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. 2022 May 18;6(4):e12702.
doi: 10.1002/rth2.12702. eCollection 2022 May.

Immune-mediated thrombotic thrombocytopenic purpura prognosis is affected by blood pressure

Adrien Joseph  1   2   3 , Martin Eloit  2   4 , Elie Azoulay  2   3 , Gilles Kaplanski  2   5 , François Provot  2   6 , Claire Presne  2   7 , Alain Wynckel  2   8 , Steven Grangé  2   9 , Éric Rondeau  2   10 , Frédéric Pène  2   11 , Yahsou Delmas  2   12 , Alexandre Lautrette  2   13 , Christelle Barbet  2   14 , Christiane Mousson  2   15 , Jean-Philippe Coindre  2   16 , Pierre Perez  2   17 , Matthieu Jamme  2   18 , Jean-François Augusto  2   19 , Pascale Poullin  2   20 , Frédéric Jacobs  2   21 , Khalil El Karoui  2   22 , Cécile Vigneau  2   23 , Marc Ulrich  2   24 , Tarik Kanouni  2   25 , Moglie Le Quintrec  2   26 , Mohamed Hamidou  2   27 , Simon Ville  2   28 , Anne Charvet-Rumpler  2   29 , Mario Ojeda-Uribe  2   30 , Pascal Godmer  2   31 , Véronique Fremeaux-Bacchi  2   32 , Agnès Veyradier  2   33 , Jean-Michel Halimi  2   14 , Paul Coppo  2   34
Affiliations

Immune-mediated thrombotic thrombocytopenic purpura prognosis is affected by blood pressure

Adrien Joseph et al. Res Pract Thromb Haemost. .

Abstract

Background: The prevalence, prognostic role, and diagnostic value of blood pressure in immune-mediated thrombotic thrombocytopenic purpura (iTTP) and other thrombotic microangiopathies (TMAs) remain unclear.

Methods: Using a national cohort of iTTP (n = 368), Shigatoxin-induced hemolytic uremic syndrome (n = 86), atypical hemolytic uremic syndrome (n = 84), and hypertension-related thrombotic microangiopathy (n = 25), we sought to compare the cohort's blood pressure profile to assess its impact on prognosis and diagnostic performances.

Results: Patients with iTTP had lower blood pressure than patients with other TMAs, systolic (130 [interquartile range (IQR) 118-143] vs 161 [IQR 142-180] mmHg) and diastolic (76 [IQR 69-83] vs 92 [IQR 79-105] mmHg, both p < 0.001). The best threshold for iTTP diagnosis corresponded to a systolic blood pressure <150 mmHg. iTTP patients presenting with hypertension had a significantly poorer survival (hazard ratio 1.80, 95% confidence interval 1.07-3.04), and this effect remained significant after multivariable adjustment (hazard ratio = 1.14, 95% confidence interval 1.00-1.30). Addition of a blood pressure criterion modestly improved the French clinical score to predict a severe A disintegrin and metalloprotease with thrombospondin type 1 deficiency in patients with an intermediate score (i.e., either platelet count <30 × 109/L or serum creatinine <200 µM).

Conclusions: Elevated blood pressure at admission affects the prognosis of iTTP patients and may help discriminate them from other TMA patients. Particular attention should be paid to blood pressure and its management in these patients.

Keywords: ADAMTS13; blood pressure; complement; hemolytic uremic syndrome; hypertension; prognosis; thrombotic microangiopathies; thrombotic thrombocytopenic purpura.

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Figures

FIGURE 1
FIGURE 1
(A) Systolic and diastolic blood pressure in patients with thrombotic microangiopathy syndromes. (B) Repartition of hypertension grades in patients with thrombotic microangiopathy syndromes. Blood pressure levels were compared using the Wilcoxon test. All patients from the study were included. Comparison iTTP/STEC‐HUS: <0.001/0.01. Abbreviations: aHUS, atypical hemolytic uremic syndrome; HT‐TMA, hypertension‐related thrombotic microangiopathy; iTTP, immune‐mediated thrombotic thrombocytopenic purpura; STEC‐HUS, Shigatoxin‐producing Escherichia coli–associated hemolytic uremic syndrome.
FIGURE 2
FIGURE 2
Kaplan–Meier curves for survival of patients with immune‐mediated thrombotic thrombocytopenic purpura with and without hypertension (systolic blood pressure ≥140 mmHg and/or diastolic blood pressure ≥90 mmHg) (A), and according to hypertension grade (grade 1 = blood pressure [140–160]/[90–100], grade 2 = blood pressure [160–180]/[100–110]. grade 3 = blood pressure ≥180/≥110 mmHg) (B). p Values were determined by the log‐rank test. Tables show the number of patients at risk in each group at baseline and at several time points
See this image and copyright information in PMC

References

    1. van den Born B‐JH, van der Hoeven NV, Groot E, et al. Association between thrombotic microangiopathy and reduced ADAMTS13 activity in malignant hypertension. Hypertension. 2008;51(4):862‐866. - PubMed
    1. Mathew RO, Nayer A, Asif A. The endothelium as the common denominator in malignant hypertension and thrombotic microangiopathy. J Am Soc Hypertens. 2016;10(4):352‐359. - PubMed
    1. Cavero T, Arjona E, Soto K, et al. Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome. Kidney Int. 2019;96(4):995‐1004. - PubMed
    1. El Karoui K, Boudhabhay I, Petitprez F, et al. Impact of hypertensive emergency and rare complement variants on the presentation and outcome of atypical hemolytic uremic syndrome. Haematologica. 2019;104(12):2501‐2511. - PMC - PubMed
    1. Goldberg RJ, Nakagawa T, Johnson RJ, Thurman JM. The role of endothelial cell injury in thrombotic microangiopathy. Am J Kidney Dis. 2010;56(6):1168‐1174. - PMC - PubMed