Diagnostic Value of Biological Parameters in Biopsy-Confirmed Thrombotic Microangiopathy–MATRIX Consortium Group

Diagnostic Value of Biological Parameters in Biopsy-Confirmed Thrombotic Microangiopathy–MATRIX Consortium Group

Introduction: The diagnosis of thrombotic microangiopathy (TMA) relies on common biological parameters, the diagnostic value of which are unknown. Methods: The presence of common biological parameters was assessed in 967 patients with TMA from 2009 to 2023 (ClinicalTrials.gov: NCT05991245). Results:...

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Main Authors: Jean-Michel Halimi, Anna Duval, Etienne Chardon, Laurent Mesnard, Marie Frimat, Fadi Fakhouri, Steven Grangé, Aude Servais, Claire Cartery, Paul Coppo, Dimitri Titeca-Beauport, Sébastien Roger, Nadine Baroukh, Nicolas Fage, Yahsou Delmas, Anne-Hélène Quérard, Guillaume Seret, Mickaël Bobot, Moglie Le Quintrec, Simon Ville, Florent von Tokarski, Sophie Chauvet, Alain Wynckel, Manon Martins, Juliet Schurder, Christelle Barbet, Bénédicte Sautenet, Philippe Gatault, Sophie Caillard, Charles Antunes, Guillaume Bayer, Carole Philipponnet, Vincent Audard, Nicolas Maillard, Vincent Vuiblet, Viviane Gnemmi, Zhour El Ouafi, Sébastien Canet, Manon Dekeyser, Éric Piver, Valentin Maisons
Format: Article
Language:English
Published: Elsevier 2025-06-01
Series:Kidney International Reports
Subjects:
biological parameters
cohort
schistocytes
thrombotic microangiopathy
Online Access:http://www.sciencedirect.com/science/article/pii/S2468024925001627
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Summary:Introduction: The diagnosis of thrombotic microangiopathy (TMA) relies on common biological parameters, the diagnostic value of which are unknown. Methods: The presence of common biological parameters was assessed in 967 patients with TMA from 2009 to 2023 (ClinicalTrials.gov: NCT05991245). Results: The median age was 49 (36–64) years and 53.2% were male. All TMA causes were represented (atypical hemolytic uremic syndrome [aHUS]: 41.6%, drugs: 24.9%, malignancy: 21.4%, autoimmune disease: 18.8%, infection: 7.6%, complement-mediated HUS: 6.8%, organ transplantation: 5.8%, pregnancy: 3.8%, bone marrow transplantation [BMT]: 2.9%, Shiga toxin Escherichia coli hemolytic uremic syndrome [STEC-HUS]: 0.6%, and thrombotic thrombocytopenic purpura [TTP]: 0.6%). The presence of TMA-related parameters concerned virtually all patients with TTP but varied widely for the other patients as follows: anemia: 81.7%, high lactate dehydrogenase (LDH) (75.4%), low haptoglobin (53.7%), and thrombocytopenia (40.3%). Their diagnostic performance was accurate only for TTP. Eleven distinct ways were used for schistocyte metrics and reporting. Relying on schistocyte presence as the single diagnostic criterion would lead to missed diagnosis in 23.8% (STEC-HUS) to 86.4% (BMT) of patients (for anemia: 8.2%–22.3%; thrombocytopenia: 31.8%–67.9%; high LDH: 10.0%–40.7%, low haptoglobin: 0%–70.4%, according to the causes of TMA). The overall risk of missed diagnosis using these parameters was ≥ 50% in all TMA, except in TTP. The best diagnostic performances were obtained when fibrinogen levels were < 5 g/l, creatinine ≥ 300 μmol/l, prothrombin time (PT) < 90%; and when TMA causes were TTP, STEC-HUS, infection, or complement-mediated HUS. Conclusion: Common biological parameters miss the diagnosis in more than 50% of TMA except when fibrinogen is < 5 g/l, creatinine ≥ 300 μmol/l, and PT < 90%. Schistocyte reporting is heterogenous, and its results are usually deceptive in TMA.
ISSN:2468-0249

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