Update on demographic data and clinical manifestations of the patient cohort in the Mainz TTP-Registry

Introduction Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy caused by a severe deficiency in the metalloprotease ADAMTS13. Approximately 30-40% of patients experience relapses, with the highest risk occuring within the first year of remission and decreasing over time [ 1 ]. Due to the potentially fatal nature of the disease, early detection or prediction of relapses is crucial and regular monitoring of ADAMTS13-activity during remission is of great clinical importance [ 2 ]. We aimed to evaluate structured follow-up of TTP patients and to identify predictive markers associated with morbidity and mortality during acute TTP episodes and relapses. Method We conducted an analysis based on our long-term prospective TTP-registry, including 188 patients enrolled between 2016 and 2024: 167 patients with immune-mediated TTP (iTTP), 6 with congenital TTP (cTTP) and 15 with other thrombotic microangiopathies. We included patients who experienced a minimum of one acute TTP episode. Data was collected using standardized case report forms. Results Our patient cohort consists mainly of women (76.7% female, 23.3% male) and had a median age at first diagnose of 35 years (range 12 – 85). A total of 82 acute episodes were documented in 51 patients, of which 62 represented relapses. During the observation period, 38 patients experienced one acute episode, 7 had two episodes, 3 had four, 2 had five and 1 patient had a total of eight episodes. 150 patients (87.7%) had at least one comorbidity, with autoimmune diseases (36.0%), thyroid diseases (33.3%) and hypertension (32.9%) being the most prevalent. The most frequent potential triggers were infections (22.1%), initiation of new medication (15.3%) and Pregnancy (1.7%) followed by other causes. The most common initial symptoms at presentation were neurological symptoms (60.0%) including headache (35.0%) and vertigo (26.7%), signs of bleeding (60.0%) such as petechiae (41.7%), hematomas (40.0%) and hematuria (16.7%), as well as fatigue (46.7%). 4 patients died during the observation period, 3 in relation to the disease. Conclusion Our registry highlights the importance of structured long-term follow-up in TTP patients. Regular monitoring of ADAMTS13 activity and clinical parameters may allow early detection and prevention of impending relapses and improve patient outcomes. Further analyses are ongoing to identify reliable biomarkers predicting morbidity and mortality in acute and relapsing TTP. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany