Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) caused by ADAMTS13 deficiency. Although the name of TTP is well known, most hematologists find its diagnosis and treatment difficult because it is ultrarare. TTP is an acute-onset and fatal disorder. Approximately 90% of TTP patients die within 2 weeks of onset without proper medical treatment. Although most doctors may remember being taught the five TTP symptoms (fever, transient central nervous system symptoms, hemolytic anemia, thrombocytopenia, and kidney dysfunction) at medical school, only 7% of TTP patients present with all five symptoms. Thus, TTP must be suspected in patients with microangiopathic hemolytic anemia and thrombocytopenia, and plasma therapy must be initiated as soon as possible after ordering the ADAMTS13 test. In this article, I describe how to differentially diagnose TMA as well as the standard and the updated therapy, such as rituximab and caplacizumab, for TTP treatment.
Keywords: ADAMTS13; Plasma exchange; Rituximab; TTP.