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Rationale: Acute pulmonary hypertension (PH) may develop during sickle-cell acute chest syndrome (ACS), and is associated with an increased mortality. Its mechanisms remain poorly known. We questioned whether there is endothelial dysfunction and hypercoagulability in severe ACS, with and without acute PH., Methods: In a prospective monocentre cohort follow-up study, all sickle-cell adult patients with ACS admitted to the intensive care unit underwent transthoracic echocardiography and measurement of biomarkers of coagulation, endothelial activation and platelet and erythrocyte activation. Acute PH was defined as a high echocardiographic probability of PH. The biological profiles of sickle-cell patients were analysed at the time of ACS, contrasting with the existence of acute PH, and compared with steady-state and with non-sickle-cell controls (healthy subjects and community-acquired pneumonia)., Results: Most patients (36 patients with 39 ACS episodes; 23 males; median age 27 years) had thoracic pain, dyspnoea and computed tomography scan lung consolidation. Acute PH was diagnosed in seven (19%) patients. Erythrocyte- and platelet-derived microparticles and the pro-coagulant activity of microparticles were higher in ACS patients with acute PH, compared with their counterparts. Compared with healthy controls, ACS patients had higher levels of tissue factor, fibrin monomers, D-dimer, release of pro-coagulant microparticles and erythrocyte- and platelet-derived microparticles. Compared with community-acquired pneumonia patients, ACS patients had increased levels of fibrin monomers and erythrocyte- and platelet-derived microparticles., Conclusions: Severe ACS is characterised by endothelial dysfunction and hypercoagulability, with a marked pro-coagulant profile in cases of associated PH., Competing Interests: Conflict of interest: E-M. Jutant has nothing to disclose. Conflict of interest: G. Voiriot reports grants and personal fees from Biomérieux, and grants from Janssen and SOS Oxygene, outside the submitted work. Conflict of interest: V. Labbé reports personal fees from AMOMED outside the submitted work. Conflict of interest: L. Savale reports personal fees and nonfinancial support from Actelion and MSD, and grants, personal fees and nonfinancial support from GSK, outside the submitted work. Conflict of interest: H. Mokrani has nothing to disclose. Conflict of interest: P. Van Dreden has nothing to disclose. Conflict of interest: G. Gerotziafas has nothing to disclose. Conflict of interest: M. Fartoukh reports grants from BioMerieux and personal fees from Pfizer, outside the submitted work., (Copyright ©The authors 2021.)