Publications

JULY, 2024  |  Highlight

von Willebrand disease

Seidizadeh O, James P et al. VWD is considered the most common inherited bleeding disorder with no geographical or ethnic predilection. Establishing the real VWD prevalence is challenging, particularly for the mild form of this condition (type 1 and some type 2), owing to ambiguous diagnosis criteria (prior to the ASH/ISTH/NHF/WFH 2021 Guidelines on Diagnosis), a wide range of clinical and laboratory manifestations, and the lack of strong penetrance and expressivity in its inheritance.


 

 

JANUARY, 2021  |  Highlight

von Willebrand disease: proposing definitions for future research

Connell T, James P et al.  In recent work developing evidence-based guidelines for von Willebrand disease (VWD), it was noted that studies on VWD often used varying definitions. We propose definitions for future use in VWD research to facilitate comparison of treatment options. It is hoped that their adoption will improve the ability of researchers to achieve consistent endpoints in future VWD clinical trials.


 

JANUARY, 2021  |  Highlight

ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease

James P et al.  von Willebrand disease (VWD) is the most common inherited bleeding disorder known in humans. Accurate and timely diagnosis presents numerous challenges. These evidence-based guidelines are intended to support patients, clinicians, and other health care professionals in their decisions about VWD diagnosis. 


 

 
Immunoblot under reducing conditions of plasma

JULY, 2017  |  Highlight

Discrepant platelet and plasma von Willebrand factor in von Willebrand disease patients with p.Pro2808Leufs*24

Bowman M et al. Clinical observations of type 3 VWD patients with the p.Pro2808Leufs*24 variant indicate a milder bleeding phenotype compared with other type 3 patients. This study assesses the effect of this variant on the molecular pathogenesis of VWD and aims to correlate this with the phenotype observed in patients.


 

Confocal images of endothelial colony forming cells

FEBRUARY, 2017  |  Highlight

Controversies in the diagnosis of Type 1 von Willebrand disease

Bowman M and James P.  Type 1 VWD is clinically characterized by mild to moderate mucocutaneous bleeding such as easy bruising, epistaxis, bleeding from minor wounds, oral cavity bleeding and menorrhagia in women. This review focuses on the current challenges and controversies faced in the diagnosis of this disease.


 

Peer-Reviewed

McDonald L, Grabell J, Leung J, Hopman W, James P. Evaluating the impact of the Self-BAT screening tool on patient outcomes: results of the Let’s Talk Period project. Published online by Haemophilia, November 2024. http://doi.org/10.1111/hae.15113 

Chaigneau M, Grabell J, Wijnker E, Bowman M, James P.  Hemophilia testing of young girls in Canada: describing the current recommendations for factor level and genetic testing and the experiences of Canadian parents. Haemophilia. 2024 Oct 5. doi: 10.1111/hae.15107. Online ahead of print. PMID: 39368063 

Skeith L, James P, Kouides P, Uminski K, Duffett L, Jackson S, Sholzberg M, Ragni MV, Cuker A, O’Beirne M, Hews-Girard J, Rydz N, Goodyear DM, Baxter J, James A, Garcia D, Vesely SK, Poon M-C, VWD Pregnancy Loss Study Group. Pregnancy loss in individuals with von Willebrand disease and unspecified mucocutaneous bleeding disorders: A multicentre cohort study.J. Thromb. Haemost 2024 Oct 23:S1538-7836(24)00627-5. doi: 10.1016/j.jtha.2024.09.037. Online ahead of print. 

Berkowitz C, Ma A, Miller V, Goraya S, Kirkland K, Grabell J, Key NS, James PD, Periprocedural hemostatic prophylaxis and outcomes in Bleeding Disorder of Unknown Cause. Res Pract Thromb Haemost. 2024 Sep 16;8(7):102572. doi: 10.1016/j.rpth.2024.102572. eCollection 2024 Oct. PMID: 39498237 

Peyvandi F, Seidizadeh O, Eikenboom J, Denis C, Flood V, James P, Peter Lenting P, Baronciani L, O'Donnell J, Lillicrap D.  von Willebrand disease. Nature Reviews Disease Primers.  2024 Jul 25;10(1):51. doi: 10.1038/s41572-024-00536-8. PMID: 39054329

Iorio A, James P, Ma A, Srivastava A. Haemostatic management of von Willebrand disease during childbirth with a plasma-derived VWF/FVIII concentrate. Journal of Thrombosis and Haemostasis. 2024 Oct;22(10):2739-2744. doi: 10.1016/j.jtha.2024.06.015. Epub 2024 Jun 29. PMID: 38950781

Lillicrap D, James P, Leebeek F, Casari C. Diagnosis and treatment of von Willebrand Disease in 2024 and beyond. Haemophilia. 2024 Mar 13. doi: 10.1111/hae.14970. Online ahead of print. PMID: 38481079

Baker R, Choi P, Curry N, Gebhart J, Gomez K, Henskens Y, Heubel-Moenen F, James P, Abdul-Kadir R, Kouides P, Lavin M, Lordkipanidze M, Lowe G, Mumford A, Mutch N, Nagler M, Othman M, Pabinger I, Sidonio Jr. R, Thomas W, O’Donnell J. Standardisation of Definition and Management for Bleeding Disorder of Unknown Cause: Communication from the SSC of the ISTH. Journal of Thrombosis and Haemostasis, J Thromb Haemost. 2024 Jul;22(7):2059-2070. doi: 10.1016/j.jtha.2024.03.005. Epub 2024 Mar 20. PMID: 38518896

Manuel P, Tang G, Weyand AC, James P, Sholzberg M.  Academic Bullying in Science and Medicine: The Need for Reform. Res Pract Thromb Haemost.2023 Nov 20;8(1):102270. doi: 10.1016/j.rpth.2023.102270. eCollection 2024 Jan. PMID: 38222079

Atiq F, Blok R, van Kwawegen C, Doherty D, Lavin M, van der Bom JG, O’Connell NM, de Meris J, Ryan K, Schols SEM, Byrne MB, Heubel-Moenen FCJI, van Galen KPM, Preston RJS, Cnossen MH, Fijnvandraat K, Baker RI, Meijer K, James PD, Di Paola J, Eikenboom JCJ, Leebeek FWG, O’Donnell JS. Type 1 VWD classification revisited - novel insights from combined analysis of the LoVIC and WiN studies. 2023 Dec 24:blood.2023022457.doi: 10.1182/blood.2023022457. Online ahead of print. PMID: 38142407 

Tijet N, Christopherson P, Habericter S, Flood V, Notley C, Montgomer R, James P, Lillicrap D. The common VWF variant p.Y1584C: Detailed pathogenic examination of an enigmatic sequence change. J Thromb Haemost. 2024 Mar;22(3):666-675. doi: 10.1016/j.jtha.2023.11.016. Epub 2023 Nov 30. PMID: 38040335

Casari C, Leung J, James P. New and Emerging Therapies for Women, Girls and People with the Potential to Menstruate (WGPPM) with VWD. Blood Adv. 2023 Dec 26;7(24):7501-7505. doi: 10.1182/bloodadvances.2023010716. PMID: 37967378 PMID: 37967378

James A., James P. Women’s Bleeding Issues: What Do We Know About Why Women Bleed and What Do We Not Know? J Thromb Haemost. 2024 Feb;22(2):315-322. doi: 10.1016/j.jtha.2023.08.034. Epub 2023 Sep 13. PMID: 37709147  PMID: 37709147.

Tang GH, Thachil J, Bowman M, Bekdache C, James P, Scholzberg M. Patient-centered care in von Willebrand disease: are we there yet? Expert Rev Hematol. 2023 Jul-Dec;16(9):641-649. doi: 10.1080/17474086.2023.2243386. Epub 2023 Aug 15. PMID: 37581602

Varghese SS, Hetu M-F, Bowman M, Herr J, Al-Turki M, Jaff Z, James P, Malik P, Payne D, Johri AM. Impact of Transcatheter Aortic Valve Implantation on Circulating von Willebrand Factor (TAVI-Factor) Study in Patients with Severe Aortic Stenosis. Hemophilia. 2023 Sep;29(5):1306-1312. doi: 10.1111/hae.14825. Epub 2023 Jul 10.PMID: 37428626. PMID: 37428626

Ocran E, Chornenki N, Bowman M, Scholzberg M, James P. Gastrointestinal bleeding in von Willebrand patients: special diagnostic and management considerations. Expert Rev Hematol. Jul-Dec;16(8):575-584. doi: 10.1080/17474086.2023.2221846. Epub 2023 Jun 12. PMID: 37278227

Leung J, James P. Commentary on Laffan et al Expert Consensus for Equitable Care for VWD. Haemophilia. 2023 May;29(3):693-694. doi: 10.1111/hae.14795. Epub 2023 May 3. PMID: 37134264

O'Sullivan J, Tootoonchian E, Ziemele B, Makris M, Federici A, Djambas KC, El Ekiaby M, Rotellini D, Sidonio Jr. R, Iorio A, Coffin D, Pierce G, Stonebraker J, James P, Lavin M. von Willebrand Disease: Gaining a Global Perspective. Haemophilia. 2023 Jul;29(4):1104-1112. doi: 10.1111/hae.14804. Epub 2023 May 22. PMID: 37216656

Kuthiala S, Grabell J, Relke N, Hopman W, Silva M, Jamieson MA, James P. Efficacy of Medical Treatments for Heavy Menstrual Bleeding in Women with Bleeding Disorders Managed in a Tertiary Care Centre. 2023 Mar 13;7(3):100119. doi: 10.1016/j.rpth.2023.100119. eCollection 2023 Mar. PMID: 37065843 

Hews-Girard J, Galica J, Goldie K, James P, Tranmer J. Identifying the Effect of Inherited Bleeding Disorders on the Development of Postpartum Hemorrhage: A Population-based, Retrospective Cohort Study. 2023 Mar 10;7(2):100104. doi: 10.1016/j.rpth.2023.100104. eCollection 2023 Feb. PMID: 37063757

Abou-Ismail Y, James P, Flood V, Connell NT. Beyond the Guidelines: how we approach challenging scenarios in the diagnosis and management of von Willebrand disease. Accepted by the Journal of Thrombosis and Haemostasis 2023 Feb;21(2):204-214. doi: 10.1016/j.jtha.2022.11.042. Epub 2022 Dec 22. PMID:35523118

Kabinetk CS, Abdelrahman N, Lavin M, Akazi S, Sanchez L, James P, Othman M. Challenges in Management of Women with type 2B von Willebrand Disease During Pregnancy and Postpartum: Evidence from Literature and Data from International Registry and Physicians Survey- Communication from the SSC of the ISTH.  2023 Jan;21(1):154-163. doi: 10.1016/j.jtha.2022.10.019. Epub 2022 Dec 22. PMID:36695378 

Doherty D, Grabell J, Christopherson PA, Montgomery RR, Coller BS, Lavin M, O’Donnell JS, James PD, on behalf of the Zimmerman Program Investigators. Variability in ISTH-BAT score with normal ageing in healthy females; contributory factors and clinical significance. Journal of Thrombosis and Haemostasis 2023 Apr;21(4):880-886. doi: 10.1016/j.jtha.2022.11.045.  PMID: 36696194

Khadadah F, Gabarin N, Jiwajee A, Nisenbaum R, Hanif H, James P, Hunchuck J, Handford C, Girdhari R, Sholzberg M. Reducing Use of Coagulation Tests in a Family Medicine Practice Setting: An Implementation Study. Res Pract Thromb Haemost. 2022 Dec 9;6(8):e12843. doi: 10.1002/rth2.12843. eCollection 2022 Nov. PMID: 36514345

Chornenki NLJ, Ocran E, James PD. Special Considerations in GI Bleeding in VWD Patients. Hematology Am Soc Hematol Educ Program. 2022 Dec 9;2022(1):624-630. doi: 10.1182/hematology.2022000390. PMID: 36485078

Kloosterman R, Zago-Schmitt M, Grabell J, Thibeault L, Chaigneau M, Hinds M, Bowman M, Harpell L, Johri A, Good D, James P. A decreased and less sustained response to hemostatic challenge contributes to bleeding in Type 1 von Willebrand disease. Haemophilia. 2023 Jan;29(1):370-373. doi: 10.1111/hae.14699. Epub 2022 Nov 26. PMID: 36433930 

Kloosterman R, Hindmarch C, Renwick N, Tyryshkin K, Grabell J, Thibeault L, Lima P, Bowman M, Zago-Schmitt M, James P. A transcriptome analysis of basal and stimulated VWF release from endothelial cells derived from Type 1 VWD patients. Blood Adv. 2022 Sep 19:bloodadvances.2022007884. doi: 10.1182/bloodadvances.2022007884. Online ahead of print. PMID: 36121439

Chaigneau M, Botros M, Grabell J, Hopman W, James P. Challenges and Knowledge Gaps facing Hemophilia Carriers today: Perspectives from Patients and Healthcare Providers. Res Pract Thromb Haemost. 2022 Sep 20;6(6):e12783. doi: 10.1002/rth2.12783. eCollection 2022 Aug. PMID: 36186104

Yeung K, McGrath C, Howse K, James P. Prise en charge des patients ayant des scores élevés au Self-BAT ou d’autres symptômes hémorragiques: Aperçu actualisé à l’intention des professionnels des soins primaires. Can Fam Physician. 2022 Jul;68(7):501-506. doi:10.46747/cfp.6807501

Yeung K, McGrath C, Howse K, James P. Management of patients with elevated Self-BAT scores or other bleeding symptoms: Updated overview for primary care practitioners. Can Fam Physician July 2022, 68(7): 494-499; DOI: https://doi.org/10.46747/cfp.6807494

Lavin M, Christopherson P, Grabell J, Abshire T, Flood V, Haberichter SL, Lillicrap D, O’Donnell JS, Montgomery RR, James PD. Longitudinal Bleeding Assessment in von Willebrand Disease Utilising an Interim Bleeding Score. J Thromb Haemost. 2022 Oct;20(10):2246-2254. doi: 10.1111/jth.15807. Epub 2022 Jul 26. PMID:35780487

Matino D, Iorio A, Keepanasseril A, Germini F, Caillaud A, Carcao M, Hews-Girard J, Iserman E, James P, Lee A, Phua CW, Sun H(L), Teitel J, Poon M-C. Switching to nonacog beta pegol in hemophilia B: Outcomes from a Canadian real-world, multicenter, retrospective study. Research and Practice in Thrombosis and Haemostasis. 2022 Mar 31;6(3):e12661. doi: 10.1002/rth2.12661. eCollection 2022 Mar.

Hews-Girard JC, Galica J, Goldie C, James P, Tranmer J. Determining the incidence of postpartum hemorrhage among Ontario women with and without inherited bleeding disorders: A Population-based cohort study. Haemophilia. 2022 Sep;28(5):832-841. doi: 10.1111/hae.14594. Epub 2022 Jun 3. PMID: 35665573

Doherty D, Lavin M, Byrne MB, Nolan M, O'Sullivan JM, Ryan K, O'Connell NM, Haberichter SL, Christopherson P, Di Paola J, James PD, O'Donnell JS. Enhanced VWF clearance in Low VWF pathogenesis - limitations of VWFpp/VWF:Ag ratio and clinical significance. Blood Adv. 2023 Feb 14;7(3):302-308. doi: 10.1182/bloodadvances.2022007340 PMID: 35523118

Bowman M, Casey L, Selvam SN, Lima P, Hinds M, Tuttle A, Grabell J, Iorio A, Walker I, Lillicrap D, James P. von Willebrand factor propeptide variants lead to impaired storage and ER retention in patient-derived endothelial colony-forming cells. J Thromb Haemost. 2022 Jul;20(7):1599-1609. doi: 10.1111/jth.15740. Epub 2022 May 3. PMID: 35466528

El Alayli A, Brignardello Petersen R, Husainat NM, Kalot MA, Aljabiri Y, Turkmani H, Britt A, El-Khechen H, Shahid S, Roller J, Motaghi S, Mansour R, Tosetto A, Abdul-Kadir R, Laffan M, Weyand A, Leebeek FWG, Arapshian A, Kouides P, James P, Connell NT, Flood VH, Mustafa RA. Outcomes of Long-Term von Willebrand Factor Prophylaxis use in von Willebrand Disease: A Systematic Literature Review. Haemophilia. 2022 May;28(3):373-387. doi: 10.1111/hae.14550. Epub 2022 Mar 26. PMID: 35339117

James P, Connell N, Flood V, Mustafa R. Response to “The 2021 von Willebrand disease guidelines: Clarity and controversy”. Haemophilia 2022 May;28(3):371-372. doi: 10.1111/hae.14528. Epub 2022 Mar 22. PMID: 35316557 

Valentino LA, Hardi ML, James P, Connell N, Flood V, Scappe N, Frick N.  von Willebrand disease (VWD) and BATs: How do they connect and why should I care? Haemophilia 2022 May;28(3):e89-e90. doi: 10.1111/hae.14549. Epub 2022 Mar 16. PMID: 35294992

Kalot MA, Husainat N, Abughanimeh O, Diab O, el Alayli A, Tayiem S, Madoukh B, Dimassi A, Qureini A, Ameer B, Eikenboom J, Giraud, Haberichter S, Jacobs-Pratt V, Konkle B, McRae S, Montgomery R, O’Donnell JS, Brignardello R, Flood V, Connell NT, James P, Mustafa RA.  Laboratory Assays of VWF Activity and Use of Desmopressin Trials in the Diagnosis of VWD: A Systematic Review and Meta-Analysis.  Blood Adv. 2022 Jun 28;6(12):3735-3745. doi: 10.1182/bloodadvances.2021005431. PMID: 35192687

Rawley O, Swystun LL, Brown C, Nesbitt K, Rand M, Hossain T, Klaassen R, James PD, Carcao MD, Lillicrap D. Novel Cysteine Substitution p.(CYS1084TYR) causes Variable Expressivity of Qualitative and Quantitative VWF Defects . Blood Adv. 2022 May 10;6(9):2908-2919. doi: 10.1182/bloodadvances.2021005928. PMID: 35020809 

Sholzberg M, Dacosta B, Tang G, Hassan R, James P, Lillicrap D, Carrier M, Beckett A, Fralick M, Middeldorp S, Cushman M, Juni P. Randomised Trials of Therapeutic Heparin for COVID-19: A Meta-Analysis. Res Pract Thromb Haemost. 2021 Dec 17;5(8):e12638. doi: 10.1002/rth2.12638. eCollection 2021 Dec. PMID: 34977448

Brignardello-Petersen R, El Alayli A, Husainat N, Kalot MA, Shahid S, Aljabirii Y, Britt A, Alturkmani HJ, El Khechen H, Motaghi S, Roller J, Abdul Kadir R, Couper S, Kouides P, Lavin M, Ozelo MC, Weyand AC, James PD, Connell NT, Flood VH, Mustafa RA. Gynecologic and obstetric management of women with von Willebrand disease: summary of 3 systematic reviews of the literature. Blood Adv. 2022 Jan 11;6(1):228-237. doi: 10.1182/bloodadvances. 2021005589. PMID: 34673921

Brignardello-Petersen R, El Alayli A, Husainat N, Kalot MA, Shahid S, Aljabirii Y, Britt A, Alturkmani HJ, El Khechen H, Motaghi S, Roller J, Dimassi AB, Abughanimeh O, Madoukh B, Arapshian A, Grow JM, Kouides P, Laffan MA, Leebeek FWG, O'Brien SH, Tosetto A, James PD, Connell NT, Flood VH, Mustafa RA. Surgical management of patients with VWD: summary of 2 systemic reviews of the literature. Blood Adv. 2022 Jan 11;6(1):121-128. doi: 10.1182/bloodadvances.2021005666. PMID: 34654053

Kalot MA, Husainat N, El Alayli A, Abughanimeh O, Diab O, Tayiem S, Madoukh B, Dimassi AB, Qureini A, Ameer B, Eikenboom JCJ, Giraud N, McLintock C, McRae S, Montgomery RR, O'Donnell J, Scappe N, Sidonio RF, Brignardello-Petersen R, Flood VH, Connell NT, James PD, Mustafa RA.  Von Willebrand Factor Levels in The Diagnosis of von Willebrand Disease: A Systematic Review and Meta-Analysis. Blood Adv. 2022 Jan 11;6(1):62-71. doi: 10.1182/bloodadvances.2021005430. PMID:34610118

Sholzberg M, James P. Commentary: Surviving Sexism in Bleeding Disorders Affecting Women. Brit J Haem. 2022 Jan;196(1):15-16. doi: 10.1111/bjh.17881. Epub 2021 Oct 7. PMID:34618910

Sholzberg M, Tang GH, Rahhal H, AlHamzah M, Kreuziger LB, Áinle FN, Alomran F, Alayed K, Alsheef M, AlSumait F, Pompilio CE, Sperlich C, Tangri S, Tang T, Jaksa P, Suryanarayan D, Almarshoodi M, Castellucci LA, James PD, Lillicrap D, Carrier M, Beckett A, Colovos C, Jayakar J, Arsenault MP, Wu C, Doyon K, Andreou ER, Dounaevskaia V, Tseng EK, Lim G, Fralick M, Middeldorp S, Lee AYY, Zuo F, da Costa BR, Thorpe KE, Negri EM, Cushman M, Jüni P; RAPID trial investigators. Effectiveness of therapeutic heparin versus prophylactic heparin on death, mechanical ventilation, or intensive care unit admission in moderately ill patients with covid-19 admitted to hospital: RAPID randomised clinical trial. BMJ. 2021 Oct 14;375:n2400. doi: 10.1136/bmj.n2400

Kalot MA, Husainat N, Tayiem S, El Alayli A, Dimassi AB, Diab O, Abughanimeh O, Madoukh B, Qureini A, Ameer B, Eikenboom JCJ, Di Paola J, Jacobs-Pratt V, McLintock C, Montgomery RR, O'Donnell J, Sidonio RF, Brignardello-Petersen R, Flood VH, Connell NT, James PD, Mustafa RA. Bleeding Assessment Tools in the Diagnosis of von Willebrand Disease in Adults and Children: A Systematic Review and Meta-Analysis of Test Accuracy. Blood Adv. 2021 Dec 14;5(23):5023-5031. doi: 10.1182/bloodadvances. 2021004368. PMID:34597385

Chaigneau M, James PD, Approach to the Patient with Bleeding. Hematol Oncol Clin North Am. 2021 Dec;35(6):1039-1049. doi: 10.1016/j.hoc.2021.07.001. Epub 2021 Sep 15. PMID: 34535288 

VanGalen KPM, Doiron R, James P, Abdul-Kadir R, Kouides P, Kulkarni R, Mahlangu J, Othman M, Peyvandi F, Rotenillini D, Winikoff R, Sidonion R. Communication from the SSC of the ISTH: A New Hemophilia Carrier Nomenclature to define hemophilia in women and girls.  J Thromb Haemost. 2021 Aug;19(8):1883-1887. doi: 10.1111/jth.15397. PMID: 34327828

Sholzberg M,  Tang GH, Rahhal H, AlHamzah M, Baumann Kreuziger L, Ní Áinle F, Alomran F, Alayed K, Alsheef M, AlSumait F,  Pompilio CE, Sperlich C, Tangri S, Tang T, Jaksa P, Suryanarayan D, Almarshoodi M, Castellucci L,  James PD, Lillicrap D, Carrier M, Beckett A, Colovos C, Jayakar J,  Arsenault M-P, Wu C, Doyon K,  Andreou ER, Dounaevskaia V,  Tseng ET, Lim G, Fralick M, Middeldorp S,  Lee AY, Zuo F,  da Costa BR,  Thorpe KE,  Negri EM, Cushman M, Jüni P, RAPID Trial investigators. Heparin for Moderately Ill Patients with Covid-19. medRxiv. 2021 Jul 12;2021.07.08.21259351. doi: 10.1101/2021.07.08.21259351. Preprint

Bannow S, McLintock C, James P. Menstruation, Anticoagulation and Contraception: VTE and Uterine Bleeding. Res Pract Thromb Haemost. 2021 Aug 1;5(5):e12570. doi: 10.1002/rth2.12570. eCollection 2021 Jul. PMID: 34368613 

Spence M, de Repentigny K, Bowman M, Hopman W, Thibeault L, James P. Validation of the Pictorial Blood Loss Assessment Chart using Modern Sanitary Products. Haemophilia. Sep;27(5):e632-e635. doi: 10.1111/hae.14373. Epub 2021 Jun 29. PMID:34185363 

Chornenki NLJ, Shanjer M, James PD. Vascular abnormalities in patients with von Willebrand Disease: A Scoping review. J Thromb Haemost. 2021 Sep;19(9):2151-2160. doi: 10.1111/jth.15410. Epub 2021 Jul 9. PMID:34060212

Sholzberg M, Tang GH, Negri E, Rahhal H, Baumann Kreuziger L, Pompilio CE, James P, Fralick M, AlHamzah M, Alomran F, Tseng E, Lim G, Lillicrap D, Carrier M, Ni Áinle F, Beckett A, R da Costa B, Thorpe K, Middeldorp S, Lee A, Cushman M, Jüni P. Coagulopathy of hospitalised COVID-19: A Pragmatic Randomised Controlled Trial of Therapeutic Anticoagulation versus Standard Care as a Rapid Response to the COVID-19 Pandemic (RAPID COVID COAG - RAPID Trial): A structured summary of a study protocol for a randomised controlled trial. Trials 2021 Mar 10;22(1):202. doi: 10.1186/s13063-021-05076-0.

Hossenbaccus L, Thibeault L, Grabell J, Braund H, Hopman W, James P. Evaluating the Effectiveness of Let’s Talk Period’s High School Educational Outreach Program: A Pilot Study. Haemophilia 2021 Feb 24. doi: 10.1111/hae.14277. Online ahead of print. PMID: 33626190

Khalife R, Aw A, Duffett L, Radhwi O, Lacasse L, Huebsch L, Bowman M, James P, Alan Tinmouth A.
To treat or not? Remission induction of acquired von Willebrand syndrome secondary to chronic lymphocytic leukemia: A case report. Clin Lymphoma Myeloma Leuk.
2021 Jan 13;S2152-2650(21)00008-2. 
doi: 10.1016/j.clml.2021.01.007. PMID: 33558201

Connell NT, Flood VH, Brignardello-Petersen R, Abdul-Kadir R, Arapshian A, Couper S, Grow JM, Kouides P, Laffan M, Lavin M, Leebeek FWG, O’Brien SH, Ozelo MG, Tosetto A, Weyand AC, James PD et al.
ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv (2021) 5(1):301–325. PMID: 33570647

Doherty BH, O’Sullivan J, Ryan K, Dougall A, Byrne M, Brafferty M, Doyle MM, DiPaola J, James PD, O’Donnell J.
Management of Elective Procedures in Low von Willebrand factor patients in the LoVIC study. 
J Thromb Haemost. 2021 Mar;19(3):701-710. doi: 10.1111/jth.15220. Epub 2021 Jan 24. PMID: 33346399

Weyand AC, James PD. Sexism in the treatment of bleeding disorders.
Research and Practice in Thrombosis and Haemostasis 2020 Dec 13;5(1):51-54.
 doi: 10.1002/rth2.12468. eCollection 2021 Jan.

Ariens R, Becattini C, Bender M, Bergmeier W, Castoldi E, Devreese K, Ellis M, Gailani D, Ignjatovic V, James PD et. al. Illustrated State-of-the-Art Capsules of the ISTH 2020 Congress. Res Pract Thromb Haemost. 2020 Jul 12;4(5):680-713. doi: 10.1002/rth2.12368.eCollection 2020 Jul. PMID: 32685876

James P. Women and Bleeding Disorders: Diagnostic Challenges. Hematology Am Soc Hematol Educ Program. 2020 Dec 4;2020(1):547-552. doi: 10.1182/hematology.2020000140. PMID: 33275722 

de Boer S, Bowman M, Notley C, Mo A, Lima P, de Jong A, Dirven R, Weijers E, Lillicrap D, James P, Eikenboom J. Endothelial characteristics in healthy endothelial colony forming cells; generating a robust and valid ex vivo model for vascular disease.  J Thromb Haemost. 2020 Jul 12. doi: 10.1111/jth.14998. PMID: 32654420

Theilmann AL, Hawke LG, Hilton LR, Whitford MKM, Cole DV, Mackeil JL, Dunham-Snary KJ, Mewburn J, James PD, Maurice DH, Archer SL, Ormiston ML. Endothelial BMPR2 Loss Drives a Proliferative Response to BMP (Bone Morphogenetic Protein) 9 via Prolonged Canonical Signaling. Arterioscler Thromb Vasc Biol. 2020 Nov;40(11):2605-2618. doi: 0.1161/ATVBAHA.119.313357. Epub 2020 Oct 1. PMID: 32998516 

Goldberg N, Nisenbaum R, Song H, Lillicrap D, Teitel J, James P, Sholzberg M. Desmopressin responsiveness by age in type 1 von Willebrand disease. Res Pract Thromb Haemost. 2020 May 30;4(6):1046-1052. doi: 10.1002/rth2.12354. eCollection 2020 Aug. PMID: 32864555

Riedl Khursigara M, Schlam D, Noone DG, Bruno V, Ortiz Sandoval CG, Pluthero FG, Kahr WHA, Bowman ML, James PD, Grinstein S, Licht. Vascular endothelial cells evade complement-mediated membrane injury via Weibel-Palade body mobilization. J Thromb Haemost. 2020 Jun;18(6):1484-1494. doi: 10.1111/jth.14767. PMID: 32073731

Patel S, Zhang L, Booth CM, James PD, Nanji S. A population-based cohort study of VTE rates following surgery and during adjuvant chemotherapy in patients with colon cancer. Dis Colon Rectum. 2020 Mar;63(3):336-345. doi: 10.1097/DCR.0000000000001557. PMID: 32045399

Selvam SN, Bowman M, Inglis M, Kloosterman R, Grabell J, Casey L, Johri A, James P. Patients with aortic stenosis have von Willebrand factor abnormalities and increased proliferation of ECFCs. J Thromb Haemost. 2020 Mar;18(3):593-603. doi: 10.1111/jth.14715. Epub 2020 Jan 22. PMID: 31860769

Kalot MA, Al-Khatib M, Connell NT, Flood V, Brignardello-Petersen R, James PD, Mustafa RA; VWD working group. An international survey to inform priorities for new guidelines on von Willebrand disease. Haemophilia. 2020 Jan;26(1):106-116. doi: 10.1111/hae.13881. Epub 2019 Nov 26. PMID: 31769905

Tang GH, Norris E, Petrucci J, James PD, Lee A, Poon MC, Floros G, Boma-Fischer L, Teitel J, Nisenbaum R, Sholzberg M. Bone health in symptomatic carriers of haemophilia A: a protocol for a multicentre prospective matched-cohort study. BMJ Open. 2019 Dec 2;9(12):e032891. doi: 10.1136/bmjopen-2019-032891. PMID: 31796490

Spradbrow J, Letourneau S, Grabell J, Liang Y, Riddel J, Hopman W, Blanchette VS, Rand ML, Coller BS, Paterson AD, James PD. Bleeding Assessment Tools to Predict von Willebrand Disease: Utility of individual bleeding symptoms. Res Pract Thromb Haemost. 2019 Oct 30;4(1):92-99. doi: 10.1002/rth2.12256. eCollection 2020 Jan. PMID: 31989089

Castaman G, James PD. Pregnancy and Delivery in Women with von Willebrand Disease. Eur J Haematol. 2019 Aug;103(2):73-79. doi: 10.1111/ejh.13250. Epub 2019 May 31. Review. PMID: 31107984

Relke N, Kuthiala S, Grabell J, Hopman W, James PD. The Bleeding score: Useful in Predicting Spontaneous Bleeding Events in Adults with Bleeding of Unknown Cause? Haemophilia. 2020 Mar;26(2):e31-e33. doi: 10.1111/hae.13775. Epub 2019 May 20. PMID: 31106930 

Aguila S, Lavin M, Dalton N, Patmore S, Chion A, Trahan GD, Jones KL, Keenan C, Brophy TM, O'Connell NM, Ryan K, Byrne M, Nolan M, Patel A, Preston RJS, James PD, Di Paola J, O'Sullivan JM, O'Donnell JS.
Increased galactose expression and enhanced clearance in patients with Low von Willebrand factor.
2019 Apr 4;133(14):1585-1596. doi: 10.1182/blood-2018-09-874636. Epub 2019 Feb 15.
PMID: 30770394

Bui J, Matyres D, James PD, Grabell J, Wu J, Steele JM, Silva MP, Rand M, Blanchette V, Barrowman N, Klaasen R. Validation of the School Age Self-Administered Pediatric Bleeding Questionnaire (Self-PBQ) in Children Aged 8-12 Years. Pediatr Blood Cancer. 2019 Jun;66(6):e27709. doi: 10.1002/pbc.27709. Epub 2019 Mar 22. PMID: 30900820

Swystun L, Notley C, Lai J, Nesbitt K, James PD, Lillicrap D. The Endothelial Lectin Clearance Receptor CLEC4M Binds and Internalizes Factor VIII in a VWF-Dependent and -Independent Manner. J Thromb Haemost. 2019 Apr;17(4):681-694. doi: 10.1111/jth.14404. Epub 2019 Mar 19. PMID: 30740857

Candy V, Whitworth H, Grabell J, Thibeault L, Harpell L, Bowman M, Good D, Hopman WM, Sidonio Jr. RF, James PD. A Decreased and Less Sustained Desmopressin Response in Hemophilia A Carriers Contributes to Bleeding.
Blood Adv. 2018 Oct 23;2(20):2629-2636. doi10.1182/bloodadvances. 2018023713. PMID: 30327371

Swystun L, Lai JD, Notley C, Georgescu I, Paine AS, Mewburn J, Nesbitt K, Schledzewski K, Geraud C, Kzhyshkowska J, Goerdt S, Hopman W, Montgomery RR, James PD, Lillicrap D. The endothelial cell receptor stabilin-2 regulates von Willebrand factor-factor VIII complex half-life and immunogenicity. J Clin Invest. 2018 Aug 31;128(9):4057-4073. doi: 10.1172/JCI96400. Epub 2018 Aug 20. PMID: 30124466

Lavin M, Aguila S, Dalton N, Nolan M, Byrne M, Ryan K, White B, O’Connell NM, O’Sullivan J, di Paola J, James PD, O’Donnell JS. Significant Gynaecological Bleeding in Women with low von Willebrand Factor Levels.
Blood Adv. 2018 Jul 24;2(14):1784-1791. doi: 10.1182/bloodadvances. 2018017418. PMID: 30042144

Pelland-Marcotte M-C, Humpl T, James PD, Rand ML, Bouskill V, Reyes JT, Bowman M, Carcao MD.
Idiopathic pulmonary arterial hypertension – a unrecognized cause of high-shear high-flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children. Br J Haematol.
2018 Aug 23. doi: 10.1111/bjh.15530. [Epub ahead of print]. PMID: 30141279

Parker JW, James PD, Haley S. Spinal Anesthesia in Two Consecutive Caesarean Deliveries in a Parturient with Type 3 von Willebrand Disease: A Case Report. A A Pract. 2019 Feb 1;12(3):79-81. doi: 10.1213/XAA.0000000000000854. PMID: 30052531

Stokhuijzen E, Rand M, Cnossen M, Biss T, James PD, Suijker MH, Peters M, Vanderlee JH, Peters B, Meijer S, Blanchette V, Fijnvandraat CJ. Identifying Children with Hereditary Coagulation disorders (iCHEC ): a protocol for a prospective cohort study.  BMJ Open. 2018 May 3;8(5):e020686. doi: 10.1136/bmjopen-2017-020686. PMID: 29724741

Thorne JG, James PD, Reid RL. Heavy Menstrual Bleeding: is Tranexamic Acid a Safe Adjunct to Combined Hormonal Contraception? Contraception. 2018 Jul;98(1):1-3. doi: 10.1016/j.contraception.2018.02.008. Epub 2018 Mar 26. PMID: 29477633

Bowman M, Rimmer E, Houston DS, Israels S, James PD. Discordant VWF Activity in Patients with VWF p.Gly1324Ser Confirmed in Vitro. Haemophilia. 2018 Mar;24(2):e57-e59. doi: 10.1111/hae.13401. Epub 2018 Jan 17. PMID: 29341351

Stokhuijzen E, Segbefia C, Biss TT, Clark D, James PD, Riddel J, Blanchette VS, Rand ML. Severity and Features of Epistaxis in Children with a Mucocutaneous Bleeding Disorder. J Pediatr. 2018 Feb;193:183-189.e2. doi: 10.1016/j.jpeds.2017.09.082. PMID: 29198540

Lavin M, Aguila S, Schneppenheim S, Dalton N, Jones KL, O'Sullivan JM, O'Connell NM, Ryan K, White B, Byrne M, Rafferty M, Doyle MM, Nolan M, Preston RJS, Budde U, James PD, Di Paola J, O'Donnell JS. Novel Insights into the Clinical Phenotype and Pathophysiology Underlying Low von Willebrand Factor LevelsBlood. Nov 23;130(21):2344-2353. PMID: 28916584

Young JE, Grabell J, Tuttle A, Bowman M, Hopman WM, Good D, Rydz N, Mahlangu JN, James PD. Evaluation of the Self-Administered Bleeding Assessment Tool (Self-BAT) in Hemophilia Carriers and Correlations with Quality of Life. Haemophilia. 2017 Nov;23(6):e536-e538. doi: 10.1111/hae.13354. PMID: 28949433

Casey LJ, Klaassen RJ, Rand ML, James PD. Reply to: Comment on: Generation and Optimization of the Self-Administered Pediatric Bleeding Questionnaire and its Validation as a Screening Tool for von Willebrand Disease.
Pediatr Blood Cancer. 2017 Dec;64(12). PMID: 28792666

Bowman ML, Pluthero FG, Tuttle A, Casey L, Li L, Christensen H, Robinson KS, Lillicrap D, Kahr WHA, James PD.
Discrepant Platelet and Plasma von Willebrand Factor in von Willebrand Disease Patients with p.Pro2808Leufs*24.
J Thromb Haemost. 2017 Jul;15(7):1403-1411. PMID: 28453889

Reynen E, Grabell J, Ellis AK, James PLet’s Talk Period! Preliminary Results of an Online Bleeding Awareness Knowledge Translation Project and Bleeding Assessment Tool Promoted on Social Media. Haemophilia. 2017 Jul;23(4):e282-e286. PMID: 28544019

Casey LJ, Tuttle A, Grabell J, Hopman W, Moorehead PC, Blanchette VS, Wu JK, Steele M, Klaassen RJ, Silva M, Rand ML, James PD. Generation and optimization of the self-administered pediatric bleeding questionnaire and its validation as a screening tool for von Willebrand disease. Pediatr Blood Cancer. 2017 Apr 28. doi: 10.1002/pbc.26588. PMID: 28453185

Selvam SN, Casey LJ, Bowman ML, Hawke LG, Longmore AJ, Mewburn J, Ormiston ML, Archer SL, Maurice DH, James PD. Abnormal Angiogenesis in Blood Outgrowth Endothelial Cells Derived from von Willebrand Disease Patients. Blood Coagul Fibrinolysis. 2017 Oct;28(7):521-533. PMID: 28362648

Labarque V, Perinparajah V, Bouskill V, Stain AM, Wakefield C, Manuel C, Blanchette VS, James PD, Lillicrap D, Carcao MD. Utility of Factor VIII and Factor VIII to von Willebrand Factor Ratio in Identifying 277 Unselected Carriers of Hemophilia A. Am J Hematol. 2017 Jun;92(6):E94-E96. PMID: 28249352

Bowman M, James P.  Bleeding scores for the diagnosis of von Willebrand disease. Seminars In Thrombosis and Hemostasis 2017 Feb 14. doi: 10.1055/s-0036-1597289. PMID: 28196380

Selvam S, James PD. Angiodysplasia in von Willebrand Disease: Understanding the Clinical and Basic Science. Semin Thromb Hemost. 2017 Sep;43(6):572-580. PMID: 28476066

Goodeve A, James P. von Willebrand Disease (January 2017) in: GeneReviews at GeneTests: Medical Genetics Information Resource. Available at http://www.genetests.org. PMID: 20301765

Michels A, Albánez S, Mewburn J, Nesbitt K, Gould TJ, Liaw PC, James PD, Swystun LL, Lillicrap D.
Histones Link Inflammation and Thrombosis Through the Induction of Weibel-Palade Body Exocytosis.
J Thromb Haemost. 2016 Nov;14(11):2274-2286. PMID: 27589692

Swystun L.L, James P. Genetic Diagnosis in Hemophilia and von Willebrand disease. Blood Rev. 2017 Jan;31(1):47-56. doi: 10.1016/j.blre.2016.08.003. Review. PMID: 27596108

James PD, Mahlangu J, Bidlingmaier C, Mingot-Castellano ME, Chitlur M, Fogarty PF, Cuker A, Mancuso ME, Holme PA, Grabell J, Satkunam N, Hopman WM, Mathew P; Global Emerging HEmostasis Experts Panel (GEHEP). Evaluation of the Utility of the ISTH-BAT in Hemophilia Carriers: a Multinational Study.
Haemophilia. 2016 Nov;22(6):912-918. PMID: 27868369

Hawke L, Grabell J, Sim W, Thibeault L, Muir E, Hopman W, Smith G, James P.  Obstetric bleeding among women with inherited bleeding disorders: a retrospective study. Haemophilia. 2016 Nov;22(6):906-911. doi:10.1111/hae.13067. PMID: 27704714

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. Haemophilia. 2017 Jan;23(1):115-121. doi: 10.1111/hae.13062. PMID: 27611464

Reynen E, James P. von Willebrand Disease and Pregnancy: A Review of Evidence and Expert Opinion.
Semin Thromb Hemost. 2016 Oct;42(7):717-723. PMID: 27648611

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. PMID: 27317792

James P. VWD Diagnosis: Improved. Blood. 2016;127(20):2372-3. PMID: 27207322

Flood V, Christopherson P, Cox Gill J, Friedman KD, Haberichter SL, Bellissimo DB, Udani RU, 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 A, James P, Lillicrap D, Peake I, Montgomery RR.
Clinical and Laboratory Variability in a Cohort of Patients Diagnosed with Type 1 VWD in the United States.
Blood. 2016;127(20):2481-8. PMID: 26862110

Albánez S, Ogiwara K, Michels A, Grabell J, James P, Lillicrap D. Aging and ABO blood type influence VWF and FVIII levels through interrelated mechanisms. J Thromb Haemost. 2016 May;14(5):953-63. PMID: 26875505

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. PMID: 27236750

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. PMID: 26637715

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). 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

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

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

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

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

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

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

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

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

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

Mollah SA, James PD, Grabell J, Barbour EM, Coller B. Diagnostic Prediction of von Willebrand Disease using Multiple Bleeding Phenomics Datasets. AMIA Jt Summits Transl Sci Proc. 2013 Mar 18;2013:184. PMID 24273489

Rydz N, Leggo J, Tinlin S, James P, Lillicrap D. The Canadian "National Program for Hemophilia Mutation Testing" database: a ten-year review. Am J Hematol Dec 2013; 88(12):1030-4. PMID: 23913812

Rydz N, Goodyear D, James PD. The Clinical Diagnosis of Mild von Willebrand Disease. Int Monit VWD 2013 May: 9 - 12.

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). J Ped Hematol Oncol 2014 May; 36(4) [PubMed-in process] PMID: 23774159

Kahlon A, Grabell J, Tuttle A, Engen D, Hopman W, Lillicrap D and James PD. Quantitation of Peri-operative Changes in von Willebrand Factor and Factor VIII During Elective Orthopedic and Cardiac Surgery in Normal Individuals. Haemophilia 2013 Sept; 19(5): 758-64. PMID: 23711418

Rimmer E, Jamieson MA, James PD. Malposition and Expulsion of the Levonorgestrel Intrauterine System Among Women with Inherited Bleeding Disorders. Haemophilia 2013 Nov; 19(6): 933-8. PMID: 23711358

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. Blood Coag & Fibrin 2013 Oct; 24(7): 719-26. PMID: 23846000

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

Berntorp E, Fuchs B, Makris M, Montgomery R, Flood V, O'Donnell JS, Federici AB, Lillicrap D, James PD, Budde U, Morfini M, Petrini P, Austin S, Kannicht C, Jiminez-Yuste V, Lee C. Third Aland islands conference on von Willebrand disease, 26 - 28 September 2012: meeting report. Haemophilia. March 19, 2013; Suppl 3:1-18. PMID 23383607.

James PD and Lillicrap D. The molecular characterization of von Willebrand disease: Good in parts. Brit J Haem 2013 Apr: 161(2): 166-76. PMID: 23406206

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

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. Transfusion Medicine and Hemotherapy, 2013 Oct;40(5):352-5. [Epub 2013 Aug 26] PMID: 24273489

James PD, Lillicrap DP and Mannucci PM. Alloantibodies in von Willebrand Disease. Blood. 2013 Aug 1;122(5):636-40. Epub 2013 Jan 7. Review.PMID: 23297130

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

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

James PD, Lillicrap D. von Willebrand Disease: Clinical and Laboratory Lessons Learned from the Large von Willebrand Disease Studies. Am J Hematol 2012 Feb 8, doi: 10.1002/ajh.23142 Epub ahead of print PMID: 22389132

Rydz N and James PD. Why is my patient bleeding or bruising? Hematol Oncol Clin N Am 2012 (26); 321-344. PMID: 22463830

Federici AB, JamesPD. Current management of patients with severe von Willebrand disease type 3: a 2012 update. Acta Haematol 2012;128(2):88-99. PMID: 22722677

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

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

Robertson, JD, Yenson PR, Rand ML, Carcao M, Blanchette VS, Lillicrap D, James PD. Expanded Phenotype-Genotype Correlations in a Pediatric Population with Type 1 von Willebrand Disease. J Thromb Hamost 2011; 9:1752-1760. PMID: 21711445

James P and Lillicrap D. The Diagnosis and Management of von Willebrand Disease in Canada. Semin Thromb Hemost 2011; 37: 522-527. PMID: 22102195

Darwish H, Mundell G, Engen D, Lillicrap D, Silva M and James PD. Early Intraoperative Blood Collection Does Not Affect CBCs, VWF or FVIII Levels in Normal Children.J Ped Hematol Oncol 2011; 33(1): 47 - 49. PMID: 21178707

Chapters/Monographs

Israels SJ, James P, Blanchette V, Rand L. 
Bleeding Assessment Tools. 
Appendix in 3rd Edition of the SickKids Handbook of Thrombosis and Hemostasis. Karger 2023.

Natesirinikul R, Lobianco e Souza L, Barnes C, James P, Blanchette V. 
von Willebrand Disease in Children.
Chapter in 3rd Edition of the SickKids Handbook of Thrombosis and Hemostasis. Karger 2023.

James P, Rawley O, Bowman M.
Structure, Biology and Genetics of von Willebrand Factor.
Chapter in Hematology Basic Principles and Practice 8th edition. Elsevier 2022.

James P, O’Brien S.  
Bleeding Assessment Tools.
Chapter in Inherited Bleeding Disorders in Women, second edition. Wiley-Blackwell 2018

James P and Lillicrap D. 
Molecular Diagnostic Approaches to Hemostasis.
Chapter in Practical Hemostasis and Thrombosis. Wiley-Blackwell 2016.

Rydz N and James PD. 
von Willebrand Factor and von Willebrand Disease.
Hoffman, Hematology 6e, Elsevier, 2011.

James PD, Tosetto A.
Clinical Aspects of von Willebrand Disease: Bleeding History.
Chapter in von Willebrand Disease: Basic and Clinical Aspects.Wiley-Blackwell 2011.

James PD, Lillicrap D.
Von Willebrand Disease: an Introduction for the Primary Care Physician, Treatment of Hemophilia Monographs.
The World Federation of Hemophilia, January 2009, No. 47.

James PD, Lillicrap D.
The Role of Molecular Genetics in Diagnosing von Willebrand Disease.
Chapter in Seminars in Thrombosis and Hemostasis 2008 Sept; 34(6): 502-8.

James PD, Lillicrap D.
Von Willebrand Factor.
Chapter in Mechanisms in Hematology, Core Health Services Inc, Concord Ontario, 2008.

James PD, Lillicrap D.
Molecular Diagnostic Approaches to Bleeding Disorders and Thrombophilia.
Textbook chapter for Practical Hemostasis and Thrombosis, Blackwell Science, 2005.

Online Publications

James P. Patient education: von Willebrand disease (Beyond the Basics). Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com. Accessed October 12, 2022

James P. von Willebrand disease (VWD): Gynecologic and obstetric considerations. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com. Accessed October 12, 2022.

James P. von Willebrand disease (VWD): Treatment of minor bleeding, use of DDAVP, and routine preventive care. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com. Accessed October 12, 2022.

James P. von Willebrand disease (VWD): Treatment of major bleeding and major surgery. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com. Accessed October 12, 2022.

James P. Pathophysiology of von Willebrand disease. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com. Accessed October 12, 2022.

James P. Clinical presentation and diagnosis of von Willebrand disease. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com. Accessed October 12, 2022.

Peer-Reviewed Abstracts

Agarwal S, Adelaja A, Stanek JR, James P, Flood V, O’Brien SH. Primary Care Screening for von Willebrand Disease in African American Adolescents with Heavy Menstrual Bleeding. Accepted to ASH 2024 

Cusano E, Hews-Girard J, Basharat M, Cowley L, Grabell J, Hodgson N, Pittman K, Rupnaraine A, Thillaye-Kerr R, Cord Lethebe B, James P, Khalifé R, Pardy N, Phua CW, Prokopchuk-Gauk O, Sholzberg M, Sun H (L), Poon M-C, Goodyear D. Clinical and Economic Outcome Analysis of First-Line Immunosuppression in Acquired Hemophilia A. Submitted Accepted to ASH 2024. 

Ocran E, Burnett E, Bowman M, Maurice D, Lillicrap D, James P. Localized recombinant human von Willebrand Factor influences in vivo angiogenesis in VWFdeficient mice. Submitted to ISTH 2024.

McDonald L, Grabell J, Leung J, Hopman W, James P. Evaluating the impact of the Self-BAT screening tool on referral outcomes. Results of the Let’s Talk Period project. Accepted for oral presentation, WFH 2024

Chaigneau M, Grabell J, James P. Exploring changing beliefs and practices regarding hemophilia carrier testing in young girls: perspectives from parents of carriers and healthcare providers in Canada. Accepted for oral presentation, WFH 2024

Adelaja A, Stanek J, Flood V, James P, Vesely S, O’Brien S. Assessment of ISTH-BAT Scores in a General Population of Menstruating Females. Blood Nov 2023, Vol 142, Suppl 1, 1243.

Tang GH, Nisenbaum R, Martin R, Manuel P, Sampat R, Teitel J, James PD, Sholzberg M. Pre-operative coagulation test results do not correlate with Self-bleeding assessment tool (Self-BAT) scores. Blood Nov 2023, Vol 142, Suppl 1, 2610.

Mainland R, Ganguli P, James P, Lui J, Satkunam N, Leung J. Modern Scurvy and Hematology: A Retrospective Chart Review in Kingston, Ontario. Blood Nov 2023, Vol 142, Suppl 1, 1239.

Chornenki NLJ, Grabell J, Bowman M, Good D, Hookey L, James P. A pilot study evaluating von Willebrand Factor in patients with Gastrointestinal Angiodysplasia. Blood Nov 2023, Vol 142, Suppl 1, 1250.

Atiq F, Blok R, van Kwawegen CB, Doherty D, Lavin M, van der Bom JG, O’ Connell NM, de Meris J, Ryan K, Schols SEM, Byrne M, FHeubel-Moenen FCJI, van Galen KPM, Preston RJS, Fijnvandraat K, RI Baker, Meijer K, James P, Di Paola J, Eikenboom J, O’Donnell JS, Leebeek FWG. Age regulates desmopressin responses in patients with Low Von Willebrand Factor and Type 1 Von Willebrand Disease in the WiN and LoVIC studies. Blood Nov 2023, Vol 142, Suppl 1, 509.

Atiq F, Blok R, van Kwawegen CB, Doherty D, Lavin M, van der Bom JG, O’ Connell NM, de Meris J, Ryan K, Schols SEM, Byrne M, FHeubel-Moenen FCJI, van Galen KPM, Preston RJS Fijnvandraat K, RI Baker, Meijer K, James P, Di Paola J, Eikenboom J, Leebeek FWG, O’Donnell JS. The relationship between Low Von Willebrand Factor, Type 1 Von Willebrand Disease and ageing – novel insights from the WiN and LoVIC cohort studies. Blood Nov 2023, Vol 142, Suppl 1, 510.

Peterson W, Martin R, Nisenbaum R, Arnold D, Carvalho B, Cuker A, Gadsden J, Provan D, Rydz N, Shore E, Kuter D, Kouides P, Lavin M, James P, Engen D, Sholzberg M. Delphi consensus recommendations for neuraxial anesthesia in adults with platelet disorders and coagulation defects. RPTH Oct 2023, Vol 7, Suppl 2, PB1033.

Skeith L, James P, Kouides O, Uminski K, Duffett L, Jackson S, Sholzberg M, Ragni MV, Cuker A, O’Beirne M, Rubinstein S, Baxter J, Hews-Girard J, Rydz N, Goodyear D, James A, Garcia D, Vesely SK, Poon M-C. A multicentre cohort study evaluating pregnancy loss in patients with von Willebrand disease and unspecified bleeding disorders. RPTH Oct 2023, Vol 7, Suppl 2, OC 25.3.

Sadler B, Christopherson P, Haller G, Antunes L, Cruchaga C, Paterson A, James P, Lillicrap D, O’Donnell J, Montgomery RR, Di Paola J and the Zimmerman Program Investigators. Whole-genome characterization of low-VWF and VWD Type 1 Patients with no Known Pathogenic VWF Variant Reveals Novel Genetic Associations. RPTH Oct 2023, Vol 7, Suppl 2, OC 13.5.

Ocran E, Burnett E, Bowman M, Rawley O, Maurice D, Lillicrap D, James P. Localized Endothelial Colony-Forming Cell-derived von Willebrand Factor (VWF) influences In Vivo Angiogenesis in VWFdeficient Mice. RPTH Oct 2023, Vol 7, Suppl 2, PB0808.

Hinds M, Bowman M, Grabell J, Yeh CH, Gross PL, Fox-Robichaud AE, Kim PY, Lillicrap D, James P. The VWF-ADAMTS13 axis as biomarkers of disease progression in severe COVID-19. RPTH Oct 2023, Vol 7, Suppl 2, PB0604.

Notley C, Bowman M, James P, Lillicrap D. Involvement of the nuclear protein, TC2N, in regulating VWF levels in endothelial cells. RPTH Oct 2023, Vol 7, Suppl 2, PB0815.

Doherty D, Christopherson P, Byrne M, Nolan M, O’Sullivan JM, Ryan K, O’Connell NM, Montgomery RR, Flood V, Haberichter SL, Di Paola J, James PD, Lavin M, O’Connell JS on behalf of the Zimmerman Program Investigators. Defining the Clinical Utility of ISHT-BAT Scores in Patients with Low von Willebrand Factor (VWF) levels. RPTH Oct 2023, Vol 7, Suppl 2, PB0829.

Ocran EK, Burnett E, Bowman ML, Rawley O, Cormier M, Maurice D, Lillicrap D, James PD. Investigating the Role of von Willebrand Factor (VWF) in Modulating In Vivo angiogenesis in VWF-Deficient Mice. Blood (2022) 140 (Supplement 1): 11222–11223

Doherty D, Byrne M, Nolan M, O'Sullivan J, Ryan K, O'Connell N, Haberichter S, Christopherson P, Di Paola J, James P, Lavin M, O'Donnell J. A novel role for subtle enhanced VWF clearance in Low VWF pathogenesis highlights limitations of VWFpp/VWF:Ag ratio assessment of clearance. Research and Practice in Thrombosis and Haemostasis 10-2-22, Vol.6(S1)

Bowman M, Hinds M, Lima P, Burnett E, Ng C, Lillicrap D, James P. Syntaxin-2 deficiency results in reduced von Willebrand factor expression in vitro and in vivo. Research and Practice in Thrombosis and Haemostasis 10-2-22, Vol.6(S1)

Noureldin A, Miljic P, Lavin M, Kazi S, Sanchez, Luceros A, James P, Othman M.  Management of women with type 2B von Willebrand disease during pregnancy and postpartum: Evidence from literature, data from international registry and Haemophilia. Volume 28, Issue S3 FP-09.06 (1159792)

Yeung K, McGrath C, Howse K, James P. Management of Patients Presenting with an Elevated Self-administered Bleeding Assessment Tool (Self-BAT) Score or other Bleeding Symptoms in Primary Care. Haemophilia. Volume 28, Issue S3 LR-11.01 (1165145)

Botros M, Chaigneau M, Grabell J, Hopman W, James P. Challenges and knowledge gaps facing hemophilia carriers as identified by patients and healthcare providers. Haemophilia. Volume 28, Issue S3 PP-82 (1164354)

Grabell J, Chaigneau M, James P. Does the Use of an Online Screening Tool affect Referral Outcomes? Preliminary Results of the Let’s Talk Period Project. Haemophilia. Volume 28, Issue S3 PP-77 (1161516)

Thakkar H, Grabell J, James P. Development and Optimization of Linguistic and Culturally-Appropriate Translations of the Self-BAT for use in India: Hindi Pilot Study. Accepted to World Federation of Hemophilia 2022 Congress, November 2021

Spence M, Grabell J, Chaigneau M, Hopman W, Good D, Smith G, Lillicrap D, James P. Coagulation Factor Levels and Postpartum Blood Boss: Preliminary Results of the Canadian Hemophilia Carriers Study-Postpartum (CHIC-P). E-Poster at World Federation of Hemophilia 2022 Congress, January 2022

Bowman M, Grabell J, Paterson AD, Poon M-C, Jackson S, Lillicrap D, Haller G, Sadler B, James P. Enrichment of Variants in Platelet Genes in Patients with Bleeding of Unknown Cause [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1).

Khalife R, Aw A, Duffett L, Radhwi O, Lacasse L, Huebsch L, Bowman M, James P, Tinmouth A. To Treat or Not? Remission Induction of Acquired von Willebrand Syndrome Secondary to Chronic Lymphocytic Leukemia: A Case Report [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1).

Iorio A, Srivastava A, James P, Ma A. Plasma-derived VWF/FVIII Concentrate (wilate®) for Haemostasis in Women with VWD during Childbirth [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1).

Meschino D, Lindsay D, Tang G, James P, Sholzberg M. A Major Knowledge Gap in Women’s Health: The Combined Effect of Antifibrinolytics and Estrogen on Risk of Thromboembolism – A Scoping Review [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1). 

Chornenki N, Shanjer M, James P. Vascular Abnormalities in Patients with Von Willebrand Disease: A Scoping Review [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1). 

Ocran E, Nesbitt K, Hinds M, Rawley O, Bowman M, Lillicrap D, James P. Evaluation of von Willebrand Factor (VWF) Replacement on in vivo Angiogenesis in VWFdeficient Mice [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1).

Kloosterman R, Zago-Schmitt M, Grabell J, Thibeault L, Bowman M, Lima PDA, Tyryshkin K, Hindmarch CCT, Renwick N, James P. Transcriptional Analysis of the Response to Stimulation in ECFCs Derived from Type 1 VWD Patients [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1).

de Repentigny K, Hinds M, Grabell J, Iorio A, Keepanasseril A, Robinson S, Sun L, Jackson S, Rydz N, James P. The CHiC Study: The Canadian Hemophilia Carriers Study – Preliminary Results. Res Pract Thromb Haemost. 2020;4(Suppl.1): PB0982.

Selvam S, Nesbitt K, Lima P, Kloosterman R, Bowman M, Lillicrap D, James P. Increased in vivo angiogenesis in von Willebrand factor-deficient mice.  Res Pract Thromb Haemost. 2019;3(Suppl. 1):OC56.1

Bowman M, Grabell J, Tuttle A, Thibeault L, Iorio A, James P. Investigations of the von Willebrand Factor (VWF) p.Arg273Trp Variant using Endothelial Colony Forming Cells (ECFCs). Res Pract Thromb Haemost. 2019;3(Suppl. 1):OC56.3

Rand ML, Wang H, Bang A, Cloutier S, Jackson S, Poon M-D, James P.  Insights into Defects in Ca2+-Mobilization in Platelets from Patients with von Willebrand Disease Type 2B. Res Pract Thromb Haemost. 2019;3(Suppl. 1):PB0434

de Boer S, Bowman M, Notley C, Mo A, Lima P, de Jong A, Dirven R, Lillicrap D, James P, Eikenboom J.  VWF synthesis and secretion and endothelial cell characteristics in health ECFCs: Generating a valid ex vivo model for VWD.  Res Pract Thromb Haemost. 2019;3(Suppl. 1):PB0073

Kalot M, Al-Khatib M, Connell N, Flood V, Brignardello-Peterson R, Clark C, Castano J, Riker E, Robinson F, Skinner M, James P, Mustafa R.  Von Willebrand Disease: An International Survey Assessing the Important Topics for Decision-Making. Res Pract Thromb Haemost. 2019;3(Suppl. 1):PB0817

Peters B, Stokhuijzen E, Cnossen M, Biss T, Peters M, Suijker M, Silva M, Blanchette V, James P, Rand ML, Fijnvandraat K. iCHEC: Refining the Pediatric Bleeding Assessment Tool.  Res Pract Thromb Haemost. 2019;3(Suppl. 1):PB0832

Rawley O, Swystun L, Brown C, Rand M, Hossain T, Klaassen R, James P, Carcao M, Lillicrap D.  Mixed Phenotype von Willebrand Disease Caused by a Novel Cysteine Variant that Results in both Qualitative and Quantitative von Willebrand Factor Defects. Res Pract Thromb Haemost. 2019;3(Suppl. 1):OC13.3

Zago-Schmitt M, Kloosterman R, Grabell J, Thibeault L, Harpell L, Bowman M, Hopman W, Johri A, Good D, James P. Decreased and Less Sustained Hemostatic Stress Responses May Contribute to Abnormal Bleeding in Type 1 VWD: Preliminary Results. HTRS/NASTH 2019 Scientific Symposium. March 2019.

Yan W, Lam CH, Archer S, Vanner S, Taylor D, James P. Designing innovative programs in Translational Medicine: A case of Developmental Evaluation. The 2019 Canadian Conference on Medical Education

Christopherson P, Haberitchter S, Friedman KD, DiPaola J, James P, Journeycake JM, Lentz S, Leissinger CA, Ragna MV, Rajpurkar M, Roberts J, Shapiro A, Sidonio Jr. RB, Flood V. Prospective Diagnosis of VWD in a Large Cohort of Patients with Bleeding Symptoms through the Zimmerman Program. Blood 2018;132(Suppl.1):979

Chaudhry H, JiwajeevZ, Scholzberg M, Nisenbaum R, James P, Girdhari G, Handford C, Hunchuck J.  Reducing Unnecessary Coagulation Testing in Outpatients (REDUCTION): A Quality Improvement Project. Blood 2018;132(Suppl.1):4703

Rawley O, Swystun L, Brown C, Rand M, Hossain T, Klaassen R, James P, Carcao M, Lillicrap D. Mixed Phenotype von Willebrand disease caused by a Novel Cysteine Variant that results in both Qualitative and Quantitative von Willebrand Factor Defects. Haematology Association of Ireland AGM September 2018

Bowman M, Tinmouth A, Selby R, Sholzberg M, Lillicrap D, Good D, James P. Discrepant von Willebrand factor (VWF) Activity Levels in two Unrelated von Willebrand Disease (VWD) Patients. Res Pract Thromb Haemost. 2018;2(Suppl. 1):PB282

Selvam SN, Bowman M, Inglis M, Grabell J, Casey L, Thibeault L, Johri AM, James P. Patients with Aortic Stenosis have von Willebrand Factor Abnormalities and Aberrant Angiogenesis in BOEC. Res Pract Thromb Haemost. 2018;2(Suppl. 1):PB107

Kuthiala S, Relke N, Grabell J, Hopman W, Silva M, Jamieson MA, James P. Safety and Efficacy of Menorrhagia Treatments in Women with Bleeding Disorders: a Retrospective Analysis. WFH 2018

Relke N, Kuthiala S, Grabell J, Hopman W, James P. The Bleeding Score: Useful in Predicting Spontaneous Bleeding Events in Adults with Bleeding of Unknown Cause? Haemophilia May 2018 24;S5 T-P-033 (195).

Thibeault L, Hossenbaccus L, Grabell J, Purcell S, James P. Let’s Talk Period: Nursing Outreach in Grade 9 Females, a Pilot Project. Haemophilia May 2018 24;S5 W-P-141 (268).

Pelland-Marcotte MC, Humpl T, James P, Rand M, Bouskill V, Reyes JT, Bowman ML, Carcao M. Acquired von Willebrand syndrome – a Complication of Idiopathic Pulmonary Hypertension in Children. Haemophilia May 2018 24;S5 W-FPYR-001 (147).

Grabell J, James P. Does the Use of an Online Screening Tool Affect Referral Outcomes? Preliminary Results of the Let’s Talk Period Project. Haemophilia May 2018 24;S5 W-P-003 (106).

Garcia J, Christopherson PA, Flood VH, Cox Gill J, Montgomery RR, James PD, Simpson P, Zhang L, Abshire TC, Zimmerman Program Investigators. Contribution of Select ISTH-BAT Subscores to the Total Score in Type 1 VWD. Blood 2017 130:1075

Goldberg N, Nisenbaum R, Song H, Lillicrap D, Teitel JM, James PD, Scholzberg M. Does Age Affect Desmopressin Responsiveness in Patients with Von Willebrand Disease? Blood 2017 130:3681

Spradbrow J, Letourneau S, Grabell J, Liang Y, Riddel J, Hopman W, Blanchette VS, Rand ML, Coller BS, Paterson AD, James PD. Use of Bleeding Assessment Tools in von Willebrand disease: Are We Asking the Right Questions? Blood 2017 130:2369

Bui J, Martyres D, James PD, Grabell J, Wu JK, Steele M, Silva M, Rand ML, Blanchette, Klaassen RJ. Validation of the Modified Self-Administered Pediatric Bleeding Assessment Questionnaire (Self-PBQ) in Children Aged 8-12 Years. Blood 2017 130:2135

Swystun LL, Paine S, Notley C, Lai J, Nesbitt K, Schledzewski K, Geraud C, Goerdt S, Hopman W, Montgomery R, James PD, Lillicrap D. Genetic Variability and Glycans Modulate the Interaction Between VWF and the Sinusoidal Endothelial Clearance Receptor Stabilin-2. Res Pract Thromb Haemost. 2017;1(Suppl. 1):152.

Notley C, Bowman M, Brown C, Tyryshkin K, Swystun L, James P, Lillicrap D. Common Single Nucleotide Variants in the von Willebrand Factor Gene Associate with “Mutation Negative” Type 1. Res Pract Thromb Haemost. 2017;1(Suppl. 1):704.

Christopherson P, Lavin M, Grabell J, Abshire T, Flood F, Gill J, Haberichter S, O’Donnell J, Montgomery R, James P and the Zimmerman Program Investigators. Exploration of Interim Bleeding Scores in VWD Subjects from Irish LoVIC, Canada and the US Zimmerman Program. Res Pract Thromb Haemost. 2017;1(Suppl. 1):703

Selvam SN, Bowman M, Pruss C, James P. Intracellular von Willebrand Factor Predominately Influences Angiogenesis. Res Pract Thromb Haemost. 2017;1(Suppl. 1):1415-16.

Candy V, Grabell J, Thibeault L, Harpell L, Bowman M, Good D, Sidonio Jr. RF, James P.  Evaluation of the Response to Desmopressin in Hemophilia A Carriers and Normal Controls: Preliminary results. Haemophilia. 2017;23(Suppl. S3):8-9.

Lavin M, Aguila S, Schneppenheim S, Dalton N, O’Sullivan JM, O’ Connell NM, Ryan K, Byrne M, Nolan M, Preston RJS, Budde U, James P, Di Paola J, O’Donnell JS. Novel Insights into the Clinical Phenotype and Pathophysiology Underlying Low VWF Levels: The Low Von Willebrand Factor Ireland Cohort (LoVIC) Study. Blood. 2016;128(22):873.

James P, Sholzberg M, Iorio A, Cosentino L on behalf of the Canadian WIL20 Working Group. Interim Canadian Data From a Prospective, Observational Study of Routine Clinical Use of a von Willebrand Factor Concentrate (Wilate®). Blood. 2016;128(22):2593.

Satkunam N, Mahlangu JN, Bidlingmaier C, Mingot-Castellano ME, Chitlur M, Fogarty PF, Cuker A, Mancuso ME, Holme PA, Grabell J, Hopman WM, Mathew P, James PD. Characterization of Bleeding in Hemophilia Carriers and Comparison to Women with Type 1 von Willebrand Disease, Type 3 von Willebrand Disease Obligate Carriers and Controls. Blood. 2016;128(22):875.

Reynen E, Grabell J, James P. Let’s Talk Period: Preliminary Results of an Online Bleeding Awareness Knowledge Translation Project and Bleeding Assessment Tool Promoted on Social Media. Blood. 2016;128(22):3775.

Selvam SN, Casey L, Longmore AJ, Bowman M, James P. Blood Outgrowth Endothelial Cells From Type 3 von Willebrand Disease Patients Display Abnormal Angiogenesis. J. Thromb Haemost.  2016

Bowman M, Casey L, Selvam SN, Tinlin S, Tuttle A, Brown C, Lillicrap D, James P. Understanding "Normal": Baseline VWF Characteristics in Normal BOECs. J. Thromb Haemost.  2016;14(Suppl.1):154-55.

DiMaria EC, Trussler A, Dyba J, Lee D, Rauh MJ, James PD, Hopman W, Hay AE. Testing for Heparin Induced Thrombocytopenia: Comparison of Practice at an Academic Centre with Choosing Wisley Guidelines. Blood. 2015;126(23):3469.

James P. The Clinical Utility of Bleeding Scores.  J. Thromb Haemost.  2015;13(Suppl. 2):26.

Casey L, Bowman M, Soundarya S, James P. Abnormal Angiogenesis in Type 2A and 2B von Willebrand Disease (VWD): Comparative Studies of Quantitative and Qualitative VWD using Blood Outgrowth Endothelial Cells. J. Thromb Haemost. 2015;13(Suppl. 2):102.

Albanez S, Swystun L, Sponagle K, Grabell J, Brown C, James P, Lillicrap D. Evaluation of Age and Glycan-Related Changes on von Willebrand Factor (VWF) Clearance in a VWF Deficient Mouse Model. J. Thromb Haemost. 2015;13 (Suppl. 2):753.

Xi M, Blanchette V, Blatny J, Collins P, Dunn A, Fischer K, Hermans C, Navarro-Ruan T, Kavakli K, Jackson S, James P, Mammen S, Morfini M, Neufeld E, Ozele M, Radossi P, Rangarajan S, Ruiz-Saez A, Teitel J, Thabane L, Young G, Iorio A and on behalf of the WAPPS Investigators. Pharmacokinetic Characteristics of Factor VIII and IX Concentrates- A Systematic Review. J Thromb Haemost. 2015;13(Suppl. 2):357.

Iorio A, Blanchette V, Blatny J, Collins P, Crouteau S, Dunn A, Fischer K, Hermans C, Kavalki K, Jackson S, James P, Mammen S, Morfini M, Navarro-Ruan T, Neufeld EJ, Ozelo M, Radossi P, Rangarajan S, Ruiz-Saez A, Teitel J, Thabane L, Young G, Xi M. A Population Approach to Hemophilia Pharmacokinetics. WAPPS: A Web-Service for Bayesian Post Hoc Estimation J. Thromb Haemost. 2015;13(Suppl. 2):44.

Xu Y, Deforest M, Grabell J, Hopman W, James P. Health-related quality of life among von Willebrand Disease patients is not related to disease severity. J. Thromb Haemost. 2015;13(Suppl. 2):496.

Young J, Grabell J, Rydz N, Hopman W, Good D, Mahlangu J, James P. Relationship between quality of life and coagulation factor level in Hemophilia Carriers: Preliminary Results. J. Thromb Haemost. 2015;13(Suppl. 2):590.

James PD, Bidlingmaier C, Mingot-Castellano ME, Chitlur M, Fogarty PF, Grabell J, Mathew P, Mahlangu P and Global Hemostasis Experts Panel (GEHEP). Validation of the International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT) in Hemophilia Carriers. J. Thromb Haemost. 2015;13(Suppl. 2):842.

Tuttle A, Grabell J, Bae S, Moorehead P, Blanchette V, Wu J, Steele M, Klaasen R, Silva M, Rand ML, James PD.
Validation of the Self-PBQ (Self-Administered Pediatric Bleeding Questionnaire) as a Screening Tool for a new Diagnosis of von Willebrand Disease (VWD). J. Thromb Haemost. 2015;13(Suppl. 2):661

Swystun LL, Notley C, Georgescu I, Sponagle K, Schledzewski K, Geraud C, Kzhyshkowska J, Goerdt S, James PD, Lillicrap D. The scavenger receptor stabilin-2 (STAB-2) mediates clearance of human von Willebrand factor and factor VIII by liver sinusoidal endothelial cells. J. Thromb Haemost. 2015;13 (Suppl. 2):252

Webster S, Hampshire D, Theophilus B, Schneppenheim R, Bellissimo D, James PD, Peake I, Goodeve A
Detection of Large Exonic and Intergenic Deletions in the VWF Locus using Array Comparative Genomic Hybridisation (aCGH). J. Thromb Haemost. 2015;13(Suppl.2):126.

Hawke L, Rivard G, James P. Identification and Characterization of Aberrant Splicing from a Novel Branch Site Mutation in von Willebrand FactorJ. Thromb Haemost. 2015;13(Suppl. 2):506

Albánez S, Ogiwara K , Grabell J , James P, Lillicrap D. Aging and ABO Blood Type Regulate von Willebrand Factor and Factor VIII Levels through Distinct Patterns of both Secretion and Clearance. J. Thromb Haemost. 2015;13(Suppl. 2):508-9.

Bidlingmaier C, Mahlangu J, James P. Global Emerging Hemophilia Panel (GEHEP). Validation of the International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT) in haemophilia carriers. 59th Annual Meeting of the Society of Thrombosis and Hemostasis Research (GTH), Düsseldorf, Germany February, 2015.

Bowman M, Casey L, Tuttle A, James PD. Distinct cellular VWF phenotypes observed in BOEC from type 3 VWD patient with VWF propeptide mutations compared to non-propeptide mutations. 8th Bari International Conference, Bari, Italy, October, 2014.

Pruss CM, Barr S, Grabell J, Tinlin S, Tuttle A, Adams MA, Garland JS, James PD and Holden RM.
von Willebrand Factor Synthesis and Circulating Half Life are Progressively Elevated, but Relative Platelet Binding Decreases in stage 3-5 Chronic Kidney Disease Patients. Canadian Hypertension Congress, October 2014

Pruss CM, Barr S, Tuttle A, Grabell J, Tinlin S, Adams MA, Hopman WM, Garland JS, James PD and Holden, RM.
Stage 3-5 CKD patient coronary artery calcification status correlates positively with von Willebrand Factor platelet binding. ASN Kidney Week, 2014.

Albanez S, Ogiwara K, Grabell J, James P, Lillicrap D. Association Between von Willebrand Factor and Factor VIII Levels and their Changes During the Aging Process. J Thromb Haemost 2014 June; 12 (Suppl 1): 2014.

Albanez S, Ogiwara K, Grabell J, James P, Lillicrap D. Age-Related Changes to the Mechanisms Responsible for Regulating Plasma Levels of von Willebrand Factor and Factor VIII. Thrombosis Research 2014 May; 133 (Suppl 3): S31.

Tuttle A, Grabell J, Clark DS, Moorehead P, Blanchette VS, Wu J, Steele M, Klaasen R, Silva M, Rand ML, James PD. Item Reduction Analysis of the Self-PBQ (Self-Administered Pediatric Bleeding Questionnaire): Healthy Children and Children Previously Diagnosed with Type 1 von Willebrand Disease (VWD). J Thromb Haemost 2014 June; 12 (Suppl 1): PHT03.

Casey, L, Bowman M, Umana B, Maurice D, James PD. Patient-Derived Blood Outgrowth Endothelial Cells from a Type 2B von Willebrand Disease Patient Exhibit Increased Angiogenesis. J Thromb Haemost 2014 June; 12 (Suppl 1): VWF04.

Deforest M, Grabell J, Hopman W, James PD. The Self-BAT (Self-administered Bleeding Assessment Tool) is an Effective Screening Tool for von Willebrand disease in Women Referred to Hematology. Haemophilia 2014;20(Suppl 3): 183.

Young J, Grabell J, Tuttle A, Deforest M, Good D, Rydz N, Mahlangu J, Hopman W, James PD. Validation of the Self-BAT (Self-administered Bleeding Assessment Tool) in Hemophilia Carriers: Preliminary Results. Haemophilia 2014;20(Suppl 3): 7.

Kaur H, Assam H, Ozelo M, Scovil S, James PD, Othman M. Assessment of bleeding phenotype in PT-VWD and other RBDs using the electronic bleeding questionnaire (eBQ): a retrospective study on 55 subjects. Haemophilia 2014; 20(Suppl 3): 108.

Hawke L, Rivard G-E, Poon M-C, James PD. Characterization of VWF Splice Variants from Exonic and Intronic Splicing Mutations. Blood (ASH Annual Meeting Abstracts), Oct 2013; 122: 21, 1069.

Swystun L, Notley C, Georgescu I, James PD, Lillicrap D. The Endothelial Lectin Receptor CLEC4M Internalizes Factor VIII and VWF Via a Clathrin-Coated Pit-Dependent Mechanism. Blood (ASH Annual Meeting Abstracts), Oct 2013; 122: 21, 1091.

Elbatarny M, Mollah S, Grabell J, Rand ML, Clark D, Mauer A, Christopherson P, Montgomery R, Zimmerman Program Investigators, Coller B, James PD. Normal Range of Bleeding Scores for the ISTH-BAT: Adult and Pediatric Data From the Merging Project. Blood (ASH Annual Meeting Abstracts), Oct 2013; 122: 21, 1107.

Swystun L, Notley C, Sponagle K, James PD, Lillicrap D. Regulation of Factor VIII Clearance by Mannose-Binding Lectins. Blood (ASH Annual Meeting Abstracts), Oct 2013; 122: 21, 2340.

Bowman M, Casey L, Hawke L, James PD. Heterogeneity of Type 3 VWD: Evidence from Patient-Derived Blood Outgrowth Endothelial Cells (BOEC). Blood (ASH Annual Meeting Abstracts), Oct 2013; 122: 21, 3517.

Michels A, Albanez S, Swystun L, Sponagle K, James PD, Lillicrap D. Association of cell-free DNA with plasma von WIllebrand factor levels in human and mouse models of inflammation. J Thromb Haemost, 2013; 11(Suppl 2), OC 85.5.

Hawke L, Poon M-C, Scully M-F, James PD. Alterations in aberrant and endothelial splicing of von Willebrand factor under high laminar shear stress. J Thromb Haemost, 2013; 11(Suppl 2), PB 1.44-1.

Rand M, Tuttle A, Grabell J, Moorehead P, Deforest M, Wu J, Steele M, Klaassen R, Silva M, James PD. Development of the Self-PBQ (Self-administered Pediatric Bleeding Questionnaire): Pre-testing and optimization.
J Thromb Haemost, 2013; 11(Suppl 2), PA 1.09-6.

Bowman M, Casey L, Morrison L, Tuttle A, Walker I, Silva M, Jacobi P, Haberichter S, Lillicrap D, James PD.
Investigation of the contribution of VWF propeptide mutations to Type 3 VWD using in vitro cellular studies and patient-derived BOEC. J Thromb Haemost, 2013; 11(Suppl 2), OC 39.5.

Mollah S, Rand M, Clark DS, Christopherson P, Mauer A, Montgomery RR, Zimmerman Program Investigators, Coller B, James PD. The Merging Project: A Machine Learning Approach to Merge and Analyze Data from Four Different Bleeding Questionnaires. J Thromb Haemost, 2013; 11(Suppl 2), PA 2.08-2.

Swystun L, Notley C, Sponagle K, James PD, Lillicrap D. The endothelial lectin CLEC4M is a novel clearance receptor for Factor VIII. J Thromb Haemost, 2013; 11(Suppl 2), OC 05.6.

Albanez S, Michels A, Sponagle K, Grabell J, James PD, Lillicrap D. An evaluation of the age-related quantitative and qualitative pathophysiology of VWF. J Thromb Haemost, 2013; 11(Suppl 2), PB 1.44-3.

Moorehead P, Thibeault L, Tuttle A, Grabell J, Dwyre L, Silva M, James PD, Lillicrap D. Rapid Immune Tolerance Induction Following Early Primary Immunologic Prophylaxis in a Hemophilia A Patient with High-Titre Inhibitor.
J Thromb Haemost, 2013; 11(Suppl 2), PO 153.

Swystun L, Rydz N, Notley C, Riches J, Paterson AD, Montgomery RR, James PD, Lillicrap D.
Genetic Variability of the CLEC4M Endothelial Lectin Receptor Modulates Binding and Internalization of Von Willebrand Factor and Contributes to Variance in Plasma VWF Levels.
Blood (ASH Annual Meeting Abstracts), Nov 2012; 120: 16.

Rimmer E, Jamieson MA, James PD. Rates of Malposition and Expulsion of Levonorgestrel Intrauterine System Among Women with Inherited Bleeding Disorders in a Canadian Centre. Blood (ASH Annual Meeting Abstracts), Nov 2012; 120: 3367.

Bowman M, Ling L, Pluthero F, Christensen H, Walker I, Kahr W, James PD. Comparative Cellular Studies of the VWF Exon 4-5 Deletion Mutation Using Patient-Derived BOEC (Blood Outgrowth Endothelial Cells) and Megakaryocytes. Blood (ASH Annual Meeting Abstracts), Nov 2012; 120: 1076.

Hawke L, Scully M-F, James PD. In Vitro and Ex Vivo Investigation of Aberrant and Alternative Splicing of von Willebrand Factor.Blood (ASH Annual Meeting Abstracts), Nov 2012; 120: 2179.

Labarque V, Blanchette V, Clark DS, James PD, Rand ML. Prospective comparison of the pediatric bleeding questionnaire (PBQ) and ISTH-BAT in children referred to a tertiary-care pediatric centre. Blood (ASH Annual Meeting Abstracts), Nov 2012; 120: 2229.

Demers C, James P, Winikoff R. Hemostatic changes during pregnancy in healthy women and in women with inherited bleeding disorders. Haemophilia 2012; 18: Suppl 3 PO-MO-266.

Deforest M, Grabell J, Tuttle A, Thibeault L, Hopman W, James P. Generation and Optimization of the Self-Administered Bleeding Assessment Tool (Self-BAT). Haemophilia 2012; 18: Suppl 3 FP-TU-04.3-4.

Rydz N, Leggo J, Tinlin S, James P, Lillicrap D and the AHCDC. The Canadian “National Program for Hemophilia Mutation Testing” database: a ten-year anniversary review. Haemophilia 2012; 18: Suppl 3 PO-MO-153.

Rydz N, Grabell J, Lillicrap D and James P. Changes in von WIllebrand factor level and activity with age in Type 1 von Willebrand disease. Haemophilia 2012; 18: Suppl 3 PO-MO-249.

Segbefia CI, Biss TT, Clark DS, James PD, Blanchette VS, Rand ML. Identification of Children with Significant Epistaxis: Comparison of the Katsanis et al Epistaxis Scoring System and the Pediatric Bleeding Quesitonnaire Epistaxis Score. ISTH Kyoto 2011 P-WE-552.

Albanez S, Grabell J, Burnett E, Sponagle K, James PD, Lillicrap D. Increased VWF Levels in Later Life are Associated with Evidence of Both Enhanced Biosynthesis and Reduced Clearance. ISTH Kyoto 2011 P-MO-444.

Rydz N, Boonyawat B, Riches J, Hopman W, Paterson A, Montgomery R, Lillicrap D, James PD. Genetic variability of the CLEC4M Endothelial Lectin Receptor in Type 1 von Willebrand Disease. ISTH Kyoto 2011 O-MO-137.

James PD, Biss TT, Clark DS, Grabell J, Riddel J, Silva M, Rapson D and Rand ML. -1 to +4 vs. 0 to +3?: Comparing scoring systems for bleeding symptoms in the Condensed MCMDM-1 VWD and the Pediatric Bleeding Questionnaires. ISTH Kyoto 2011 P-MO-455.

James P, Bowman M, Grabell J, Dwyre L, Rapson D. Prospective Validation of the Condensed MCMDM1-VWD Bleeding Questionnaire for Platelet Function Disorders. ISTH Kyoto 2011 P-WE-092.

Jackson S, Poon M-C, Grabell J, Lillicrap D and James P. The Condensed MCMDM-1 VWD Bleeding Questionnaire: Utility as a Diagnostic Tool in the Hematology Clinic. ISTH Kyoto 2011 P-TU-459.