German S3 guideline on the diagnosis, treatment, and follow-up care of thrombotic thrombocytopenic purpura (TTP)

Introduction Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening disease. It is a thrombotic microangiopathy (TMA) caused by ADAMTS13 (VWF-cleaving protease) deficiency. Blood clots in the capillary bed lead to oxygen deficiency in vital organs. Without rapid diagnosis and treatment, patients die within a few days. With optimal treatment, the survival rate is over 90% [ 1 ]. Despite new treatment options, there is limited high-quality data available. German guidelines were therefore necessary to offer standardized recommendations for patient care, quality assurance, and medical decision-making. Method The development process was carried out in accordance with the AWMF guidance (version 2.1 dated September 5, 2023) for S3-guidelines. In September 2022 a multidisciplinary guideline panel was established. For PICO questions, a systematic search and selection of the available scientific evidence was carried out. The evidence was evaluated using the GRADE method. Recommendations were drafted and graded based on evidence, clinical experience of the guideline panel and patient preferences. They were adopted through a structured consensus process, taking conflicts of interest into account. An internal review was followed by public consultation, approval by the executive boards of all participating medical societies and the Innovation Fund of the Federal Committee (GBA). Results Representatives of 17 German medical societies participated in the guideline group. In addition, 2 patient representatives and 14 recognized TTP experts were invited. 38 clinically relevant PICO questions were formulated. The panel agreed on 9 statements and 76 treatment-oriented recommendations based on evidence ranging from very low to moderate certainty. The guideline recommendations cover epidemiology, diagnosis, treatment, follow-up of TTP and of pregnancy and TTP. For first acute episodes and relapses of iTTP, the panel made a strong recommendation for adding corticosteroids to therapeutic plasma exchange (TPE), a weak recommendation for adding rituximab and a consensus based recommendation for adding caplacizumab. For asymptomatic iTTP with low plasma ADAMTS13 activity, the panel made a weak recommendation for the preemptive use of rituximab. For asymptomatic cTTP, the panel made weak recommendation for prophylactic plasma infusion and a neutral recommendation for prophylactic rADAMTS13 infusion. Conclusion This is the first German S3-TTP guideline. The panel’s recommendations provide structured evidence-based guidance for this rare and life-threatening disease, including suppressing inflammation, blocking platelet clumping, replacing the missing and/or inhibited ADAMTS13, and suppressing the formation of ADAMTS13 autoantibody. TTP can thus be treated quickly and comprehensively, even in primary care settings and by inexperienced colleagues. In particular, this should facilitate the logistics of special diagnostics, the use of newly approved drugs and nationwide follow-up care. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany