An 18-year-old man with a history of type 3 von Willebrand disease (VWD) presented with a spontaneous pyohaemothorax. Type 3 VWD may present with both mucocutaneous and deep-seated bleeds, such as visceral haemorrhages, intracranial bleeds and haemarthrosis. There have been very few cases described in children of spontaneous pyohaemothorax. Management of this patient was challenging due to risks of bleeding following surgical drainage, requiring constant replacement with von Willebrand factor concentrate, while monitoring factor VIII levels to balance the risks of thrombosis.
- haematology (incl blood transfusion)
- paediatrics (drugs and medicines)
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Contributors VDN coauthored this article with AS, LB and VCR. There are no prior publications or submissions with any overlapping information. The idea for the article was conceived by all four authors. VDN did the literature search. VDN wrote the initial draft and the coauthors revised the complete draft multiple times. As the first and corresponding author, VDN will be the guarantor and responsible for all further communications. The case was managed by all the authors in different roles.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer-reviewed.