Bleeding Symptoms in Patients Diagnosed As Type 3 Von Willebrand Disease: Results From 3Winters-Ips, an International and Collaborative Cross-Sectional Study

Bleeding Symptoms in Patients Diagnosed As Type 3 Von Willebrand Disease: Results From 3Winters-Ips, an International and Collaborative Cross-Sectional Study Publisher Pubmed

Tosetto A¹ ; Badiee Z² ; Baghaipour MR³ ; Baronciani L⁴ ; Battle J⁵ ; Berntorp E⁶ ; Bodo I⁷ ; Budde U⁸ ; Castaman G⁹ ; Eikenboom JCJ¹⁰ ; Eshghi P¹¹ ; Ettorre C¹² ; Goodeve A¹³ ; Goudemand J¹⁴ Show All Authors

Tosetto A¹
Badiee Z²
Baghaipour MR³
Baronciani L⁴
Battle J⁵
Berntorp E⁶
Bodo I⁷
Budde U⁸
Castaman G⁹
Eikenboom JCJ¹⁰
Eshghi P¹¹
Ettorre C¹²
Goodeve A¹³
Goudemand J¹⁴
Hay CRM¹⁵
Hoorfar H¹⁶
Karimi M¹⁷
Keikhaei B¹⁸
Lassila R¹⁹
Leebeek FWG²⁰
Lopez Fernandez MF⁵
Mannucci PM⁴
Mazzucconi MG²¹
Morfini M⁹
Oldenburg J²²
Peake I¹³
Parra Lopez R²³
Peyvandi F^{4, 24}
Schneppenheim R²⁵
Tiede A²⁶
Toogeh G²⁷
Trossaert M²⁸
Zekavat O²⁹
Zetterberg EMK⁶
Federici AB³⁰

Show Affiliations

1. Hemophilia and Thrombosis Center, Hematology Department, San Bortolo Hospital, Vicenza, Italy
2. Hemophilia-Thalassemia Center, Mashhad University of Medical Science, Mashad, Iran
3. Iranian Hemophilia Comprehensive Treatment Centre, Tehran, Iran
4. Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico and Fondazione Luigi Villa, Milan, Italy
5. Complejo Hospitalario Universitario de A Coruna—Servicio de Hematologia y Hemoterapia, A Coruna, Spain
6. Skane University Hospital, Malmo, Sweden
7. Department of Internal Medicine and Hematology, Semmelweis University, Budapest, Hungary
8. MEDILYS Labor Gesellschaft, Hamburg, Germany
9. Center for Bleeding Disorders and Coagulation, Careggi University Hospital, Florence, Italy
10. Division of Thrombosis and Hemostasis, Department of Internal Medicine, Leiden University Medical Center, Leiden, Netherlands
11. Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
12. Hemostasis and Thrombosis Center, Azienda Ospedaliera Policlinico Consorziale, Bari, Italy
13. University of Sheffield, Sheffield, United Kingdom
14. Department of Haematology Transfusion, University Lille—CRMW, CHRU Lille, Lille, France
15. Manchester Royal Infirmary, Manchester, United Kingdom
16. Hemophilia Center—Esfahan University of Medical Science, Esfahan, Iran
17. Hematology Research Center, Nemazee Hospital, Shiraz University of Medical Science, Shiraz, Iran
18. Thalassemia and Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
19. Coagulation Disorders, Helsinki University Central Hospital, Helsinki, Finland
20. Erasmus Medical Center, Rotterdam, Netherlands
21. Hemophilia and Thrombosis Center—University of Rome, Rome, Italy
22. Institute of Experimental Haematology and Transfusion Medicine, University of Bonn, Bonn, Germany
23. Unidad de Hemofilia—Hospital Universitari General Vall d'Hebron, Barcelona, Spain
24. Department of Pathophysiology and Transplantation, Universita degli Studi di Milano, Milan, Italy
25. Department of Pediatric Hematology and Oncology, University Medical Centre, Hamburg, Germany
26. Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany
27. Thrombosis Hemostasis Research Center—Vali-Asr Hospital—Emam Khameini Complex Hospital, Tehran University of Medical Science, Tehran, Iran
28. Centre Regional de Traitement de l’Hemophilie—Laboratoire d’Hematologie, Nantes, France
29. Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
30. Hematology and Transfusion Medicine, Department of Oncology and Oncohematology, L. Sacco University Hospital, University of Milan, Milano, Italy

Source: Journal of Thrombosis and Haemostasis Published:2020

Abstract

Background: Type 3 von Willebrand's disease (VWD) patients present markedly reduced levels of von Willebrand factor and factor VIII. Because of its rarity, the bleeding phenotype of type 3 VWD is poorly described, as compared to type 1 VWD. Aims: To evaluate the frequency and the severity of bleeding symptoms across age and sex groups in type 3 patients and to compare these with those observed in type 1 VWD patients to investigate any possible clustering of bleeding symptoms within type 3 patients. Methods: We compared the bleeding phenotype and computed the bleeding score (BS) using the MCMDM-1VWD bleeding questionnaire in patients enrolled in the 3WINTERS-IPS and MCMDM-1VWD studies. Results: In 223 unrelated type 3 VWD patients, both the BS and the number of clinically relevant bleeding symptoms were increased in type 3 as compared to type 1 VWD patients (15 versus 6 and 5 versus 3). Intracranial bleeding, oral cavity, hemarthroses, and deep hematomas were at least five-fold over-represented in type 3 VWD. A more severe bleeding phenotype was evident in patients having von Willebrand factor antigen levels < 20 IU/dL at diagnosis in the two merged cohorts. In type 3 patients, there was an apparent clustering of hemarthrosis with gastrointestinal bleeding and epistaxis, whereas bleeding after surgery or tooth extraction clusters with oral bleeding and menorrhagia. Conclusions: In the largest cohort of type 3 VWD patients, we were able to describe a distinct clinical phenotype that is associated with the presence of a more severe hemostatic defect. © 2020 International Society on Thrombosis and Haemostasis

2. The Prevalence of Von Willebrand Disease in Patients With Menorrhagia, Journal of Isfahan Medical School (2018)

3. Impact of Factor Viii and Von Willebrand Factor Plasma Levels on Cerebral Venous and Sinus Thrombosis: Are They Independent Risk Factors?, European Neurology (2011)

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Fariba Behnamfar (1)

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Style	Citing Format
MLA	Tosetto A, et al.. "Bleeding Symptoms in Patients Diagnosed As Type 3 Von Willebrand Disease: Results From 3Winters-Ips, an International and Collaborative Cross-Sectional Study." Journal of Thrombosis and Haemostasis, vol. 18, no. 9, 2020, pp. 2145-2154.
APA	Tosetto A, Badiee Z, Baghaipour MR, Baronciani L, Battle J, Berntorp E, Bodo I, Budde U, Castaman G, Eikenboom JCJ, Eshghi P, Ettorre C, Goodeve A, Goudemand J, Hay CRM, Hoorfar H, Karimi M, Keikhaei B, Lassila R, ... Federici AB (2020). Bleeding Symptoms in Patients Diagnosed As Type 3 Von Willebrand Disease: Results From 3Winters-Ips, an International and Collaborative Cross-Sectional Study. Journal of Thrombosis and Haemostasis, 18(9), 2145-2154.
Chicago	Tosetto A, Badiee Z, Baghaipour MR, Baronciani L, Battle J, Berntorp E, Bodo I, et al.. "Bleeding Symptoms in Patients Diagnosed As Type 3 Von Willebrand Disease: Results From 3Winters-Ips, an International and Collaborative Cross-Sectional Study." Journal of Thrombosis and Haemostasis 18, no. 9 (2020): 2145-2154.
Harvard	Tosetto A et al. (2020) 'Bleeding Symptoms in Patients Diagnosed As Type 3 Von Willebrand Disease: Results From 3Winters-Ips, an International and Collaborative Cross-Sectional Study', Journal of Thrombosis and Haemostasis, 18(9), pp. 2145-2154.
Vancouver	Tosetto A, Badiee Z, Baghaipour MR, Baronciani L, Battle J, Berntorp E, et al.. Bleeding Symptoms in Patients Diagnosed As Type 3 Von Willebrand Disease: Results From 3Winters-Ips, an International and Collaborative Cross-Sectional Study. Journal of Thrombosis and Haemostasis. 2020;18(9):2145-2154.
BibTex	@article{ author = {Tosetto A and Badiee Z and Baghaipour MR and Baronciani L and Battle J and Berntorp E and Bodo I and Budde U and Castaman G and Eikenboom JCJ and Eshghi P and Ettorre C and Goodeve A and Goudemand J and Hay CRM and Hoorfar H and Karimi M and Keikhaei B and Lassila R and Leebeek FWG and Lopez Fernandez MF and Mannucci PM and Mazzucconi MG and Morfini M and Oldenburg J and Peake I and Parra Lopez R and Peyvandi F and Schneppenheim R and Tiede A and Toogeh G and Trossaert M and Zekavat O and Zetterberg EMK and Federici AB}, title = {Bleeding Symptoms in Patients Diagnosed As Type 3 Von Willebrand Disease: Results From 3Winters-Ips, an International and Collaborative Cross-Sectional Study}, journal = {Journal of Thrombosis and Haemostasis}, volume = {18}, number = {9}, pages = {2145-2154}, year = {2020} }
RIS	TY - JOUR AU - Tosetto A AU - Badiee Z AU - Baghaipour MR AU - Baronciani L AU - Battle J AU - Berntorp E AU - Bodo I AU - Budde U AU - Castaman G AU - Eikenboom JCJ AU - Eshghi P AU - Ettorre C AU - Goodeve A AU - Goudemand J AU - Hay CRM AU - Hoorfar H AU - Karimi M AU - Keikhaei B AU - Lassila R AU - Leebeek FWG AU - Lopez Fernandez MF AU - Mannucci PM AU - Mazzucconi MG AU - Morfini M AU - Oldenburg J AU - Peake I AU - Parra Lopez R AU - Peyvandi F AU - Schneppenheim R AU - Tiede A AU - Toogeh G AU - Trossaert M AU - Zekavat O AU - Zetterberg EMK AU - Federici AB TI - Bleeding Symptoms in Patients Diagnosed As Type 3 Von Willebrand Disease: Results From 3Winters-Ips, an International and Collaborative Cross-Sectional Study JO - Journal of Thrombosis and Haemostasis VL - 18 IS - 9 SP - 2145 EP - 2154 PY - 2020 ER -

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