Transplant-associated thrombotic microangiopathy (TA-TMA) is an increasingly recognized complication of hematopoietic stem cell transplant (HSCT) with high morbidity and mortality. The triad of endothelial cell activation, complement dysregulation, and microvascular hemolytic anemia has the potential to cause end organ dysfunction, multiple organ dysfunction syndrome and death, but clinical features mimic other disorders following HSCT, delaying diagnosis. Recent advances have implicated complement as a major contributor and the therapeutic potential of complement inhibition has been explored. Eculizumab has emerged as an effective therapy and narsoplimab (OMS721) has been granted priority review by the FDA. Large studies performed mostly in pediatric patients suggest that earlier recognition and treatment may lead to improved outcomes. Here we present a clinically focused summary of recently published literature and propose a diagnostic and treatment algorithm.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8338557PMC
http://dx.doi.org/10.1038/s41409-021-01283-0DOI Listing

Publication Analysis

Top Keywords

transplant-associated thrombotic
8
thrombotic microangiopathy
8
organ dysfunction
8
microangiopathy theoretical
4
theoretical considerations
4
considerations practical
4
practical approach
4
approach unrefined
4
unrefined diagnosis
4
diagnosis transplant-associated
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!