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References 1. Shetty S, Kasatkar P, Ghosh K. Pathophysiology of acquired von Willebrand disease: a concise review. Eur J Haematol 2011; 87(2):99–106. DOI: 10.1111/j.1600-0609.2011.01636.x 2. Franchini M, Lippi G. Acquired von Willebrand syndrome: an update. Am J Hematol. 2007;82(5):368–75. DOI: 10.1002/ajh.20830 3. Mohri H. Acquired von Willebrand syndrome: Its pathophysiology, laboratory features and management. J Thromb Thrombolysis. 2003;15(3):141–9. DOI: 10.1023/B:THRO.0000011369.70824.e6 4. Mohri H. Acquired von Willebrand syndrome: features and management

REFERENCES 1. Favaloro EJ, Pasalic L, Curnow J. Laboratory tests used to help diagnose von Willebrand disease: an update. Pathology 2016; 48(4): 303–318. 2. Kubisz P, Sokol J, Simurda T, et al. Diagnosis and management of von Willebrand disease in Slovakia; Ann Blood 2018;3:9. 3. Penka M, Tesarova E et al. Hematologie a transfúzní lekářství I. Bratislava: Grada Slovakia; 2011, 421 s. 4. Stockschlaeder M, Scheppenheim R, Budde, U. Update on von Willebrand factor multimers: focus on high-molecular-weight multimers and their role in hemostasis. Blood Coagul

Von Willebrand disease (VWD) is the most common hereditary coagulation abnormality, presenting in an estimated 1% of the population [ 1 , 2 , 3 , 4 , 5 , 6 ] . In those with VWD, there is a deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that controls platelet haemostatic function and stabilises blood clotting with factor VIII (FVIII). Those with VWD often experience symptoms related to mucosal bleeding, with VWF levels greatly affecting the amount of bleeding experienced. Symptoms include epistaxis, gingival bleeding, menorrhagia

References Ewenstein BM. Von Willebrand's disease. Annu Rev Med 1997; 48: 525-42. Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease. Blood 1987; 69: 454-9. Federici AB, Santagostino E, Rumi MG, Russo A, Mancuso ME, Soffredini R, et al. The natural history of hepatitis C virus infection in Italian patients with von Willebrand's disease: a cohort study. Haematologica 2006; 91: 503-8. Manns MP, McHutchison JG, Gordon SC, Rustgi VK, Shiffman M, Reindollar R, et al. Peginterferon alfa-2b plus ribavirin

’s disease Blood 1987 69 454 9 3 Werner EJ, Broxson EH, Tucker EL, et al. Prevalence of von Willebrand disease in children: a multi-ethnic study. J Pediatr 1993; 123: 893-8. 10.1016/S0022-3476(05)80384-1 Werner EJ Broxson EH Tucker EL et al. Prevalence of von Willebrand disease in children: a multi-ethnic study J Pediatr 1993 123 893 8 4 Laffan MA, Lester W, O’Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J

Type 3 von Willebrand disease is a rare, severe bleeding disorder requiring the inheritance of two von Willebrand factor gene mutations. As such, it is an autosomal recessive disorder with patients either being homozygous for a VWF mutation (more commonly seen when parents are consanguineous) or being compound heterozygotes [ 1 , 7 ]. The disorder is characterised by a complete quantitative deficiency of von Willebrand factor (VWF) in plasma [ 1 , 7 ]. The incidence of Type 3 VWD is 0.1-5.3 per million and varies by region [ 2 ]. The highest prevalence of Type 3

function is not dependent on VWF size. The multiple binding sites, assembly into large multimers and requirement for shear stress for optimum function make VWF a complicated molecule to assess in the laboratory. Moreover, these multiple interactions, multimeric structure and the simple fact that the VWF gene is very large mean that there are many different ways in which the VWF molecule can malfunction. These different forms of VWF deficiency form the basis of the Von Willebrand disease (VWD) classification. A discussion of these problems and a practical approach to

paediatric team urgently with the results of his initial blood tests, which showed that he was severely anaemic (Hb 23 g/L). Congenital anaemia and haemolysis were excluded as he had been well at birth and was not jaundiced. Clinical examination and ultrasound scans performed in the resuscitation room did not identify a clear source of bleeding. However, his coagulation screen showed a disproportionately prolonged APTT ( Table 1 ) raising the suspicion of severe occult bleeding due to haemophilia or, possibly, severe von Willebrand disease. He was treated with packed red

responses of WBD to the survey has been published [ 1 ] . Women with bleeding disorders Responses were received from 709 WBD, of whom 94% were from Western Europe, 4% from Central Europe and 2% from Eastern Europe. Over half (56%) were aged 19–45, 30% were 46–60 years old, 10% were aged 61 or older, and 4% were under 18. The largest groups were women with von Willebrand disease (VWD, 28%) or who described themselves as carriers of haemophilia with factor level >40% (24%) or <40% (27%); approximately 9% of respondents had a platelet disorder (see Tables 1A - 1C) . Table

The WFH and women with bleeding disorders The 2017 World Federation of Hemophilia (WFH) annual global survey included over 315,000 people with a bleeding disorder [ 1 ] . The results revealed a marked disparity in apparent gender distribution between haemophilia on one hand and von Willebrand disease and other bleeding disorders on the other. According to these data, women account for only 3% of people with haemophilia A and 5% of those with haemophilia B, whereas 61% of people with von Willebrand disease (VWD) are women. The figures for haemophilia are