Diagnosis and management of severe congenital protein C deficiency (SCPCD): Communication from the SSC of the ISTH

• Published in: Journal of thrombosis and haemostasis Vol. 20; no. 7; pp. 1735 - 1743
• Main 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
• Format: Journal Article
• Language: English
• Published: England Elsevier Limited 01-07-2022
• Subjects: Child; children; Clinical Medicine; Diagnosis; Disease management; Disseminated Intravascular Coagulation; Female; Hemostasis; Humans; Infant, Newborn; Klinisk medicin; Medical and Health Sciences; Medicin och hälsovetenskap; Neonates; Obstetrics, Gynecology and Reproductive Medicine; Pediatrics; Pregnancy; Protein C; Protein C Deficiency - diagnosis; Protein deficiency; purpura fulminans; Reproduktionsmedicin och gynekologi; Standardization; Thrombosis; Thrombosis - diagnosis; Thrombosis - therapy; thrombosis; neonates; purpura fulminans; children; disseminated intravascular coagulation
• ISSN: 1538-7933; 1538-7836; 1538-7836
• DOI: 10.1111/jth.15732

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.