Citation
J Haem Pract 2016; 3(1):44-46. doi: 10.17225/jhp00064

Authors: Emma Fosbury, Raoul Blumberg, Ri Liesner, Keith Sibson

Emma Fosbury
Haematology Department, Royal Free Hospital London NHS Foundation Trust, Pond Street, London NW3 2QG, UK. Email: emma.fosbury@nhs.net

Raoul Blumberg
Paediatric Department, Whittington Hospital, Magdala Avenue, London N19 5NF, UK.

Ri Liesner
Haemophilia Centre, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London WC1N 3JH, UK.

Keith Sibson
Haemophilia Centre, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London WC1N 3JH, UK.

Abstract

Healthy, term neonates rarely encounter problems with bleeding, despite physiological differences in their levels of clotting factors, reflected in prolongation of the prothrombin time (PT), activated partial thromboplastin time (APTT) and thrombin time (TT). Their risk of bleeding, however, is significantly increased by the presence of a severe congenital bleeding disorder. Establishing such a diagnosis can present a particular challenge, given the rarity of these conditions and the difficulty in performing and interpreting laboratory assays in this age group. However, a delay in diagnosis and implementation of appropriate treatment can result in catastrophic sequelae. Therefore, the presentation of a healthy child at birth, whose condition rapidly deteriorates as a result of bleeding, should prompt the urgent investigation of a congenital haemostatic defect and involvement of expert haematological advice. We describe a very unusual presentation of a severe bleeding disorder in the first few days of life to highlight these issues.

Acknowledgements

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited

References

  1. Berntorp E, Windyga J. Treatment and prevention of acute bleedings in von Willebrand disease – efficacy and safety of Wilate, a new generation of von Willebrand factor / factor VIII concentrate. Haemophilia 2009; 15:122-130. doi: 10.1111/j.1365-2516.2008.01901.x.
  2. Chalmers EA. Neonatal coagulation problems. Arch Dis Child Fetal Neonatal Ed 2004; 89: F475-F478.
  3. Kujovich JL. Von Willebrand disease and pregnancy. J Thromb Haemost 2005; 3: 246-253.
  4. Richards M, Lavigne Lissalde G, Combescure C, et al. Neonatal bleeding in haemophilia: a European cohort study. Br J Haematol 2011; 156: 374-382. doi: 10.1111/j.1365-2141.2011.08967.x.
  5. Chalmers E, Williams M, Brennand J, et al. Guideline on the management of haemophilia in the fetus and neonate. Br J Haematol 2011; 154: 208-215. doi: 10.1111/j.1365-2141.2010.08545.x

The Journal of Haemophilia Practice is published by Haemnet.

Haemnet is a registered charity that brings together and gives a voice to haemophilia nurses, physiotherapists and allied health care professionals, providing forums for collaborative research, educational activities and support.