Status on pathogenesis, diagnosis and treatment of hematopoietic stem cell transplantation associated thrombotic microangiopathy

Abstract

Transplantation associated thrombotic microangiopathy (TA-TMA) is a significant complication after hematopoietic stem cell transplantation (HSCT), and may lead to a high risk of death. It is a multifactorial disorder focused on small vessel endothelial injury which can be trigged by varieties of mechanisms during HSCT. The complement system is found to play a role in the development of TA-TMA. Current laboratory diagnostic criteria are unsatisfactory and the latest diagnostic criterion includes activation of the complement system. But the pathological diagnosis remains to be the golden standard. Blocking the complement system with eculizumab may be one of the effective treatment for high-risk patients with TA-TMA. This article reviews the status on pathogenesis, diagnosis and treatment of TA-TMA. Key words: Thrombotic microangiopathies; Hematopoietic stem cell transplantation; Endothelium, vascular; Complement system proteins; Transplantation associated thrombotic microangiopathy; Endothelial injury; Eculizumab