Report on Experts Forum «Thrombotic microangiopathies and atypical hemolytic-uremic syndrome in obstetrics. The first step to cross-disciplinary consensus»

2017 ◽  
Vol 17 (1) ◽  
pp. 118 ◽  
Author(s):  
V. I. Krasnopolsky ◽  
E. M. Shifman ◽  
A. V. Kulikov ◽  
N. L. Kozlovskaya ◽  
N. V. Artymuk ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Thrombotic Microangiopathies ◽  
Uremic Syndrome

Treatment of Atypical Hemolytic Uremic Syndrome and Thrombotic Microangiopathies: A Focus on Eculizumab

2013 ◽  
Vol 61 (2) ◽  
pp. 289-299 ◽  
Author(s):  
Jan Schmidtko ◽  
Sven Peine ◽  
Youssef El-Housseini ◽  
Manuel Pascual ◽  
Pascal Meier
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Thrombotic Microangiopathies ◽  
Uremic Syndrome

scholarly journals Complement Activation and Thrombotic Microangiopathies

2019 ◽  
Vol 14 (12) ◽  
pp. 1719-1732 ◽  
Author(s):  
Marta Palomo ◽  
Miquel Blasco ◽  
Patricia Molina ◽  
Miquel Lozano ◽  
Manuel Praga ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Hemolytic Uremic Syndrome ◽  
Acute Phase ◽  
Complement Activation ◽  
Hellp Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Malignant Hypertension ◽  
Clinical Relapse ◽  
Thrombotic Microangiopathies ◽  
Uremic Syndrome

Background and objectivesAtypical hemolytic uremic syndrome is a form of thrombotic microangiopathy caused by dysregulation of the alternative complement pathway. There is evidence showing complement activation in other thrombotic microangiopathies. The aim of this study was to evaluate complement activation in different thrombotic microangiopathies and to monitor treatment response.Design, setting, participants, & measurementsComplement activation was assessed by exposing endothelial cells to sera or activated-patient plasma—citrated plasma mixed with a control sera pool (1:1)—to analyze C5b-9 deposits by immunofluorescence. Patients with atypical hemolytic uremic syndrome (n=34) at different stages of the disease, HELLP syndrome (a pregnancy complication characterized by hemolysis, elevated liver enzymes, and low platelet count) or severe preeclampsia (n=10), and malignant hypertension (n=5) were included.ResultsAcute phase atypical hemolytic uremic syndrome–activated plasma induced an increased C5b-9 deposition on endothelial cells. Standard and lower doses of eculizumab inhibited C5b-9 deposition in all patients with atypical hemolytic uremic syndrome, except in two who showed partial remission and clinical relapse. Significant fibrin formation was observed together with C5b-9 deposition. Results obtained using activated-plasma samples were more marked and reproducible than those obtained with sera. C5b-9 deposition was also increased with samples from patients with HELLP (all cases) and preeclampsia (90%) at disease onset. This increase was sustained in those with HELLP after 40 days, and levels normalized in patients with both HELLP and preeclampsia after 6–9 months. Complement activation in those with malignant hypertension was at control levels.ConclusionsThe proposed methodology identifies complement overactivation in patients with atypical hemolytic uremic syndrome at acute phase and in other diseases such as HELLP syndrome and preeclampsia. Moreover, it is sensitive enough to individually assess the efficiency of the C5 inhibition treatment.

scholarly journals Modified Ham test for atypical hemolytic uremic syndrome

Blood ◽  
2015 ◽  
Vol 125 (23) ◽  
pp. 3637-3646 ◽  
Author(s):  
Eleni Gavriilaki ◽  
Xuan Yuan ◽  
Zhaohui Ye ◽  
Alexander J. Ambinder ◽  
Satish P. Shanbhag ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Cell Lines ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Cell Killing ◽  
Thrombotic Microangiopathies ◽  
Gpi Anchor ◽  
Deficient Cell ◽  
Key Points ◽  
Uremic Syndrome

Key Points GPI-anchor–deficient cell lines are more vulnerable to complement C5b-9 deposition and cell killing from aHUS serum. PIGA-null reagent cell lines can be used to rapidly and reliably distinguish aHUS from other thrombotic microangiopathies.

scholarly journals Retrospective and Systematic Analysis of Causes and Outcomes of Thrombotic Microangiopathies in Routine Clinical Practice: An 11-Year Study

2021 ◽  
Vol 8 ◽  
Author(s):  
Nicolas Henry ◽  
Chloé Mellaza ◽  
Nicolas Fage ◽  
François Beloncle ◽  
Franck Genevieve ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Routine Clinical Practice ◽  
Complement Pathway ◽  
Thrombotic Microangiopathies ◽  
Thrombocytopenic Purpura ◽  
Multiple Factors ◽  
Alternative Complement ◽  
Uremic Syndrome

Background: Thrombotic microangiopathies (TMAs) are highly suspected in patients showing mechanical hemolytic anemia, thrombocytopenia, and haptoglobin consumption. Primary [thrombotic thrombocytopenic purpura (TTP) and atypical hemolytic uremic syndrome] and secondary TMA are considered. Even if ADAMTS13 measurements and alternative complement pathway explorations have greatly improved the ability to identify primary TMA, their diagnosis remains difficult, and their frequency relative to that of secondary TMA is undetermined. The objectives of the present study were, therefore, to describe the etiologies, management, and the outcomes of patients presenting with TMA in real-life clinical practice.Methods: We conducted a retrospective study between 01/01/2008 and 31/12/2018 that included all consecutive patients presenting with biological TMA syndrome at admission or developing during hospitalization. Patients were identified from the laboratory databases, and their medical files were reviewed to confirm TMA diagnosis, to determine etiology, and to analyze their therapeutic management and outcomes.Results: During this period, 239 patients with a full TMA biological syndrome were identified, and the TMA diagnosis was finally confirmed in 216 (90.4%) after the cases were reviewed. Primary TMAs (thrombotic thrombocytopenic purpura or atypical hemolytic uremic syndrome) were diagnosed in 20 of 216 patients (9.3%). Typical HUS was diagnosed in eight patients (3.7%), and the most frequent secondary TMAs were HELLP syndrome (79/216, 36.6%) and active malignancies (30/219, 13.9%). ADAMTS13 measurements and alternative complement pathway analyses were performed in a minority of patients. Multiple factors identified as TMA triggers were present in most patients, in 55% of patients with primary TMA, vs. 44.7% of patients with secondary TMA (p = 0.377). Death occurred in 57 patients (23.4%) during follow-up, and dialysis was required in 51 patients (23.6%). Active malignancies [odds ratio (OR) 13.7], transplantation (OR 4.43), male sex (OR 2.89), and older age (OR 1.07) were significantly associated with death.Conclusion: Secondary TMAs represent many TMA causes in patients presenting a full TMA biological syndrome during routine clinical practice. Multiple factors favoring TMA are present in about half of primary or secondary TMA. ADAMTS13 and complement pathway were poorly explored in our cohort. The risk of death is particularly high in patients with malignancies as compared with patients with other TMA.

scholarly journals Histopathological features of thrombotic microangiopathies in renal biopsies

2019 ◽  
Vol 8 (3) ◽  
pp. 27-27
Author(s):  
Miguel Ernandes Neto ◽  
Lucas de Moraes Soler ◽  
Halita Vieira Gallindo Vasconcelos ◽  
Daniela Cristina dos Santos ◽  
Rosa Marlene Viero ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Shiga Toxin ◽  
Membranoproliferative Glomerulonephritis ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Thrombotic Microangiopathies ◽  
Cross Sectional ◽  
Histopathological Correlation ◽  
Uremic Syndrome

Background: Thrombotic microangiopathy (TMA) is a morphologic lesion characterized by thrombi occluding microvasculature related to endothelial injury. Objectives: This study aimed to assess the association between histopathological findings and etiology of TMA. Patients and Methods: This cross-sectional study comprised a sample of 34 patients who underwent renal biopsy and received an initial TMA diagnoses resulting in 29 definitive TMA cases. We evaluated the TMA features and clinical histopathological correlation. Results: The most frequent etiologies were atypical hemolytic uremic syndrome (aHUS) (n= 10; 34.5%), hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli (STECHUS) (n=6; 24.1%) and secondary causes of TMA (n= 12; 41.4%). We found the following histological features; patients with aHUS had thrombi in 60% of biopsies, membranoproliferative glomerulonephritis (MPGN)-like pattern in 20% and ischemia in 20%; patients with STEC-HUS had thrombi (14.3%), MPGN-like pattern (14.3%), endothelial swelling (14.3%) and ischemia (57.1%); patients with secondary etiologies had thrombi (58.3%), endothelial swelling (16.7%), ischemia (16.7%) and MPGN-like pattern (8.3%). Conclusions: The distribution of classic TMA findings was not related to etiology in spite of microthrombi having been found mostly in aHUS and secondary etiologies, whereas ischemia was found mainly in STEC-HUS. We did not find a histopathological pattern to each etiology of TMA.

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