Diagnosis and Management of Thrombotic Microangiopathies
摘要
Thrombotic microangiopathies (TMAs) are a group of disorders which share common characteristics such as intraluminal thrombosis, anemia, and thrombocytopenia. Thrombotic thrombocytopenic purpura (TTP) and atypical hemolytic uremic syndrome (aHUS) constitute the most common TMAs. TTP is caused due to deficiency of von Willebrand factor (vWF)-cleaving specific serine metalloprotease ADAMTS13. A combination of ischemic multisystem features along with laboratory investigation such as schistocytes on peripheral smear helps to diagnose TTP. Therapeutic plasma exchange (TPE) remains at the forefront of treating TTP. ICU admission and vigilant monitoring remains at the forefront of achieving successful outcomes with TTP. TMAs other than TTP are less common with patients treated with a personalized approach to all TMAs.