Sepsis-induced disseminated intravascular coagulation with features of thrombotic thrombocytopenic purpura: a fatal fulminant syndrome.
Disseminated intravascular coagulation (DIC) and thrombotic thrombocytopenic purpura (TTP) are different disease states, while ADAMTS13 deficiency could occur in sepsis-induced DIC. We report 2 patients who had septic DIC with features of idiopathic TTP characterized by low ADAMTS13 activity and positive ADAMTS13 inhibitor. They had a specific fulminant course and fatal outcome, which might represent a new specific syndrome.
Wang
Zhaoyue
Z
Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Suzhou, China. zwang11@sina.com
Yu
Ziqiang
Z
Su
Jian
J
Cao
Lijuan
L
Zhao
Xiaojuan
X
Ruan
Changgeng
C
D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000328:Adult; D004211:Disseminated Intravascular Coagulation / Q000097:blood / Q000209:etiology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008875:Middle Aged; D011480:Protease Inhibitors / Q000097:blood; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000209:etiology*; D018805:Sepsis / Q000097:blood / Q000150:complications*; D013577:Syndrome
D011480:Protease Inhibitors; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human