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©2013 UpToDate ® Print Email Algorithm: suggested management of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) The clinical course of patients with TTP may be complex and cannot be easily represented by a single diagram. Continued search for alternative etiologies for the patient's clinical features is critical, even after beginning plasma exchange. The clinical basis for suspecting severe ADAMTS13 deficiency is described in the text. Exacerbations of TTP, either while continuing daily plasma exchange or after plasma exchange is stopped, and relapses rarely occur in patients without ADAMTS13 deficiency. Although rituximab may be appropriate for three different situations illustrated in this algorithm, we have never used more than a single course of rituximab for any patient. Definitions for response, exacerbation, remission, and relapse have been previously described. [1] Broken lines represent complications that occur in a minority of patients. PEX: plasma exchange; CVC: central venous catheter. Reference: Vesely SK, George JN, Lämmle B, et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood. 2003; 101(1):60-68. This research was originally published in Blood. George JN. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood 2010; 116:4060. Copyright © 2010 American Society of Hematology.