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Endothelial dysfunction and hypercoagulability in severe sickle-cell acute chest syndrome
Etienne-Marie Jutant1, Guillaume Voiriot1,2, Vincent Labbé1,2
1Service de Médecine Intensive Réanimation, Département Médico-Universitaire APPROCHES, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
ERJ Open Research
|December 16, 2021
Summary
Sickle cell acute chest syndrome (ACS) involves endothelial dysfunction and hypercoagulability. Associated acute pulmonary hypertension (PH) in ACS patients shows a more pronounced pro-coagulant profile.
Area of Science:
- Hematology
- Pulmonology
- Critical Care Medicine
Background:
- Acute pulmonary hypertension (PH) is a severe complication of sickle cell acute chest syndrome (ACS) linked to increased mortality.
- The underlying mechanisms of PH in ACS are not well understood.
- This study investigates endothelial dysfunction and hypercoagulability in severe ACS, with and without acute PH.
Purpose of the Study:
- To determine the presence of endothelial dysfunction and hypercoagulability in severe sickle cell acute chest syndrome (ACS).
- To compare the biological profiles of ACS patients with and without acute pulmonary hypertension (PH).
- To assess these profiles against healthy controls and community-acquired pneumonia patients.
Main Methods:
- Prospective cohort follow-up study of adult sickle cell patients with ACS admitted to intensive care.
- Transthoracic echocardiography and measurement of coagulation and endothelial activation biomarkers.
- Comparison of biological profiles in ACS with acute PH versus without, and against steady-state and control groups.
Main Results:
- Acute PH was diagnosed in 19% of ACS patients.
- ACS patients with acute PH exhibited higher levels of erythrocyte- and platelet-derived microparticles and pro-coagulant activity.
- Compared to healthy controls, ACS patients showed elevated markers of coagulation and microparticle activation.
Conclusions:
- Severe sickle cell acute chest syndrome (ACS) is characterized by endothelial dysfunction and hypercoagulability.
- Patients with co-existing acute pulmonary hypertension (PH) display a significantly heightened pro-coagulant state.
- These findings highlight the critical role of coagulation pathways in ACS pathophysiology.
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