs) at day +14 are associated with thrombotic microangiopathy (TMA) after HSCT, mechanistically linking endothelial activation to complement activation. We wanted to compare post-HSCT survival and incidence of endothelial injury syndromes in children with TDT with and without addition of abatacept to standard GVHD prophylaxis. We performed a retrospective review of children with TDT who underwent HSCT at our center. Pa

SUBMITTER: Khandelwal P 

PROVIDER: S-EPMC12744282 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature