A new paradigm: Diagnosis and management of HSCT associated thrombotic microangiopathy as multi system endothelial injury.
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.
Blood reviews