| Theme |
Antiplatelet and Anticoagulation Therapy in Patients with Chronic Kidney Disease -- up to date of evidence |
| Title |
Pathogenesis and treatment of thrombotic microangiopathy (TTP, HUS) |
| Author |
Eriko Kinugasa |
Department of Internal Medicine, Showa University Northern Yokohama Hospital |
| Author |
Yoshinori Saitoh |
Department of Internal Medicine, Showa University Northern Yokohama Hospital |
| Author |
Noriyo Yoshida |
Department of Internal Medicine, Showa University Northern Yokohama Hospital |
| Author |
Akiko Sakashita |
Department of Internal Medicine, Showa University Northern Yokohama Hospital |
| [ Summary ] |
Thrombotic microangipathy (TMA) is a syndrome consisting of microangiopathic hemolytic anemia, destructive thrombocytopenia and microvascular thrombi. Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndorome (HUS) are diseases representative of TMA. Recently, it has been noted that decreased activity of the von Willebrand factor cleaving enzyme, ADAMTS 13, plays a central role in the pathogenesis of certain types of TTP. Plasma exchange (PE) is effective not only in recovery of ADAMST 13 activity, but also in improving the prognosis for TTP. More recently, it has been reported that rituximab is also useful in controlling TTP. Future studies are expected on combination therapies with PE. HUS developes primarily in children after pathogenic E. coli infections, which produce verotoxin. Supportive care including, dialysis therapy, is sufficient for these cases and produces better outcomes. On the other hand, the prognosis for renal disease patients is generally poor for those with HUS. |