How I treat How I treat patients with thrombotic thrombocytopenic purpura: 2010

(TTP) is the common name for adults with microangiopathic hemolytic anemia, thrombocytopenia, with or without neuro-logic or renal abnormalities, and without another etiology; children without renal failure are also described as TTP. The diagnosis of TTP is an indication for plasma exchange treatment, but begin-ning treatment requires sufficient confi-dence in the diagnosis to justify the risk of plasma exchange complications. Docu-mentation of a severe deficiency of plasma ADAMTS13 activity, defined as less than 10 % of normal, is not essential for the diagnosis of TTP. Some patients without severe ADAMTS13 deficiency may benefit from plasma exchange treatment; in addition, some patients with severe ADAMTS13 deficiency may subsequently be diagnosed with another cause for their clinical features. However, severe ac-quired ADAMTS13 deficiency does define a subgroup of patients who appear to benefit from treatment with corticoste-roids and other immunosuppressive agents in addition to plasma exchange but who have a high risk for relapse. Approximately 80 % of patients survive their acute episode, a survival rate that has not changed since the introduction of plasma exchange treatment. Although re-covery may appear to be complete, many patients have persistent minor cognitive abnormalities. More effective as well as safer treatment for TTP is needed. (Blood. 2010;116(20):4060-4069