Research Interests

  • The clinical impact of inherited bleeding disorders including disease prevalence of quantitative assessments of bleeding
  • The use of bleeding scores as a screening tool for inherited bleeding disorders and to assess disease severity
  • The underlying pathophysiology of inherited bleeding disorders at a cellular and molecular level

 Current Models

  • BOEC (Blood Outgrowth Endothelial Cells)

 Active/Future Studies

  • The use of BOEC to understand angiodysplasia in von Willebrand disease (VWD) and to investigate potential novel treatments
  • A national study evaluating abnormal bleeding in female carriers of hemophilia
  • Identification of undiagnosed individuals affected with an inherited bleeding disorder using a social media campaign

Important Publications

Deforest M, Grabell J, Albert S, Young J, Tuttle A, Hopman WM, James PD. Generation and Optimization of the Self-Administered Bleeding Assessment Tool (Self-BAT) and its Validation as a Screening Test for von Willebrand Disease (VWD). Haemophilia  July 14, 2015 published as an Early View online; DOI:10.1111/hae.12747

Rydz N, Swystun L, Notley C, Paterson A, Riches J, Boonyarat B, Montgomery RR, James PD, Lillicrap D.  The C-type lectin receptor CLEC4M (L-SIGN) binds to, internalizes and contributes to the clearance of von WIllebrand factor and variation in plasma von Willebrand factor levels. Blood 2013 Jun 27; 121(26): 5228-37. PMID: 23529928       

 

Bowman M, Tuttle A, Notley C, Brown C, Tinlin S, Deforest M, Leggo J, Blanchette VS, Lillicrap D, James P for the Association of Hemophilia Clinic Directors of Canada.  The Genetics of Canadian Type 3 von WIllebrand Disease (VWD): Further Evidence for Co-dominant Inheritance of Type 3 VWD Alleles. J Thromb Haemost 2013 Mar; 11(3): 512-20. PMID 23311757

 

Bowman M, Mundell G, Grabell J, Hopman W, Rapson D, Lillicrap D, James P.  Generation and Validation of the Condensed MCMDM1-VWD Bleeding Questionnaire.  J Thromb Haemost 2008; 6: 2062-6. PMID: 18983516