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

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

Free article

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

Adrian Minford et al. J Thromb Haemost. 2022 Jul.

Free article

. 2022 Jul;20(7):1735-1743.

doi: 10.1111/jth.15732. Epub 2022 May 15.

Authors

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

Erratum in

Corrigendum.
[No authors listed] [No authors listed] J Thromb Haemost. 2022 Oct;20(10):2449. doi: 10.1111/jth.15865. J Thromb Haemost. 2022. PMID: 36123578 No abstract available.
Corrigendum to 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].
Minford A, Brandão LR, Othman M, Male C, Abdul-Kadir R, Monagle P, Mumford AD, Adcock D, Dahlbäck B, Miljic P, DeSancho MT, Teruya J. Minford A, et al. J Thromb Haemost. 2023 Apr;21(4):1069. doi: 10.1016/j.jtha.2023.01.019. Epub 2023 Jan 31. J Thromb Haemost. 2023. PMID: 36732161 No abstract available.

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.

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References

REFERENCES

1. Goldenberg NA, Manco-Johnson MJ. Protein C deficiency. Haemophilia. 2008;14(6):1214-1221.
1. Marlar RA, Gausman JN. Laboratory testing issues for protein C, protein S, and antithrombin. Int J Lab Hematol. 2014;36(3):289-295.
1. Monagle P, Cuello CA, Augustine C, et al. American society of hematology 2018 guidelines for management of venous thromboembolism: treatment of pediatric venous thromboembolism. Blood Adv. 2018;2(22):3292-3316.
1. Manco-Johnson MJ, Bomgaars L, Palascak J, et al. Efficacy and safety of protein C concentrate to treat purpura fulminans and thromboembolic events in severe congenital protein C deficiency. Thromb Haemost. 2016;116(1):58-68.
1. Dreyfus M, Ladouzi A, Chambost H, et al. Treatment of inherited protein C deficiency by replacement therapy with the French purified plasma-derived protein C concentrate (PROTEXEL). Vox Sang. 2007;93(3):233-240. doi:10.1111/j.1423-0410.2007.00953.x

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

Affiliations

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

Authors

Affiliations

Erratum in

Abstract

References

REFERENCES

MeSH terms

LinkOut - more resources

Full Text Sources

Medical