PUBLICATIONS

2016

Albánez S, Ogiwara K, Michels A, Hopman W, Grabell J, James P, Lillicrap D.
Aging and ABO blood type influence von Willebrand factor and factor VIII levels through interrelated mechanisms.
J Thromb Haemost. 2016 May;14(5):953-63.

Bowman M, James P.
Bleeding scores for the diagnosis of von Willebrand disease.
Accepted by Seminars In Thrombosis and Hemostasis June 2016.

Flood VH, Christopherson PA, Gill JC, Friedman KD, Haberichter SL, Bellissimo DB, Udani RA, Dasgupta M, Hoffmann RG, Ragni MV, Shapiro AD, Lusher JM, Lentz SR, Abshire TC, Leissinger C, Hoots WK, Manco-Johnson MJ, Gruppo RA, Boggio LN, Montgomery KT, Goodeve AC, James PD, Lillicrap D, Peake IR, Montgomery RR.
Clinical and laboratory variability in a cohort of patients diagnosed with type 1 VWD in the United States.
Blood. 2016 May 19;127(20):2481-8.

Georgescu MT, Lai JD, Hough C, Lillicrap D.
War and peace: Factor VIII and the adaptive immune response.
Cell Immunol. 2016;301:2-7.

Hawke L, Grabell J, Sim W, Thibeault L, Muir E, Hopman W, James P.
Obstetric bleeding among women with inherited bleeding disorders: a retrospective study.
Accepted by Haemophilia July 2016

Hawke L, Bowman M, Poon M-C, Scully M-F, Rivard G.E, James P.D.
Characterization of aberrant splicing of von Willebrand factor (VWF) in von Willebrand disease (VWD): an under- recognized mechanism.
Blood 2016; 128:584-593.

James P.
VWD Diagnosis: Improved.
Blood. 2016;127(20):2372-3.

Krishnamoorthy S, Liu T, Drager D, Patarroyo-White S, Chhabra ES, Peters R, Josephson N, Lillicrap D, Blumberg RS, Pierce GF, Jiang H.
Recombinant factor VIII Fc (rFVIIIFc) fusion protein reduces immunogenicity and induces tolerance in hemophilia A mice.
Cell Immunol. 2016 Mar;301:30-9.

Lai JD, Moorehead PC, Sponagle K, Steinitz KN, Reipert BM, Hough C, Lillicrap D.
Concurrent influenza vaccination reduces anti-FVIII antibody responses in murine hemophilia A.
Blood. 2016;127(26):3439-49.

Lai JD, Georgescu MT, Hough C, Lillicrap D.
To clear or to fear: An innate perspective on factor VIII immunity.
Cell Immunol. 2016 Mar;301:82-9.

Nichols TC, Hough C, Agersø H, Ezban M, Lillicrap D.
Canine models of inherited bleeding disorders in the development of coagulation assays, novel protein replacement and gene therapies.
J Thromb Haemost. 2016;14(5):894-905.

Noone DG, Riedl M, Pluthero FG, Bowman ML, Liszewski MK, Lu L, Quan Y, Balgobin S, Schneppenheim S, Schneppenheim R, Budde U, James PD, Atkinson JP, Palaniyar N, Kahr WHA and Licht C.
Von Willebrand Factor regulates complement on endothelial cells.
Kidney Int. 2016;90:123-134.

Othman M, Kaur H, Favaloro EJ, Lillicrap D, Di Paola J, Harrison P, Gresele P; Subcommittees on von Willebrand Disease and Platelet Physiology.
Platelet type von Willebrand disease and registry report: communication from the SSC of the ISTH.
J Thromb Haemost. 2016 Feb;14(2):411-4.

Reynen E, James P.
Von Willebrand Disease and Pregnancy: A Review of Evidence and Expert Opinion.
Accepted by Seminars in Thrombosis and Hemostasis June 2016.

Shima M, Lillicrap D, Kruse-Jarres R.
Alternative therapies for the management of inhibitors.Haemophilia.
2016 Jul;22 Suppl 5:36-41. doi: 0.1111/hae.13005.

Swystun LL, Lillicrap D.
How much do we really know about von Willebrand disease?
Curr Opin Hematol. 2016 Jul 15. [Epub ahead of print]

Swystun LL, Lillicrap D.
Gene Therapy for Coagulation Disorders.
Circ Res. 2016 Apr 29;118(9):1443-52.

Xu Y, Deforest M, Grabell J, Hopman W, James P.
Relative Contributions of Bleeding Scores and Iron Status on Health-related Quality of Life in von Willebrand Disease.
Accepted by Haemophilia July 2016.


2015

Avila M, Lee J, Bouskill V, Rand M, James P, Carcao M.
Acquired von Willebrand Syndrome in Pediatric Patients with Congenital Heart Disease: Challenges in the Diagnosis and Management of this Rare Condition.
Haemophilia. 2015 Jan;21(1):e89-92. PMID: 25495773

Crawford B, Ozelo MC, Ogiwara K, Ahlin J, Albanez S, Hegadorn C, Harpell L, Hough C, Lillicrap D.
Transgene-host cell interactions mediate significant influences on the production, stability, and function of recombinant canine FVIII.
Mol Ther Methods Clin Dev. 18;2: 15033, 2015

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).
Hemophilia. 2015 Sep; 21(5): 384-8. PMID:26179127

Fogarty P, Mancuso ME, Kasthuri R, Bidlingmaier C, Chitlur M, Gomez K, Holme P, James P, Kruse-Jarres R, Mahlangu J, Mingot-Castellano ME, Soni A.
Presentation and Management of Acute Coronary Syndromes Among Adult Persons with Haemophilia: Results of an International, Retrospective, 10-year Survey.
Haemophilia. 2015 Sep; 21(5): 589-97. PMID: 25689278

Krishnamoorthy S, Liu T, Drager D, Patarroyo-White S, Chhabra ES, Peters R, Josephson N, Lillicrap D, Blumberg RS, Pierce GF, Jiang H.
Recombinant factor VIII Fc (rFVIIIFc) fusion protein reduces immunogenicity and induces tolerance in hemophilia A mice.
Cell Immunol. Dec 29. Epub ahead of print, 2015.

Lillicrap D.
Introduction to a series of reviews on inherited bleeding disorders.
Blood. 125:2011, 2015

Lillicrap D, Schiviz A, Apostol C, Wojciechowski P, Horling F, Lai CK, Piskernik C, Hoellriegl W, Lollar P.
Porcine recombinant factor VIII (Obizur; OBI-1; BAX801): product characteristics and preclinical profile.
Haemophilia. Aug 17, Epub ahead of print, 2015

Mittal N, Naridze R, James P, Shott S, Valentino LA.
Utility of a Paediatric Bleeding Questionnaire as a screening tool for von Willebrand disease in apparently healthy children.
Haemophilia. 2015 May 16. doi:10.1111/hae.12689. [Epub ahead of print]. PMID: 25982122

Moorehead PC, Thibeault L, Tuttle A, Grabell J, Dwyre L, Silva M, James P, Lillicrap D.
Rapid Acquisition of Immunologic Tolerance to Factor VIII and Disappearance of Anti-Factor VIII IgG4 After Prophylactic Therapy in a Hemophilia A Patient With High-titer Factor VIII Inhibitor.
J Pediatr Hematol Oncol. 2015 May; 37(4): e220-2. PMID: 25411864

Oldenburg J, Lacroix-Desmazes S, Lillicrap D.
Alloantibodies to therapeutic factor VIII in hemophilia A: the role of von Willebrand factor in regulating factor VIII immunogenicity.
Haematologica. 100(2):149-156, 2015

Rydz N, Grabell J, Lillicrap D, James PD.
Changes in von Willebrand factor level and activity with age in Type 1 von Willebrandllebrand Disease.
Haemophilia. 2015 Sep; 21(5): 636-41. PMID: 25756206

Swystun LL, James P.
Using genetic diagnostics in hemophilia and von Willebrand disease.
Hematology Am Soc Hematol Educ Program. 2015 Dec 5;2015(1):152-9.


2014

Chitlur M, Rivard GE, Lillicrap D, Mann K, Shima M, Young G; Factor VIII, Factor IX, and Rare Coagulation Disorders Subcommittee of the Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis.
Recommendations for performing thromboelastography/thromboelastometry in hemophilia: communication from the SSC of the ISTH.
J Thromb Haemost. 12(1):103-6. 2014

Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Clark D, Mauer A, Bowman M, Riddel J, Christopherson P, Montgomery R, Zimmerman Program Investigators, Rand M, Coller B, James PD.
Normal Range of Bleeding Scores for the ISTH-BAT: Adult and Pediatric Data from The Merging Project.
Haemophilia 2014 Nov;20(6): 831-5. PMID: 25196510

Holden R, Booth S, Tuttle A, James P, Morton A, Hopman W, Nolan R, Garland J.
Sequence variation in vitamin K epoxide reductase gene is associated with survival and progressive coronary calcification in chronic kidney disease.
Arterioscler Thromb Vasc Biol 2014 July; 34(7):1591-6. PMID: 24855061

High KH, Nathwani A, Spencer T, Lillicrap D.
Current status of haemophilia gene therapy.
Haemophilia. 20 Suppl 4:43-9. 2014

Lee A, Sinclair G, Valentine K, James P, Poon M-C.
Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status.
Blood 2014 Jul 31; 124(5):e1-3. PMID: 24951428

James P, Salomon O, Mikovic D, Peyvandi F.
Rare bleeding disorders-bleeding assessment tools, laboratory aspects and phenotype and therapy of FXI deficiency.
Haemophilia 2014 May; 20 (Suppl 4):71-5. PMID: 24762279

James P.
Can bleeding score predict the risk of future bleeding? No BS here!
Blood 2014 Jun 26; 123(26):4008-9. PMID: 24970927

Kaur H, Ozela M, Scovil S, James PD, Othman M.
Systematic analysis of bleeding phenotype in PT-VWD compared to type 2B VWD using an electronic bleeding questionnaire.
Clin Appl Thromb Hemost 2014 Nov;20(8):765-71. doi:10.1177/1076029614543825. Epub 2014 Jul 25. PMID: 25063765

Lillicrap D, Fijnvandraat K, Santagostino E.
Inhibitors - genetic and environmental factors.
Haemophilia. 20 Suppl 4:87-93. 2014

Lillicrap D.
Syntaxin-binding protein 5 exocytosis regulation: differential role in endothelial cells and platelets.
J Clin Invest. 124(10):4231-3. 2014

Maceachern K, Kaur H, Toukh M, Mumal I, Hamilton A, Scovil S, James P, Elbatarny HS, Othman M.
Comprehensive Evaluation of Hemostasis in Normal Women: Impact on the Diagnosis of Mild Bleeding Disorders.
Clin Appl Thromb Hemost. 2015 Jan;21(1):72-81. PMID: 24715368

Matino D, Lillicrap D, Astermark J, Dolan G, Kessler C, Lambert T, Makris M, O'Donnell J, Pipe S, Santagostino E, Saint-Remy JM, Schramm W, Iorio A.
Switching clotting factor concentrates: considerations in estimating the risk of immunogenicity.
Haemophilia. 20(2):200-6. 2014

Ozelo MC, Vidal B, Brown C, Notley C, Hegadorn C, Webster S, Harpell L, Ahlin J, Winterborn A, Handforth J, Arruda VR, Hough C, Lillicrap D.
Omental implantation of BOECs in hemophilia dogs results in circulating FVIII antigen and a complex immune response.
Blood. 123(26):4045-53. 2014

Shapiro AD, Ragni MV, Kulkarni R, Oldenberg J, Srivastava A, Quon DV, Pasi KJ, Hanabusa H, Pabinger I, Mahlangu J, Fogarty P, Lillicrap D, Kulke S, Potts J, Neelakantan S, Nestorov I, Li S, Dumont JA, Jiang H, Brennan A, Pierce GF.
Recombinant factor VIII Fc fusion protein: extended-interval dosing maintains low bleeding rates and correlates with von Willebrand factor levels.
J Thromb Haemost. 12:1788-800, 2014

Shida Y, Rydz N, Stegner D, Brown C, Mewburn J, Sponagle K, Danisment O, Crawford B, Vidal B, Hegadorn CA, Pruss CM, Nieswandt B, Lillicrap D.
Analysis of the role of von Willebrand factor, platelet glycoprotein VI-, and α2β1-mediated collagen binding in thrombus formation.
Blood. 124(11):1799-807, 2014

Swystun LL, Lillicrap D.
FVIII stabilization: VWF D'D3 will do.
Blood. 124(3):313-5. 2014

Traore AN, Chan AK, Webert KE, Heddle N, Ritchie B, St-Louis J, Teitel J, Lillicrap D, Iorio A, Walker I.
First analysis of 10-year trends in national factor concentrates usage in haemophilia: data from CHARMS, the Canadian Hemophilia Assessment and Resource Management System.
Haemophilia. 20(4):e251-9. 2014

Vézina C, Carcao M, Infante-Rivard C, Lillicrap D, Stain AM, Paradis E, Teitel J, Rivard GE; the Association of Hemophilia Clinic Directors of Canada and of the Canadian Association of Nurses in Hemophilia Care.
Incidence and risk factors for inhibitor development in previously untreated severe haemophilia A patients born between 2005 and 2010.
Haemophilia. J Clin Invest. 124(10):4231-3. 2014


2013

Berntorp E, Fuchs B, Makris M, Montgomery R, Flood V, O'Donnell JS, Federici AB, Lillicrap D, James P, Budde U, Morfini M, Petrini P, Austin S, Kannicht C, Jiménez-Yuste V, Lee C.
Third Åland islands conference on von Willebrand disease, 26-28 September 2012: meeting report.
Haemophilia. 19 Suppl 3:1-18. 2013.

Bowman M, Tuttle A, Notley C, Brown C, Tinlin S, Deforest M, Leggo J, Blanchette VS, Lillicrap D, James P;
Association of Hemophilia Clinic Directors of Canada.
The genetics of Canadian type 3 von Willebrand disease: further evidence for co-dominant inheritance of mutant alleles.
J Thromb Haemost. 11(3):512-20, 2013.

Golder M, Mewburn J, Lillicrap D.
In vitro and in vivo evaluation of the effect of elevated factor VIII on the thrombogenic process.
Thromb Haemost 109(1):53-60, 2013.

Holden R, Tuttle A, Burbidge T, Hegadorn C, Grabell J and James PD.
Quantitative and qualitative changes of von Willebrand factor and their impact on mortality in patients with end stage kidney disease.
Accepted by Blood, Coagulation & Fibrinolysis, March 2013

James PD, Lillicrap D.
The molecular characterization of von Willebrand disease: good in parts.
Br J Haematol. 161(2):166-76, 2013.

James PD, Lillicrap D, Mannucci PM.
Alloantibodies in von Willebrand Disease.
Blood. Jan 7. [Epub ahead of print, 2013.]

James P.
Bleeding assessment tools.
Japanese Journal of Thrombosis and Hemostasis 01/2013; 24 (6):670-674.

Kahlon A, Grabell J, Tuttle A, Engen D, Hopman W, Lillicrap D, James P.
Quantification of perioperative changes in von Willebrand factor and factor VIII during elective orthopaedic surgery in normal individuals.
Haemophilia May 28 [Epub ahead of print, 2013.]

Lewis KB, Hughes RJ, Epstein MS, Josephson NC, Kempton CL, Kessler CM, Key NS, Howard TE, Kruse-Jarres R, Lusher JM, Walsh CE, Watts RG, Ettinger RA, Pratt KP; PATH (Personalized Alternative Therapies for Haemophilia) Study Investigators.
Phenotypes of allo- and autoimmune antibody responses to FVIII characterized by surface plasmon resonance.
PLoS One. 8;8(5):e61120. 2013.

Lillicrap,D.
Molecular testing for disorders of hemostasis.
Int J Lab Hematol. 35(3):290-6, 2013.

Lillicrap D.
The future of hemostasis management.
Ped Blood & Cancer 60 Suppl 1, S44-7, 2013.

Lillicrap D.
von Willebrand disease: advances in pathogenetic understanding, diagnosis, and therapy.
Hematology Am Soc Hematol Educ Program. 2013:254-60. 2013.

Lillicrap D.
Translational medicine advances in von Willebrand disease.
J Thromb Haemost. 11 Suppl 1:75-83. 2013.

Lillicrap D.
Introduction to a series of reviews on cancer-associated thrombotic disease. Blood.
5;122(10):1687-8. 2013.

Pandey GS, Yanover C, Miller-Jenkins LM, Garfield S, Cole SA, Curran JE, Moses EK, Rydz N, Simhadri V, Kimchi-Sarfaty C, Lillicrap D, Viel KR, Przytycka TM, Pierce GF, Howard TE, Sauna ZE; PATH (Personalized Alternative Therapies for Hemophilia) Study Investigators.
Endogenous factor VIII synthesis from the intron 22-inverted F8 locus may modulate the immunogenicity of replacement therapy for hemophilia A.
Nat Med. 19(10):1318-24. 2013.

Peyvandi F, Kunicki T, Lillicrap D.
Genetic sequence analysis of inherited bleeding diseases.
Blood. 122(20):3423-31. 2013.

Rae C, Furlong W, Horsman J, Pullenayegum E, Demers C, St-Louis J, Lillicrap D, Barr R.
Bleeding disorders, menorrhagia and iron deficiency: impacts on health-related quality of life.
Haemophilia 19 (3):385-391. 2013

Rimmer E, Jamieson MA, James PD.
Malposition and Expulsion of the Levonorgestrel Intrauterine System Among Women with Inherited Bleeding Disorders.
Haemophilia 2013 [Epub ahead of print] DOI: 10.1111/hae.12184.

Rydz N, Swystun LL, Notley C, Paterson AD, Riches JJ, Sponagle K, Boonyawat B, Montgomery RR, James PD, Lillicrap D.
The C-type lectin receptor CLEC4M binds, internalizes and clears von Willebrand factor and contributes to the variation in plasma von Willebrand factor levels.
Blood. Mar 25. [Epub ahead of print, 2013.]

Sim Yan, Bowman M, Hopman W, Engen D, Silva M and James PD.
Predicting Operative Bleeding in Elective Pediatric Surgeries using the Pediatric Bleeding Questionnaire (PBQ).
Accepted by J Ped Hematol Oncol May 2013.

Tatsumi K, Sugimoto M, Lillicrap D, Shima M, Ohashi K, Okano T, Matsui H.
A Novel Cell-Sheet Technology That Achieves Durable Factor VIII Delivery in a Mouse Model of Hemophilia A.
PLoS One. 16;8(12):e83280. 2013.


2012

Webert KE, Rivard GE, Teitel J, Carcao M, Lillicrap D, St-Louis J, Walker IR.
Low prevalence of inhibitor antibodies in the Canadian haemophilia population.
Haemophilia 18(3):e254-9, 2012.

Foley JH, Petersen KU, Rea CJ, Harpell L, Powell S, Lillicrap D, Nesheim ME, Sorensen B.
Solulin increases clot stability in whole blood from humans and dogs with hemophilia.
Blood, 119(15):3622-8, 2012.

Pruss CM, Golder M, Bryant A, Hegadorn C, Haberichter S, Lillicrap D.
Use of a mouse model to elucidate the phenotypic effects of the von Willebrand factor cleavage mutants, Y1605A/M1606A and R1597W. J Thromb Haemost 10(5):940-50, 2012.

James PD, Lillicrap D.
von Willebrand disease: clinical and laboratory lessons learned from the large von Willebrand disease studies.
Am J Hematol 87 Suppl 1, S4-11, 2012.

Lillicrap D.
The World Federation of Hemophilia and Research.
Haemophilia 18 Suppl 4, 24-7, 2012.

Blomback M, Eikenboom J, Lane D, Denis C, Lillicrap D.
von Willebrand disease biology.
Haemophilia 18 Suppl 4, 141-7, 2012.

Berntorp E, Peake I, Budde U, Laffan M, Montgomery R, Windyga J, Goodeve A, Petrini P, von Depka M, Miesbach W, Lillicrap D, Federici AB, Lassila R, White G.
von Willebrand's disease: A report from a meeting in the Aland islands.
Haemophilia 18 Suppl 6, 1-13, 2012.

Rae C, Furlong W, Horsman J, Pullenayegum E, Demers C, St-Louis J, Lillicrap D, Barr R.
Bleeding disorders, menorrhagia and iron deficiency: impacts on health-related quality of life.
Haemophilia Epub ahead of print Sept 21 2012.

Lillicrap D.
A complex substitute: antibody therapy for hemophilia.
Nature Med 18(10):1460-1, 2012.

James PD, Kasthuri R, Kruse-Jarres R, Soni A, Kulkarni R, Dolan G. Global Emerging HEmophilia Panel (GEHEP):
A Multinational Collaboration for Advancing Hemophilia Research and Treatment.
Accepted for publication in Transfusion Medicine and Hemotherapy, December 20, 2012.

Rydz N and James PD.
The Evolution and Value of Bleeding Assessment Tools.
J Thromb Haemost 2012: 10: 2223-2229. PMID: 22974079.

Rydz N and James PD.
Approach to the diagnosis and management of common bleeding disorders.
Sem Thromb Haemost 2012; 38: 711-719. PMID: 22941792

James PD and Coller B.
Phenotyping Bleeding.
Current Opinion in Hematology 2012, 19:406-412. PMID: 22759628

Rydz N and James PD.
Why is my patient bleeding or bruising?
Hematol Oncol Clin N Am 2012 (26); 321-344.

Federici AB, James PD. 
Current management of patients with severe von Willebrand disease type 3: a 2012 update. 
Accepted by Haematologica, February 2012.

Sanders S, Purcell S, Silva M, Palerme S, James PD. 
Relationship between diagnosis and intervention in women with inherited bleeding disorders and menorrhagia. 
Haemophilia, 2012 Jan 5, doi: 10.1111/j.1365-2516.2011.02740.x Epub ahead of print PMID: 22221914

2011

Coutu, DL, Cuerquis J, El Ayoubi R, Forner KA, Roy R, François M, Griffith M, Lillicrap, D, Yousefi AM, Blostein MD, Galipeau J.
Hierarchial scaffold design for mesenchymal stem cell-based gene therapy of hemophilia B.
Biomaterials 32:295-305, 2011.

Darwish, H, Mundell G, Engen D, Lillicrap D, Silva M, James P.
Early intraoperative blood collection does not affect complete blood counts, von Willebrand factor or factor VIII levels in normal children.
J Pediatr Hematol Oncol 33(1):47-9, 2011.

Qadura M, Waters B, Burnett E, Chegeni R, Hough C, Othman M, Lillicrap D.
Immunoglobulin isotypes and functional anti-FVIII antibodies in response to FVIII treatment in Balb/c and C57BL/6 haemophilia A mice.
Haemophilia 17(2):288-95, 2011.

Hamilton A, Ozelo M, Leggo J, Notley C, Brown H, Frontroth JP, Angelillo-Scherrar A, Baghaei F, Enayat SM, Favaloro, E., Lillicrap D, Othman M.
Frequency of platelet type versus type 2B von Willebrand disease. An International registry-based study.
Thromb Haemost 105(3):501-8, 2011.

Chegeni R, Vickars L, Favaloro EJ, Lillicrap D, Othman M.
Functional analysis of three recombinant A-VWF domain mutants in comparison to wild-type and plasma-derived VWF facilitates subtyping in type 2 von Willebrand disease.
Thromb Res 127(2):161-6, 2011.

Matsui H, Hegadorn C, Ozelo M, Burnett E, Tuttle A, Labelle A, McCray PB Jr., Naldini L, Brown B, Hough C, Lillicrap D.
A microRNA-regulated and GP64-pseudotyped Lentiviral vector mediates stable expression of FVIII in a murine model of hemophilia A.
Mol Ther 19(4):723-30, 2011.

DiPaola J, Lillicrap D.
Challenges and innovations in the treatment of bleeding disorders.
Thromb Res 127 Suppl 1:S1-2, 2011.

James PD, Lillicrap D.
The diagnosis and management of von Willebrand disease in Canada
Semin Thromb Hemost. 37(5):522-7, 2011.

Hubbard AR, Hamill M, Beeharry M, Bevan SA, Heath AB; SSC sub-committee on von Willebrand factor of ISTH.
Value assignment of the WHO 2nd International Standard von Willebrand factor, concentrate (09/182).
J Thromb Haemost. 9(8):1638-40, 2011.

Robertson JD, Yenson PR, Rand ML, Blanchette VS, Carcao MD, Notley C, Lillicrap D, James PD.
Expanded phenotype-genotype correlations in a pediatric population with type 1 von Willebrand disease.
J Thromb Haemost. 9(9):1752-60, 2011.

Viuff D, Barrowcliffe T, Saugstrup T, Ezban M, Lillicrap D.
International comparative field study of N8 evaluating factor VIII assay performance.
Haemophilia. 17(4):695-702, 2011.

Gupta M, Lillicrap D, Stain AM, Friedman KD, Carcao MD.
Therapeutic consequences for misdiagnosis of type 2N von Willebrand disease.
Pediatr Blood Cancer. 1;57(6):1081-3, 2011.

Pruss CM, Golder M, Bryant A, Hegadorn CA, Burnett E, Laverty K, Sponagle K, Dhala A, Notley C, Haberichter S, Lillicrap D.
Pathologic mechanisms of type 1 VWD mutations R1205H and Y1584C through in vitro and in vivo mouse models.
Blood. 117(16):4358-66, 2011.

Hang MX, Blanchette VS, Pullenayegum E, McLimont M, Feldman BM; Canadian Hemophilia Primary Prophylaxis Study Group.
Age at first joint bleed and bleeding severity in boys with severe hemophilia A: Canadian Hemophilia Primary Prophylaxis Study.
J Thromb Haemost. 9(5):1067-9, 2011.

Coutu DL, Cuerquis J, El Ayoubi R, Forner KA, Roy R, François M, Griffith M, Lillicrap D, Yousefi AM, Blostein MD, Galipeau J.
Hierarchical scaffold design for mesenchymal stem cell-based gene therapy of hemophilia B.
Biomaterials. 32(1):295-305, 2011.

Goodeve A, James P.
von WIllebrand Disease (October 2011) in: GeneReviews at GeneTests: Medical Genetics Information Resource.
Available at http://www.genetests.org. PMID: 20301765

James P and Goodeve A.
von Willebrand Disease.
Genetics in Medicine 2011 May; 13(5): 365-376. PMID 21289515

Levac B, Parlow J, vanVlyman J, James P, Tuttle A, Shepherd L.
Ringer’s Lactate is Compatible with SAGM Preserved Packed Red Blood Cells for Rapid Transfusion.
Can J Anaesth 2010; 57(12): 1071- 1077.

2010

Biss TT, Blanchette VS, Clark DS, Bowman M, Wakefield C, Silva M, James PD and Rand ML. 
Quantitation of Bleeding Symptoms in Children with von Willebrand Disease (VWD): Use of a Standardized Pediatric Bleeding Questionnaire. 
Online accepted J Thromb Haemost February 2, 2010. DOI: 10.1111/j.1538-7836.2010.03796.
 
M. Bowman, W.M. Hopman, D. Rapson, D. Lillicrap and P. James. 
The Prevalence of Symptomatic VWD in Primary Care Practice.
J Thromb Haemost 2010; 8:213-216.

Reipert B, Arruda V, Lillicrap D.
Animal models of inhibitors.
Haemophilia 16 Suppl 5:47-53, 2010.

Bowman M, Hopman WM, Rapson D, Lillicrap D, Silva M, James P.
A prospective evaluation of the prevalence of symptomatic von Willebrand disease (VWD) in a pediatric primary care population.
Pediatr Blood Cancer 55:171-3, 2010.

Golder M, Pruss CM, Hegadorn C, Mewburn J, Laverty K, Sponagle K, Lillicrap D.
Mutation-specific hemostatic variability in mice expressing common type 2B von Willebrand disease substitutions.
Blood 115:4862-9, 2010.

Lillicrap D.
Improvements in factor concentrates.
Curr Opin Hematol 17:393-7, 2010.

Othman M, Chirinian Y, Brown C, Notley C, Hickson N, Hampshire D, Buckley S, Waddington S, Parker AL, Baker A, James P, Lillicrap D
Functional characterization of a 13 bp deletion (c.-1522_11510del13) in the promoter of the von Willebrand factor gene in type 1 von Willebrand disease.
Blood 116:3645-3652, 2010.

Thakur A, Sengupta R, Matsui H, Lillicrap D, Jones K, Hortelano G.
Characterization of viability and proliferation of alginate-poly-L-lysine-alginate encapsulated myoblasts using flow cytometry.
J Biomed Mater Res B Appl Biomater 94:296-304, 2010.

Rodeghiero F, Tosetto A, Abshire T, Arnold DM, Coller B, James P. Neunert C, Lillicrap D; on behalf of the ISTH/SSC Joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group
ISTH/SCC Bleeding Assessment Tool: A standardized Questionnaire and a Proposal for a New Bleeding Score for Inherited Bleeding Disorders.
J. Thromb Haemost 8:2063-2065, 2010.

Finn JD, Ozelo MC, Sabatino DE, Franck HW, Merricks EP, Crudele JM, Zhou S, Kazazian HH, Lillicrap D, Nichols TC, Arruda VR
Eradication of neutralizing antibodies to factor VIII in canine hemophilia A following liver gene therapy.
Blood 116:5842-5845, 2010.

Othman M, Powell S, Hopman, WM, Lillicrap D
Variability of thromboelastographic responses following the administration of rFVIIa to haemophilia A dogs supports the individualization of therapy with a global test of haemostasis
Haemophilia 16(6):919-25, 2010.

James P and DiPaola J.
The Application of Genetics to Inherited Bleeding Disorders.
Haemophilia 2010; 16 (Suppl 5):1-5.

Hickson N, Hampshire D, Winship P, Goudemand J, Schneppenheim R, Budde U, Castaman G, Rodeghiero F, Federici A, James P, Peake I, Eikenboom J, Goodeve A on behalf of EU-VWD and ZPMCB-VWD Study Groups.
Von Willebrand Factor Variant p.Arg924Gln Marks an Allele Associated with Reduced von Willebrand Factor and Factor VIII Levels.
J Thromb Haemost 2010; 8:1986-93.

Biss TT, Blanchette VS, Clark DS, Wakefield C, James PD and Rand ML.
Use of a quantitative Pediatric Bleeding Questionnaire to assess mucocutaneous bleeding symptoms in children with a platelet function disorder.
J Thromb Haemost 2010; 8:1416-1419.

Biss TT, Blanchette VS, Clark DS, Bowman M, Wakefield C, Silva M, James PD and Rand ML.
Quantitation of Bleeding Symptoms in Children with von Willebrand Disease (VWD): Use of a Standardized Pediatric Bleeding Questionnaire.
J Thromb Haemost 2010; 8:950-956.

2009

Mittal N, James P and Valentino L. 
Diagnosis of von Willebrand Disease in Children. 
Current Pediatric Reviews 2009; 5:169-175.
 
Berber E, James PD, Hough C, Lillicrap D. 
An assessment of the pathogenic significance of the R924Q von Willebrand factor substitution. 
J Thromb Haemost 2009; 7:1672-79.

Albert K, Van Vlyman J, James P, Parlow J.
Ringer's Lactate is Compatible with the Rapid Infusion of AS-3 Preserved Packed Red Blood Cells
Canadian Journal of Anaesthesia 2009; 56:352-356

Bowman M, Riddel J, Rand M, Tosetto A, Silva M, and James P.
Evaluation of the Diagnostic Utility for von Willebrand Disease of a Pediatric Bleeding Questionnaire.
J Thromb Haemost 2009; 7:1418-21

James, P., Lillicrap, D.
The role of molecular genetics in diagnosing von Willebrand disease.
Semin. Thromb. Hemost. 34(6):502-8, 2009

Lillicrap, D.
The improved factor concentrate
Haemostaseologie 28(1):71-3, 2009

Waters B, Qadura M, Burnett E, Chegeni R, Labelle A, Thompson P, Hough C, Lillicrap D.
Anti-CD3 therapy in hemophilia A mice prevents factor VIII inhibitor formation by increasing regulatory CD4+CD25+ T cells.
Blood, 113:193-203, 2009

Vincent AM, Lillicrap D, Boulanger A, Meilleur C, Amesse C, St-Louis J, Rivard GE.
Non-neutralizing anti-FVIII antibodies: different binding specificity to different recombinant FVIII concentrates.
Haemophilia. 2009 Jan;15(1):374-6

Qadura M, Waters B, Burnett E, Chegeni R, Bradshaw S, Hough C, Othman M, Lillicrap
Recombinant and plasma-derived factor VIII products induce distinct splenic cytokine microenvironments in hemophilia A mice.D.
Blood, 114:871-80, 2009

Matsui H, Shibata M, Brown B, Labelle A, Hegadorn C, Andrews C, Chuah M, Vandendriessche T, Miao CH, Hough C, Lillicrap D.
A murine model for induction of long-term immunologic tolerance to factor VIII does not require persistent detectable levels of plasma factor VIII and involves contributions from Foxp3+ T regulatory cells.
Blood, 114:677-85, 2009

Mullah-Ali AM, Chan AK, Lillicrap D, Decker K, Seroski W, Moffat K, Walker I, Pai MK.
Undetected factor VIII in a patient with type 3 von Willebrands disease mistaken as severe haemophilia A.
Haemophilia. 15:1258-1261, 2009

Berber E, James PD, Hough C, Lillicrap D.
An assessment of the pathogenic significance of the R924Q von Willebrand factor substitution.
J Thromb Haemost. 7:1672-1679, 2009

2008

Stakiw J, Bowman M, Hegadorn C, Pruss C, Notley C, Groot E, Lenting P, Lillicrap D, James PD.
The Effect of Exercise on Individuals with Type 1 and Type 2B von Willebrand Disease (VWD).
J.Thromb.Haemostas. 6:90-96, 2008

Prasad S, Lillicrap D, Labelle A, Knappe S, Keller T, Burnett E, Powell S, Johnson KW.
Efficacy and Safety of a New-Class of Hemostatic Drug Candidate, AV513, in Hemophilia A Dogs
Blood, 111:272-279, 2008


Rubinger M, Lillicrap D, Rivard GE, Teitel J, Carcao M, Hensman C, Walker I. The association of Hemophilia Clinic Directors of Canada.
A prospective surveillance study of factor VIII inhibitor development in the Canadian hemophilia A population following the switch to a recombinant factor VIII product formulated with sucrose.
Haemophilia 14:281-6, 2008

Hough C, Cameron C, Notley C, Brown C, O’Brien L, Keightley A,M, Berber E, Lillicrap D.
Influence of a GT Repeat Element on Shear Stress-Responsiveness of the VWF Gene Promoter.
J.Thromb.Haemost. 6 (7):1183-70, 2008

Pruss,C.M., Notley, C.R.P., Hegadorn, C.A., O'Brien, L.A., Lillicrap, D.
ADAMTS13 Cleavage Efficiency is Altered by Polymorphic and Mutagenic Sequence Changes in the A1 and A2 Domains of von Willebrand Factor (VWF).
Br.J.Haematol 143:552-558, 2008. 

James PD, Stakiw J, Leggo J, Walker I, Lillicrap D.
A case of non-resolving hemophilia B Leyden in a 42-year-old male (F9 promoter + 13 A>G)
J Thromb Haemost. 6(5):885-6, 2008

Robertson J, Lillicrap D, James PD.
von Willebrand disease.
Pediatr Clin North Am. 55(2):377-92, 2008

Pipe SW, High KA, Ohashi K, Ural AU, Lillicrap D.
Progress in the molecular biology of inherited bleeding disorders.
Haemophilia. 14 Suppl 3:130-7, 2008

Collins PW, Cumming AM, Goodeve AC, Lillicrap D.
Type 1 von Willebrand disease: application of emerging data to clinical practice.
Haemophilia. 14(4):685-96, 2008

Qadura M, Othman M, Waters B, Chegeni R, Walker K, Labelle A, Hough C, Lillicrap D.
Reduction of the immune response to Factor VIII mediated through tolerogenic Factor VIII presentation by immature dendritic cells.
J.Thromb.Haemostas. 6:2095-104, September 2008


Bowman M, Mundell G, Grabell J, Hopman WM, Rapson D, Lillicrap D, James P.
Generation and validation of the Condensed MCMDM-1VWD Bleeding Questionnaire for von Willebrand disease.
J Thromb Haemost.6:2062-6, 2008

2007


James PD, Notley C, Hegadorn C, Leggo J, Tuttle A, Tinlin S, Brown C, Andrews C, Labelle A, Chirinian Y, O’Brien L, Othman M, Rivard G, Rapson D, Hough C, Lillicrap D
The Mutational Spectrum of Type 1 von Willebrand Disease: Results from a Canadian Cohort Study.
Blood 109:145-54, 2007

Othman M, Labelle A, Mazzetti I, Elbatarny H and Lillicrap D
Adenovirus-induced thrombocytopenia: The role of P-selectin and von Willebrand factor in mediating accelerated platelet clearance.
Blood 109:2832-39, 2007

Matsui H, Shibata M, Brown B, Labelle A, Hegadorn C, Andrews C, Hebbel RP, Galipeau J, Hough C, Lillicrap D
Ex vivo gene therapy for Hemophilia A that enhances safe delivery and sustained in vivo FVIII expression from lentivirally-engineered endothelial progenitors.
Stem Cells, 25:2660-2669, 2007

Barnes C, Blanchette V, Lillicrap D, Mann K, Stain AM, Leggo J, Carcao M.
Different clinical phenotype in triplets with Haemophilia A
Haemophilia 13:202-5, 2007


Lenting PJ, de Groot PG, Denis CV, Marx I, Lillicrap D, Pruss C, Vanhoorelbeke K, De Meyer SF
Correction of the bleeding time in von Willebrand factor (VWF)-deficient mice using murine VWF.
Blood, 109: 2267-2268, 2007

James PD, Notley C, Hegadorn C, Poon MC, Walker I, Rapson D, Association of Hemophilia Clinic Directors of Canada, Lillicrap D.
Challenges in Defining Type 2M von Willebrand Disease: Results from a Canadian Cohort Study.
J.Thromb.Haemostas. 5:1914-1922, 2007

Lillicrap D.
Von Willebrand disease - phenotype versus genotype: deficiency versus disease.
Thromb Res. 120 Suppl 1:S11-6, 2007

Othman M, Lillicrap D.
Distinguishing between non-identical twins: platelet type and type 2B von Willebrand disease.
Br J Haematol. 138(5):665-6, 2007.

Pierce GF, Lillicrap D, Pipe SW, Vandendriessche T.
Gene therapy, bioengineered clotting factors and novel technologies for hemophilia treatment.
J Thromb Haemost. 5(5):901-6, 2007.

Lillicrap D.
Inducing Tregs with hepatic gene therapy .
Blood 110: 1089, 2007

2006

van Damme A, Thorrez L, Ma L, Vandenburgh H, Eyckmans J, Dell’accio F, De Bari C, Luyten F, Lillicrap D,
Coleen D
Efficient lentiviral transduction and improved engraftment of human bone marrow mesenchymal cells
Stem Cells 24:896-907, 2006.

Roland K, Rapson D, Lillicrap D, James P
The value of genetic testing for Type 2B von Willebrand Disease.
Clin.Lab Haem 28:17-21, 2006

James PD, Paterson AD, Notley C, Hegadorn C, Tinlin C, Brown C, Tuttle A, Association of Hemophilia Clinic Directors of Canada, Lillicrap D
Genetic linkage and association analysis in Type 1 von Willebrand Disease (VWD): Results from the Canadian Type 1 VWD Study.
J.Thromb.Hemost. 4:783-92, 2006.

Lillicrap D, Nair SC, Srivastava A, Rodeghiero F, Pabinger I, Federici AB
Laboratory issues in bleeding disorders.
Haemophilia 12 Suppl 3:68-75, 2006

Lillicrap D, Vandendriessche T, High K
Cellular and genetic therapies for haemophilia.
Haemophilia. 12 Suppl 3:36-41, 2006

Dimichele D, Chuansumrit A, London AJ, Thompson AR, Cooper CG, Killian R
Ross, LF, Lillicrap D, Kimmelman J
Ethical issues in haemophilia.
Haemophilia. 12 Suppl 3:30-5, 2006

Jiang H, Lillicrap D, Patarroyo-White S, Liu T, Qian X, Scallan CD, Powell S, Keller T, McMurray M, Labelle A, Nagy D, Vargas JA, Zhou S, Couto LB, Pierce GF
Multi-year Therapeutic Benefit of AAV Serotypes 2, 6 and 8 Delivering Factor VIII to Hemophilia A Mice and Dogs.
Blood 108:107-15, 2006.

James P and Lillicrap D
Genetic Testing for von Willebrand Disease: The Canadian Experience
Seminars in Thrombosis and Haemostasis, 32:546-52, 2006

Berber E, Leggo J, Brown C, Berber E, Gallo N, Feilotter, H, Lillicrap D
DNA Microarray Analysis for the Detection of Mutations in Hemophilia A.
Journal of Thrombosis and Haemostasis, 4:1756-62, 2006

Rawle FE, Pratt KP, Labelle A, Weiner HL, Hough C, Lillicrap D
Induction of Partial Immune Tolerance to Factor VIII Through Prior Mucosal Exposure to the Factor VIII C2 Domain.
Journal of Thrombosis and Haemostasis, 4:2172-9, 2006

Sadler JE, Budde U, Eikenboom JC, Favaloro EJ, Hill FG, Holmberg L, Ingerslev J, Lee CA, Lillicrap D, Mannucci PM, Mazurier C, Meyer D, Nicols WL, Nishino M, Peake IR, Rodeghiero F, Schneppenheim R, Ruggeri ZM, Srivastava A, Montgomery RR, Federici AB, The Working Party on von Willebrand Disease classification.
Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor.
J Thromb Haemost 4:2103-14, 2006

Lee CA, Lillicrap D, Astermark J
Inhibitor development in hemophiliacs; the roles of genetic versus environmental factors.
Semin Thromb Hemost 32 Suppl 2:10-4, 2006

Feldman BM, Pai M, Rivard GE, Israels S, Poon M-C, Demers C, Robinson S, Luke KH, Wu J, Gill K, Lillicrap D, Babyn P, McLimont M, Blanchette VS. Association of Hemophilia Clinic Directors of Canada Prophylaxis Study Group
Tailored prophylaxis in severe hemophilia A: interim results from the first 5 years of the Canadian Hemophilia Primary Prophylaxis Study (CHPS).
J Thromb Hemost 4:1228-36, 2006.

Barnes C, Lillicrap D, Pazmino-Canizares J, Blanchette, VS, Stain, AM, Clark, D, Hensmen, C, Carcao, M
Pharmacokinetics of recombinant Factor VIII (Kogenate-FS) in children and causes of inter patient pharmacokinetic variability. Haemophilia, 12 Suppl 4:40-9, 2006

Lillicrap D.
The Role of Immunomodulation in the Management of Factor VIII Inhibitors
Hematology Am Soc Hematol Educ Program 421-5, 2006


Updated Aug 30, 2016