Minford, Adrian (6602893631)Adrian (6602893631)MinfordBrandão, Leonardo R. (12788818600)Leonardo R. (12788818600)BrandãoOthman, Maha (7006122614)Maha (7006122614)OthmanMale, Christoph (7003604062)Christoph (7003604062)MaleAbdul-Kadir, Rezan (7004016849)Rezan (7004016849)Abdul-KadirMonagle, Paul (7004308240)Paul (7004308240)MonagleMumford, Andrew D. (7003755145)Andrew D. (7003755145)MumfordAdcock, Dorothy (7003902745)Dorothy (7003902745)AdcockDahlbäck, Björn (7103328699)Björn (7103328699)DahlbäckMiljic, Predrag (6604038486)Predrag (6604038486)MiljicDeSancho, Maria T. (11138767900)Maria T. (11138767900)DeSanchoTeruya, Jun (8679464900)Jun (8679464900)Teruya2025-06-122025-06-122022https://doi.org/10.1111/jth.15732https://www.scopus.com/inward/record.uri?eid=2-s2.0-85132647530&doi=10.1111%2fjth.15732&partnerID=40&md5=e6ac2ccf619a1a35c998ae5a4320155fhttps://remedy.med.bg.ac.rs/handle/123456789/3490Severe congenital protein C deficiency (SCPCD) is rare and there is currently substantial variation in the management of this condition. A joint project by three Scientific and Standardization Committees of the ISTH: Plasma Coagulation Inhibitors, Pediatric/Neonatal Thrombosis and Hemostasis, and Women’s Health Issues in Thrombosis and Hemostasis, was developed to review the current evidence and help guide on diagnosis and management of SCPCD. We provide a summary of the clinical presentations, differential diagnoses, appropriate investigations to confirm the diagnosis, approaches for management of the acute situation, and options for long-term management including subsequent pregnancies. We finally provide a set of recommendations to help in this regard. © 2022 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis.childrendisseminated intravascular coagulationneonatespurpura fulminansthrombosis