Recurrent Disease in 25 Patients with Acquired Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome during 6.5 Years Follow-Up

Abstract

Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are manifestations of excessive microvascular platelet aggregation and present dangerous thrombotic microangiopathies (TMA). The clinical presentation of TTP/HUS is variable in terms of type and severity of clinical symptoms. Before the introduction of plasma exchange (PEX) for the treatment of acute TTP episode mortality reached 90%, but decreased to 8-30% following the adoption of PEX. Acute idiopathic TTP is the most common type of TTP seen in clinical practice. The clinical course of idiopathic TTP is characterized by recurrent disease episodes in up to 50% of cases; with a higher risk of recurrence in those with severe deficiency of the von Willebrand factor cleavingprotease ADAMTS13 during acute episodes. We report on the recurrences among 25 patients with TTP and HUS, in whom we monitored routine laboratory parameters, response to treatment as well as ADAMTS13 activity and anti-ADAMTS13 antibodies in acute phase and remission.