Diagnosis and management of severe congenital protein C deficiency (SCPCD): Communication from the SSC of the ISTH. (15th May 2022)

• Record Type: Journal Article
• Authors: Minford, Adrian; Brandão, Leonardo R.; Othman, Maha; Male, Christoph; Abdul‐Kadir, Rezan; Monagle, Paul; Mumford, Andrew D.; Adcock, Dorothy; Dahlbäck, Björn; Miljic, Predrag; DeSancho, Maria T.; Teruya, Jun
• Is Part Of: Journal of thrombosis and haemostasis. Volume 20:Number 7(2022)
• Page Start: 1735
• Page End: 1743
• Publication Date: 2022-05-15
• Subjects: children -- disseminated intravascular coagulation -- neonates -- purpura fulminans -- thrombosis; Thrombosis -- Periodicals; Hemostasis -- Periodicals; Blood coagulation disorders -- Periodicals; 616.1
• DOI: 10.1111/jth.15732
• Languages: English
• ISSNs: 1538-7933

Abstract

Abstract: Severe 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.