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. 2025 Mar 17;10(6):1950-1959.
doi: 10.1016/j.ekir.2025.03.019. eCollection 2025 Jun.

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

Jean-Michel Halimi  1   2   3 , Anna Duval  4 , Etienne Chardon  2   5 , Laurent Mesnard  6 , Marie Frimat  7 , Fadi Fakhouri  8 , Steven Grangé  9 , Aude Servais  10 , Claire Cartery  11 , Paul Coppo  12 , Dimitri Titeca-Beauport  13 , Sébastien Roger  2 , Nadine Baroukh  1   2 , Nicolas Fage  14 , Yahsou Delmas  15 , Anne-Hélène Quérard  16 , Guillaume Seret  17 , Mickaël Bobot  18   19 , Moglie Le Quintrec  20 , Simon Ville  21 , Florent von Tokarski  22 , Sophie Chauvet  23 , Alain Wynckel  24 , Manon Martins  25 , Juliet Schurder  26 , Christelle Barbet  1 , Bénédicte Sautenet  1   27 , Philippe Gatault  1   2 , Sophie Caillard  4 , Charles Antunes  28 , Guillaume Bayer  29 , Carole Philipponnet  30 , Vincent Audard  31 , Nicolas Maillard  32 , Vincent Vuiblet  33 , Viviane Gnemmi  7 , Zhour El Ouafi  34 , Sébastien Canet  35 , Manon Dekeyser  36 , Éric Piver  5   37 , Valentin Maisons  1   27 ; MATRIX Consortium Group
Affiliations

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

Jean-Michel Halimi et al. Kidney Int Rep. .

Abstract

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.

Keywords: biological parameters; cohort; schistocytes; thrombotic microangiopathy.

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Figures

None
Graphical abstract
Figure 1
Figure 1
Impacts of fibrinogen levels and prothrombin time on the diagnostic value of biological parameters to suspect TMA. The proportion of patients with a given parameter in our patients with TMA was expressed as the percentage of patients with the biological parameters, according to fibrinogen levels and prothrombin time. LDH, lactate dehydrogenase; TMA, thrombotic microangiopathy.
Figure 2
Figure 2
Combined impact of fibrinogen and serum creatinine levels. The proportion of patients with a given parameter in our patients with TMA was expressed as the percentage of patients with the biological parameters, according to fibrinogen and serum creatinine levels. LDH, lactate dehydrogenase; TMA, thrombotic microangiopathy.
See this image and copyright information in PMC

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