Clinical Analysis of 15 Adult Patients with Thrombotic Thrombocytopenic Purpura

ABSTRACT

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia ascribable to platelet-rich microthrombi. TTP is related to a severe deficiency in ADAMTS13, the specific von Willebrand factor-cleaving metalloprotease. The aim of our study is to analyze the clinical characteristics, treatment and outcome of patients with TTP. Method(s) We retrospectively analyzed 15 patients with TTP treated Farhat Hached hospital, Sousse, Tunisia, from2004 to 2021. Result(s) Among the 15 patients, there were 7 males and 8 females, with a median age of 45,5 (30~72) years. Two of them had evolutive cancer and 1 had covid-19 vaccination 3 months earlier. Twelve patients had neurological presentations (80%), 4 had mucocutaneous bleeding and 2 had fever. Biology showed thrombopenia, mechanical hemolytic anemia in all patients and renal failure in 2 patients. No abnormalities in coagulation tests were detected. The ADAMTS13 activity was performed in only 3 patients (Due to test unavailability) showing a level <=10% in all 3 patients. All patients were treated with plasma exchange/ plasma infusion + glucocorticoid, combined with rituximab in 2 cases. Ten patients showed complete remission, 1 relapsed within the first year, 2 died and 2 others were lost to follow-up. Discussion/Conclusion: Most TTP patients presented with the triad of microangiopathic anemia, thrombocytopenia, and neurologic abnormalities and improved with plasma therapy.