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Published on: April 6, 2017
Suspected collagen disorders in the bleeding disorder clinic: a case-control study
S C Jackson1, L Odiaman, R T Card
1Division of Hematology, Department of Medicine, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada. sjackson@providencehematology.com
Summary
Symptomatic joint hypermobility (SJH) is significantly more common in patients with mucocutaneous bleeding disorders. This suggests collagen disorders are often unrecognized contributors to bleeding symptoms in these patients.
Area of Science:
- Hematology
- Genetics
- Rheumatology
Background:
- Disorders of collagen can cause mild bleeding due to impaired primary hemostasis and soft tissue fragility.
- The prevalence of collagen abnormalities contributing to mucocutaneous bleeding disorders is not well-established.
- Generalized symptomatic joint hypermobility (SJH) is a common feature of collagen disorders.
Purpose of the Study:
- To investigate the association between symptomatic joint hypermobility (SJH) and mucocutaneous bleeding disorders.
- To determine the prevalence of SJH and suspected collagen disorders in a bleeding disorder clinic population.
Main Methods:
- A case-control study was conducted with 55 patients from a bleeding disorder clinic and 50 matched controls.
- Participants were assessed for joint hypermobility using the Beighton score and revised Brighton criteria.
- Bleeding history was collected using the Condensed MCMDM1-VWD bleeding questionnaire, alongside laboratory hemostasis studies.
Main Results:
- SJH or a suspected collagen disorder was present in 24% of the bleeding disorder clinic patients, versus 2% in controls (OR 15).
- Of the SJH patients in the clinic, 77% had a personal or family history of connective tissue disorders like Ehlers-Danlos Syndrome or Osteogenesis Imperfecta.
- SJH was significantly associated with an increased likelihood of having an underlying mucocutaneous bleeding disorder.
Conclusions:
- Symptomatic joint hypermobility is strongly associated with mucocutaneous bleeding disorders.
- Collagen disorders are frequently unrecognized contributors to bleeding symptoms in patients presenting to bleeding disorder clinics.
- Screening for SJH may help identify underlying collagen disorders in patients with mucocutaneous bleeding issues.
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