Assessment of clinical characteristics, treatment responses, relapses, and survival in patients with thrombotic thrombocytopenic purpura undergoing therapeutic plasma exchange: A single-center experience


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Doğan A., İpek R.

Pakistan Journal of Medical Sciences, cilt.42, sa.4, ss.961-968, 2026 (SCI-Expanded, Scopus) identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 42 Sayı: 4
  • Basım Tarihi: 2026
  • Doi Numarası: 10.12669/pjms.42.4.14962
  • Dergi Adı: Pakistan Journal of Medical Sciences
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE, Directory of Open Access Journals
  • Sayfa Sayıları: ss.961-968
  • Anahtar Kelimeler: ADAMTS13, Inhibitor, Plasma exchange, PLASMIC score, Therapeutic, Thrombotic thrombocytopenic purpura
  • Açık Arşiv Koleksiyonu: AVESİS Açık Erişim Koleksiyonu
  • Van Yüzüncü Yıl Üniversitesi Adresli: Evet

Özet

Objective: The present study evaluates the clinical characteristics, laboratory values, treatment responses, relapse rates, and survival outcomes in patients diagnosed with thrombotic thrombocytopenic purpura (TTP) undergoing therapeutic plasma exchange (TPE). Methodology: Included in this retrospective single-center study were 55 patients who underwent TPE with a diagnosis of TTP between 2012 to 2025. The study was conducted between October and December 2025 at Van Yuzuncu Yil University Medical Faculty Hospital. Demographic characteristics, clinical findings, laboratory results, administered therapies, complications, treatment responses, and survival data were retrieved from the hospital automation system and patient medical records. Results: The median age of the patients was 34 years, and 67.3% were female. Based on PLASMIC scores, 76.4% of the patients were classified as high risk, among whom 36.4% tested positive for an ADAMTS13 inhibitor (a disintegrin and metalloproteinase with a thrombospondin type-1 motif, member 13). The complete response rate to TPE was 75.5%, whereas the relapse rate was 23.6%, and among those who relapsed, 84.6% tested positive for the ADAMTS13 inhibitor. The median event-free survival (EFS) was 158 months, and the overall survival (OS) rate was 92.7%. The mean OS duration was determined to be 157.2 months. The median EFS was 64.8 months in those who tested positive for the ADAMTS13 inhibitor, and 158 months in those who tested negative. Conclusion: In the present study, TPE achieved high response and survival rates. High PLASMIC scores strengthen the clinical utility of the scoring system in the diagnosis of TTP. ADAMTS13 inhibitor positivity was a significant indicator of relapse risk.