Schimpf Judith, Haller Patrizia, Zitt Emanuel
Department of Internal Medicine 3 (Nephrology, Dialysis and Hypertension), Landeskrankenhaus (LKH) Feldkirch, Feldkirch, Austria.
Vorarlberg Institute for Vascular Investigation and Treatment (VIVIT), Feldkirch, Austria.
Front Immunol. 2025 Apr 28;16:1588471. doi: 10.3389/fimmu.2025.1588471. eCollection 2025.
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening disorder due to a severe acquired or inherited ADAMTS13 deficiency. So far, therapeutic algorithms almost universally include the prompt initiation of therapeutic plasma exchange (TPE). We firstly report a 55-year-old female with a history of relapsing TTP who was managed exclusively with caplacizumab, steroids and the second generation fully humanized anti-CD20 monoclonal antibody obinutuzumab during a relapse without the need of TPE throughout the whole disease course. This case illustrates the safety and effectiveness of a TPE-free TTP management using prompt initiation of caplacizumab and obinutuzumab.
血栓性血小板减少性紫癜(TTP)是一种罕见且危及生命的疾病,由严重的获得性或遗传性ADAMTS13缺乏引起。到目前为止,治疗方案几乎普遍包括立即开始治疗性血浆置换(TPE)。我们首次报告了一名55岁复发性TTP女性患者,在复发期间仅使用卡泊单抗、类固醇和第二代全人源抗CD20单克隆抗体奥妥珠单抗进行治疗,整个病程无需TPE。该病例说明了立即使用卡泊单抗和奥妥珠单抗进行无TPE的TTP管理的安全性和有效性。