Turk J Hematol. Ahead of Print: TJH-25307 | DOI: 10.4274/tjh.galenos.2025.2025.0134

Clinical Manifestations, Treatment Characteristics and Clinical Outcome in Patients with Immune Thrombotic Thrombocytopenic Purpura (iTTP) in the Real-World Setting: An Interim Analysis of the Turkish iTTP Registry

Sema Karakuş¹, Şule Mine Bakanay², Sevgi Kalayoğlu Beşişik³, Ahmet Emre Eşkazan⁴, Orhan Ayyıldız⁵, Emel Gürkan⁶, Ozan Salim⁷, Volkan Karakuş⁸, Nil Güler⁹, Muzaffer Keklik¹⁰, Osman İlhami Özcebe¹¹, Fahir Özkalemkaş¹², Vildan Özkocaman¹², Umut Yılmaz⁴, Senem Dadin³, Mehmet Ali Uçar⁶, Mehmet Sönmez¹³, Abdullah Karakuş⁵, Ünal Ataş⁷, Lütfullah Zahit Koç⁸, Eren Gündüz¹⁴, Sibel Kabukçu Hacıoğlu⁹, Başak Ünver Koluman⁹, Gülsüm Özet², Tekin Güney Et Al.^2
1Başkent University Faculty of Medicine, Department of Hematology, Ankara, Türkiye
²Ankara Bilkent City Hospital, Clinic of Hematology, Ankara, Türkiye
³İstanbul University, İstanbul Faculty of Medicine, Department of Hematology, İstanbul, Türkiye
⁴İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Hematology, İstanbul, Türkiye
⁵Dicle University Faculty of Medicine, Department of Hematology, Diyarbakır, Türkiye
⁶Çukurova University Faculty of Medicine, Department of Hematology, Adana, Türkiye
⁷Akdeniz University Faculty of Medicine, Department of Hematology, Antalya, Türkiye
⁸University of Health Sciences Türkiye, Antalya Training and Research Hospital, Clinic of Hematology, Antalya, Türkiye
⁹Pamukkale University Faculty of Medicine, Department of Hematology, Denizli, Türkiye
¹⁰Erciyes University Faculty of Medicine, Department of Hematology, Kayseri, Türkiye
¹¹Hacettepe University Faculty of Medicine, Department of Hematology, Ankara, Türkiye
¹²Bursa Uludağ University Faculty of Medicine, Department of Hematology, Bursa, Türkiye
¹³Karadeniz Technical University Faculty of Medicine, Department of Hematology, Trabzon, Türkiye
¹⁴Eskişehir Osmangazi University Faculty of Medicine, Department of Hematology, Eskişehir, Türkiye

Objective: This study aimed to investigate the clinical manifestations, treatment patterns and clinical outcomes in patients with iTTP across Türkiye via an interim analysis of the Turkish iTTP registry.
Materials and Methods: A total of 215 patients with iTTP (median age at diagnosis 41 years, 58.6% female) diagnosed between 2001 and 2023 were retrospectively analyzed in the interim analysis of the prospective noninterventional observational multicenter iTTP registry study conducted at 19 tertiary hematology centers. Data on patient demographics, disease characteristics on initial admission, treatment characteristics, and responses, exacerbations/relapses, and the survival outcome were registered via electronic case report forms.
Results: Infection (15.0%), new drug initiation (9.7%), and pregnancy/postpartum (6.3%) within 3 weeks before diagnosis were the most prevalent potential triggers. Patients presented more commonly with systemic/constitutional (fatigue 68.8%; fever 18.1%) and neurologic (headache 40.0%, vertigo 32.1%), followed by hemorrhagic, gastrointestinal, renal, and cardiovascular manifestations. Based on the PLASMIC risk scoring, 77.8% of patients were initially at high risk for TTP. The initial treatment was commenced within the first 48 hours of hospital admission in 64.1% of patients (36.2% on the day of admission). Treatment was mainly based on TPE exchange (92.1%) and steroids (63.7%), while rituximab was used in 15.8% of patients. The clinical response rate was 79.9%, and clinical remission was achieved in 68.2% of patients. As for the ADAMTS13 levels, partial and complete responses were achieved in 17.7% and 14.6%, respectively. During a median of 30 months (range, 0.1 to 262.4 months) follow-up, 35 patients experienced exacerbations/relapses. Mortality occurred in 11 (5.5%) patients and was found to be disease-related in 6 (3.0%).
Conclusion: This interim analysis of the nationwide Turkish iTTP registry study provided valuable data on the real-world clinical practice in the diagnosis and management of iTTP at different hematology clinics across the country.

Keywords: Thrombotic thrombocytopenic purpura, Acquired thrombotic disorders, Acquired platelet disorders, Autoimmune disorders, Apheresis

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