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

Adrian Minford; Leonardo R Brandão; Maha Othman; Christoph Male; Rezan Abdul-Kadir; Paul Monagle; Andrew D Mumford; Dorothy Adcock; Björn Dahlbäck; Predrag Miljic; Maria T DeSancho; Jun Teruya

doi:10.1111/jth.15732

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

J Thromb Haemost. 2022 Jul;20(7):1735-1743. doi: 10.1111/jth.15732. Epub 2022 May 15.

Authors

Adrian Minford¹, Leonardo R Brandão^{2

3}, Maha Othman^{4

5}, Christoph Male⁶, Rezan Abdul-Kadir^{7

8}, Paul Monagle⁹, Andrew D Mumford¹⁰, Dorothy Adcock¹¹, Björn Dahlbäck¹², Predrag Miljic¹³, Maria T DeSancho¹⁴, Jun Teruya¹⁵

Affiliations

¹ Department of Paediatrics, Bradford Royal Infirmary, West Yorkshire, UK.
² The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
³ Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada.
⁴ School of Medicine, Queen's University, Kingston, ON, Canada.
⁵ School of Baccalaureate Nursing, St. Lawrence College, Kingston, ON, Canada.
⁶ Department of Paediatrics, Medical University of Vienna, Vienna, Austria.
⁷ Katharine Dormandy Haemophilia and Thrombosis Centre and Department of Obstetrics and Gynaecology, The Royal Free NHS Foundation Hospital, London, London, UK.
⁸ Institute for Women's Health, University College London, London, UK.
⁹ Kids Cancer Centre, Department of Haematology, Sydney Children's Hospital, Royal Children's Hospital Melbourne, Murdoch Children's Research Institute and University of Melbourne, Melbourne, Victoria, Australia.
¹⁰ School of Cellular and Molecular Medicine, University of Bristol, Bristol, UK.
¹¹ Laboratory Corporation of America, Burlington, North Carolina, USA.
¹² Department of Translational Medicine, Lund University, Malmö, Sweden.
¹³ Faculty of Medicine, Clinic of Hematology, Clinical Center of Serbia, University in Belgrade, Belgrade, Serbia.
¹⁴ Division of Hematology-Oncology, Department of Medicine, Weill Cornell Medicine, New York Presbyterian Hospital, New York, New York, USA.
¹⁵ Departments of Pathology & Immunology, Pediatrics, and Medicine, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas, USA.

PMID: 35570324
DOI: 10.1111/jth.15732

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.

Keywords: children; disseminated intravascular coagulation; neonates; purpura fulminans; thrombosis.

MeSH terms

Child
Disseminated Intravascular Coagulation*
Female
Hemostasis
Humans
Infant, Newborn
Pregnancy
Protein C Deficiency* / diagnosis
Thrombosis* / diagnosis
Thrombosis* / therapy