title: Unclassified bleeding disorders: outcome of haemostatic challenges following tranexamic acid and/or desmopressin.
abstract: BACKGROUND
  In a minority of patients with a significant bleeding history no cause is found despite extensive testing and we diagnose such cases as unclassified bleeding disorders (UBD). UBDs may have diverse underlying causes and currently no standard management strategy exists in the event of a haemorrhage or to cover surgery.
  
  
  OBJECTIVE
  To document the clinical characteristics and response to treatment of UBDs.
  
  
  METHODS
  We performed a retrospective chart review of all patients with UBDs who had an invasive procedure at our centre between 1998 and 2014.
  
  
  RESULTS
  The commonest symptoms were menorrhagia (89%) and bleeding at the time of surgery (88%) or dental extraction (85%). A total of 33 patients underwent 78 minor and major haemostatic challenges. Haemostatic cover was provided in 28 procedures with tranexamic acid alone, two with desmopressin and 45 with both agents in combination. A successful haemostatic outcome was observed in 70/78 (90%) cases. No patient required additional surgical intervention to achieve haemostasis, but one patient required a platelet transfusion to control postoperative bleeding.
  
  
  CONCLUSIONS
  This is the first study to report on the investigation and treatment of UBD. Future studies are needed to further our understanding of the bleeding phenotype and identify any underlying causes.
authors.affiliation: Department of Haematology, School of Medicine, Cardiff University, Cardiff, UK.
authors.forename: S
authors.initials: S
authors.lastname: Obaji
authors.affiliation: Arthur Bloom Haemophilia Centre, University Hospital of Wales, Cardiff, UK.
authors.forename: R
authors.initials: R
authors.lastname: Alikhan
authors.affiliation: Arthur Bloom Haemophilia Centre, University Hospital of Wales, Cardiff, UK.
authors.forename: R
authors.initials: R
authors.lastname: Rayment
authors.affiliation: Arthur Bloom Haemophilia Centre, University Hospital of Wales, Cardiff, UK.
authors.forename: P
authors.initials: P
authors.lastname: Carter
authors.affiliation: Arthur Bloom Haemophilia Centre, University Hospital of Wales, Cardiff, UK.
authors.forename: N
authors.initials: N
authors.lastname: Macartney
authors.affiliation: Arthur Bloom Haemophilia Centre, University Hospital of Wales, Cardiff, UK.
authors.forename: P
authors.initials: P
authors.lastname: Collins
keywords: bleeding score; desmopressin; tranexamic acid; unclassified bleeding disorders