Outcomes of patients with complement-mediated thrombotic microangiopathy

Authors : Davut Akın

Pages : 151-155

Doi:10.31362/patd.1579336

View : 43 | Download : 115

Publication Date : 2025-01-01

Article Type : Research Paper

Abstract :Purpose: Complement-mediated thrombotic microangiopathy (CM-TMA) is a rare, progressive and lifethreatening type of thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia and associated acute kidney disease (AKI) caused by dysregulation of the alternative complement pathway. The aim of this study was to retrospectively analyze the clinical features, follow-up, treatment and mortality of patients with CM-TMA. Materials and methods: This was a retrospective study evaluating 13 patients diagnosed with CM-TMA who were followed retrospectively from 2024. Data were collected through a comprehensive review of electronic medical records of patients diagnosed with CM-TMA and receiving Eculizumab in the Department of Hematology and Nephrology. Results: Thirteen patients with a mean age at diagnosis of 36.0±17.8 years were included. Age at disease onset ranged from 17 to 66 years. Only 3 (23.1%) patients were over 50 years of age. All patients were female. The mean follow-up period was 78.6±34.6 months. After an increase in GFR with eculizumab treatment, 76.9% of patients were weaned from dialysis. Conclusion: CM-TMA was found to be predominant in young women. Eculizumab treatment provided significant improvements in clinical and laboratory values of the patients.
Keywords : CM-TMA, aHUS, C5 inhibitörleri, eculizumab, kompleman aracılı trombotik mikroanjiyopati