A new paradigm: Diagnosis and management of HSCT-associated thrombotic microangiopathy as multi-system endothelial injury. Issue 3 (May 2015)

• Record Type: Journal Article
• Authors: Jodele, Sonata; Laskin, Benjamin L.; Dandoy, Christopher E.; Myers, Kasiani C.; El-Bietar, Javier; Davies, Stella M.; Goebel, Jens; Dixon, Bradley P.
• Is Part Of: Blood reviews. Volume 29:Issue 3(2015:May)
• Page Start: 191
• Page End: 204
• Publication Date: 2015-05
• Subjects: Thrombotic microangiopathy -- TA-TMA -- Kidney disease -- Complement activation -- Hematopoietic cell transplant -- Eculizumab; Hematology -- Periodicals; Hematology -- Periodicals; Hématologie -- Périodiques; Hematology; Electronic journals; Periodicals; 616.15
• DOI: 10.1016/j.blre.2014.11.001
• Languages: English
• ISSNs: 0268-960X

Abstract

Abstract: Hematopoietic stem cell transplantation (HSCT)-associated thrombotic microangiopathy (TA-TMA) is now a well-recognized and potentially severe complication of HSCT that carries a high risk of death. In those who survive, TA-TMA may be associated with long-term morbidity and chronic organ injury. Recently, there have been new insights into the incidence, pathophysiology, and management of TA-TMA. Specifically, TA-TMA can manifest as a multi-system disease occurring after various triggers of small vessel endothelial injury, leading to subsequent tissue damage in different organs. While the kidney is most commonly affected, TA-TMA involving organs such as the lung, bowel, heart, and brain is now known to have specific clinical presentations. We now review the most up-to-date research on TA-TMA, focusing on the pathogenesis of endothelial injury, the diagnosis of TA-TMA affecting the kidney and other organs, and new clinical approaches to the management of this complication after HSCT.