Dataset Information

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

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.

SUBMITTER: Joseph A

PROVIDER: S-EPMC9115975 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

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

Joseph Adrien A Eloit Martin M Azoulay Elie E Kaplanski Gilles G Provot François F Presne Claire C Wynckel Alain A Grangé Steven S Rondeau Éric É Pène Frédéric F Delmas Yahsou Y Lautrette Alexandre A Barbet Christelle C Mousson Christiane C Coindre Jean-Philippe JP Perez Pierre P Jamme Matthieu M Augusto Jean-François JF Poullin Pascale P Jacobs Frédéric F El Karoui Khalil K Vigneau Cécile C Ulrich Marc M Kanouni Tarik T Le Quintrec Moglie M Hamidou Mohamed M Ville Simon S Charvet-Rumpler Anne A Ojeda-Uribe Mario M Godmer Pascal P Fremeaux-Bacchi Véronique V Veyradier Agnès A Halimi Jean-Michel JM Coppo Paul P

Research and practice in thrombosis and haemostasis 20220518 4

<h4>Background</h4>The prevalence, prognostic role, and diagnostic value of blood pressure in immune-mediated thrombotic thrombocytopenic purpura (iTTP) and other thrombotic microangiopathies (TMAs) remain unclear.<h4>Methods</h4>Using a national cohort of iTTP (<i>n</i> = 368), Shigatoxin-induced hemolytic uremic syndrome (<i>n</i> = 86), atypical hemolytic uremic syndrome (<i>n</i> = 84), and hypertension-related thrombotic microangiopathy (<i>n</i> = 25), we sought to compare the cohort's blo ...[more]

PMID: 35599703

Similar Datasets

Project description:BackgroundImmune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare disease characterized by arteriolar and capillary microthrombosis precipitating organ failure. However, the contribution of endothelial dysfunction on impaired microvascular blood flow in iTTP patients has been poorly explored. This pilot observational study aimed to explore endothelial-mediated vasoreactivity in iTTP patients at admission and its changes after plasma exchange therapy (PE).MethodsWe conducted a prospective observational study in patients (> 18-year old) admitted in ICU for iTTP. Using laser Doppler flowmetry and acetylcholine (Ach) iontophoresis in the forearm, we recorded the skin microvascular blood flow and the endothelium-mediated vasoreactivity at admission and after PE. Demographics, biological, clinical courses, and outcomes were also collected. As a control group, we used a previously published cohort of young diabetic patients after correction of ketoacidosis.ResultsEighteen confirmed iTTP patients and 34 controls were included in the study, mainly female (72%) aged 43 ± 16-year-old. At admission, 55% had neurological abnormalities, 50% cardiac issues and 27.8% an acute kidney injury. Median platelet count was 19 G/mL [10-37]. Baseline microvascular blood flow was decreased in iTTP patients when compared to controls (5.97 ± 4.5 vs. 10.1 ± 6.3 PU, P = 0.03), associated with markedly impaired endothelial-mediated skin microvascular reactivity (AUC: 9627 ± 8122 vs. 16,475 ± 11,738, P = 0.03). Microvascular reactivity improved after the first PE session (AUC: 9627 ± 8122 vs 16,558 ± 10,699, P = 0.007, respectively, baseline and post-PE1) and much more after the second session (26,431 ± 23,181, P = 0.04 post-PE1 vs post-PE2). Hemolysis biomarkers (LDH and bilirubin) negatively correlated with skin microvascular flow and vasoreactivity.ConclusionWe highlighted a marked yet reversible skin endothelium-mediated microvascular hyporeactivity in iTTP patients that could participate in organ injury pathophysiology.

Dataset Information

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

Background

Methods

Results

Conclusions

Publications

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

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets