Clinicopathologic spectrum and prognostic determinants in biopsy-proven thrombotic microangiopathy.

Authors

Taha Enes Cetin, Veysel Baran Tomar, Yagmur Eken, Betul Ogut, Ipek Isik Gonul, Ozant Helvaci, Ulver Derici, Galip Guz, Omer Faruk Akcay

Published in

Medicina clinica. Volume 166. Issue 8. Pages 107509. Jun 27, 2026. Epub Jun 27, 2026.

Abstract

Thrombotic microangiopathy (TMA) represents a heterogeneous clinicopathologic entity characterized by endothelial injury, microvascular thrombosis, and variable clinical outcomes. Data on prognostic factors in biopsy-proven TMA remain limited.
This retrospective single-center study included 46 adult patients with biopsy-proven TMA diagnosed between 2000 and 2025. Demographic, clinical, laboratory, histopathologic, and genetic parameters were analyzed. The composite adverse outcome was defined as death or progression to end-stage renal disease (ESRD). Logistic regression analysis was performed to identify prognostic determinants.
The most common etiologies were atypical hemolytic uremic syndrome (37.0%) and peripartum (pregnancy-associated) thrombotic microangiopathy (26.1%). During follow-up, 43.5% of patients progressed to end-stage renal disease or died. In univariate analyses, the presence of acute kidney injury at diagnosis (p=0.017) and lower estimated glomerular filtration rate (eGFR) (p=0.016) were associated with poorer outcomes. In contrast, both early treatment response within the first week (p=0.004) and peripartum TMA (p=0.042) were associated with more favorable outcomes. Notably, early treatment response remained significantly associated with improved outcomes in multivariate analyses (odds ratio [OR]=0.15; 95% confidence interval [CI], 0.03-0.75; p=0.021). Complement gene variants were identified in 5 of 10 tested patients, supporting a potential role for complement dysregulation in the pathogenesis of TMA.
Early on-treatment response may serve as a dynamic prognostic marker reflecting short-term disease trajectory in biopsy-proven TMA. These findings highlight the potential importance of timely recognition and close monitoring of treatment response in patients with biopsy-proven TMA.

PMID:
42364282
Bibliographic data and abstract were imported from PubMed on 28 Jun 2026.

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