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Tytuł pozycji:

Bleeding symptoms in patients diagnosed as type 3 von Willebrand disease: Results from 3WINTERS-IPS, an international and collaborative cross-sectional study.

Tytuł:
Bleeding symptoms in patients diagnosed as type 3 von Willebrand disease: Results from 3WINTERS-IPS, an international and collaborative cross-sectional study.
Autorzy:
Tosetto A; Hemophilia and Thrombosis Center, Hematology Department, San Bortolo Hospital, Vicenza, Italy.
Badiee Z; Hemophilia-Thalassemia Center, Mashhad University of Medical Science, Mashad, Islamic Republic of Iran.
Baghaipour MR; Iranian Hemophilia Comprehensive Treatment Centre, Tehran, Islamic Republic of Iran.
Baronciani L; Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Fondazione Luigi Villa, Milan, Italy.
Battle J; Complejo Hospitalario Universitario de A Coruña-Servicio de Hematología y Hemoterapia, A Coruña, Spain.
Berntorp E; Skane University Hospital, Malmo, Sweden.
Bodó I; Department of Internal Medicine and Hematology, Semmelweis University, Budapest, Hungary.
Budde U; MEDILYS Labor Gesellschaft, Hamburg, Germany.
Castaman G; Center for Bleeding Disorders and Coagulation, Careggi University Hospital, Florence, Italy.
Eikenboom JCJ; Division of Thrombosis and Hemostasis, Department of Internal Medicine, Leiden University Medical Center, Leiden, the Netherlands.
Eshghi P; Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Islamic Republic of Iran.
Ettorre C; Hemostasis and Thrombosis Center, Azienda Ospedaliera Policlinico Consorziale, Bari, Italy.
Goodeve A; University of Sheffield, Sheffield, UK.
Goudemand J; Department of Haematology Transfusion, University Lille-CRMW, CHRU Lille, Lille, France.
Hay CRM; Manchester Royal Infirmary, Manchester, UK.
Hoorfar H; Hemophilia Center-Esfahan University of Medical Science, Esfahan, Islamic Republic of Iran.
Karimi M; Hematology Research Center, Nemazee Hospital, Shiraz University of Medical Science, Shiraz, Islamic Republic of Iran.
Keikhaei B; Thalassemia and Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
Lassila R; Coagulation Disorders, Helsinki University Central Hospital, Helsinki, Finland.
Leebeek FWG; Erasmus Medical Center, Rotterdam, the Netherlands.
Lopez Fernandez MF; Complejo Hospitalario Universitario de A Coruña-Servicio de Hematología y Hemoterapia, A Coruña, Spain.
Mannucci PM; Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Fondazione Luigi Villa, Milan, Italy.
Mazzucconi MG; Hemophilia and Thrombosis Center-University of Rome, Rome, Italy.
Morfini M; Center for Bleeding Disorders and Coagulation, Careggi University Hospital, Florence, Italy.
Oldenburg J; Institute of Experimental Haematology and Transfusion Medicine, University of Bonn, Bonn, Germany.
Peake I; University of Sheffield, Sheffield, UK.
Parra Lòpez R; Unidad de Hemofilia-Hospital Universitari General Vall d'Hebron, Barcelona, Spain.
Peyvandi F; Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Fondazione Luigi Villa, Milan, Italy.; Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
Schneppenheim R; Department of Pediatric Hematology and Oncology, University Medical Centre, Hamburg, Germany.
Tiede A; Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany.
Toogeh G; Thrombosis Hemostasis Research Center-Vali-Asr Hospital-Emam Khameini Complex Hospital, Tehran University of Medical Science, Tehran, Islamic Republic of Iran.
Trossaert M; Centre Régional de Traitement de l'Hémophilie-Laboratoire d'Hématologie, Nantes, France.
Zekavat O; Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Islamic Republic of Iran.
Zetterberg EMK; Skane University Hospital, Malmo, Sweden.
Federici AB; Hematology and Transfusion Medicine, Department of Oncology and Oncohematology, L. Sacco University Hospital, University of Milan, Milano, Italy.
Źródło:
Journal of thrombosis and haemostasis : JTH [J Thromb Haemost] 2020 Sep; Vol. 18 (9), pp. 2145-2154. Date of Electronic Publication: 2020 Aug 25.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
Język:
English
Imprint Name(s):
Publication: 2023- : [New York] : Elsevier
Original Publication: Oxford : Blackwell Pub.
MeSH Terms:
von Willebrand Disease, Type 1*/diagnosis
von Willebrand Disease, Type 3*/diagnosis
von Willebrand Disease, Type 3*/epidemiology
von Willebrand Diseases*/diagnosis
von Willebrand Diseases*/epidemiology
Cross-Sectional Studies ; Female ; Hemarthrosis ; Humans ; von Willebrand Factor
References:
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Contributed Indexing:
Keywords: type 1 epidemiology hemorrhage blood coagulation disorders; type 3 von Willebrand disease; von Willebrand disease
Substance Nomenclature:
0 (von Willebrand Factor)
Entry Date(s):
Date Created: 20200508 Date Completed: 20210519 Latest Revision: 20230829
Update Code:
20240105
DOI:
10.1111/jth.14886
PMID:
32379400
Czasopismo naukowe
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.)
Comment in: J Thromb Haemost. 2020 Oct;18(10):2779-2780. (PMID: 33460294)

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