دورية أكاديمية

Von Willebrand Factor Multimeric Assay in Acquired von Willebrand Disease Diagnosis: A Report of Experience from North Estonia Medical Centre.

التفاصيل البيبلوغرافية
العنوان: Von Willebrand Factor Multimeric Assay in Acquired von Willebrand Disease Diagnosis: A Report of Experience from North Estonia Medical Centre.
المؤلفون: Pikta M; Department of Health Technologies, Tallinn University of Technology, Tallinn, Estonia.; Department of Laboratory Medicine, North Estonia Medical Centre, Tallinn, Estonia., Banys V; Department of Physiology, Biochemistry, Microbiology and Laboratory Medicine, Faculty of Medicine, Institute of Biomedical Sciences, Vilnius University, Vilnius, Lithuania., Szanto T; Research Program Unit of Systems Oncology, Helsinki University Hospital, Unit of Coagulation Disorders, Helsinki, Finland., Joutsi-Korhonen L; Department of Clinical Chemistry, Unit of Coagulation Disorders, HUSLAB Laboratory Services, Helsinki University Hospital and University of Helsinki, Helsinki, Finland., Vaide I; Hemato-Oncology Clinic, Institute of Clinical Medicine, Tartu University, Tartu, Estonia., Varik M; Hematology Department, North Estonia Medical Centre, Tallinn, Estonia., Lehtinen AE; Department of Hematology, Unit of Coagulation Disorders, Helsinki University Hospital Comprehensive Cancer Center and University of Helsinki, Helsinki, Finland., Giangrande P; Green Templeton College, University of Oxford, Oxford, United Kingdom., Laane E; Hemato-Oncology Clinic, Institute of Clinical Medicine, Tartu University, Tartu, Estonia.
المصدر: Journal of laboratory physicians [J Lab Physicians] 2021 Sep; Vol. 13 (3), pp. 195-201. Date of Electronic Publication: 2021 Jun 28.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Thieme Country of Publication: Germany NLM ID: 101551511 Publication Model: Print-Electronic Cited Medium: Print ISSN: 0974-2727 (Print) Linking ISSN: 09742727 NLM ISO Abbreviation: J Lab Physicians Subsets: PubMed not MEDLINE
أسماء مطبوعة: Publication: 2020- : [Stuttgart] : Thieme
Original Publication: Mumbai : Medknow Publications
مستخلص: Objectives Acquired von Willebrand syndrome (AVWS) is a rare and frequently underdiagnosed bleeding disorder with an unknown prevalence. The diagnosis of AVWS is made based on laboratory investigations and the presence of clinical symptoms. Evaluation and management of affected patients are complex due to the need for multiple laboratory assays. Materials and Methods Here, we describe the clinical and laboratory data of seven patients with a diagnosis of AVWS. All patients met the criteria for AVWS based on laboratory findings, bleeding symptoms, and the absence of any previous history of a bleeding disorder. Results In all cases, the laboratory findings, lack of bleeding anamnesis, and family history suggested the presence of AVWS. Von Willebrand factor multimeric analysis showed decreased high-molecular weight (HMW) multimers in six cases. Patients with lower HMW multimers experienced more severe bleeding complications. Conclusions The diagnosis of AVWS is complex and requires extensive laboratory evaluation. Interdisciplinary collaboration and complex laboratory evaluations are of paramount importance for the early recognition of AVWS and optimal AVWS diagnosis as well as successful clinical management.
Competing Interests: Conflicts of Interests None declared.
(The Indian Association of Laboratory Physicians. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)
References: Haemophilia. 2016 Jul;22 Suppl 5:54-9. (PMID: 27405677)
Methods Mol Biol. 2017;1646:495-511. (PMID: 28804850)
Blood. 2011 Jun 23;117(25):6777-85. (PMID: 21540459)
Eur J Haematol. 2011 Aug;87(2):99-106. (PMID: 21535159)
Haemophilia. 2008 Jul;14 Suppl 3:49-55. (PMID: 18510522)
Am J Hematol. 2007 May;82(5):368-75. (PMID: 17133419)
Am J Clin Pathol. 1987 Jul;88(1):102-6. (PMID: 3496780)
J Thromb Haemost. 2011 Aug;9(8):1645-7. (PMID: 21679294)
Blood. 1984 Sep;64(3):614-21. (PMID: 6432075)
Lancet. 1987 Jun 6;1(8545):1314-5. (PMID: 2884431)
Haemophilia. 1999 Sep;5(5):318-26. (PMID: 10583513)
J Med Biochem. 2021 Mar 12;40(2):167-172. (PMID: 33776566)
J Thromb Haemost. 2008 Apr;6(4):569-76. (PMID: 18208537)
Semin Thromb Hemost. 1997;23(5):425-31. (PMID: 9387201)
Pol Arch Med Wewn. 2015;125(12):914-20. (PMID: 26658493)
Blood. 1998 Oct 15;92(8):2707-11. (PMID: 9763553)
Haemophilia. 2017 Jul;23(4):e373-e377. (PMID: 28497866)
Semin Thromb Hemost. 2006 Jul;32(5):514-21. (PMID: 16862525)
Best Pract Res Clin Haematol. 2001 Jun;14(2):401-36. (PMID: 11686107)
Br J Haematol. 1985 Oct;61(2):307-14. (PMID: 3929829)
Hamostaseologie. 2004 Feb;24(1):50-5. (PMID: 15029273)
J Clin Lab Anal. 2018 Jul;32(6):e22416. (PMID: 29453814)
Adv Clin Exp Med. 2016 Nov-Dec;25(6):1337-1344. (PMID: 28028990)
Res Pract Thromb Haemost. 2018 Aug 12;2(4):790-799. (PMID: 30349898)
Int J Lab Hematol. 2018 Oct;40(5):586-591. (PMID: 29920949)
Thromb Haemost. 2000 Aug;84(2):345-9. (PMID: 10959711)
فهرسة مساهمة: Keywords: acquired von Willebrand syndrome; von Willebrand factor; von Willebrand factor multimers
تواريخ الأحداث: Date Created: 20211004 Latest Revision: 20211005
رمز التحديث: 20221213
مُعرف محوري في PubMed: PMC8478502
DOI: 10.1055/s-0041-1730818
PMID: 34602781
قاعدة البيانات: MEDLINE
الوصف
تدمد:0974-2727
DOI:10.1055/s-0041-1730818