{"title":"获得性血管性血友病(AvWS)","authors":"Petr Drenko, Petra Přenosilová, Alena Lavičková","doi":"10.36290/vnl.2022.055","DOIUrl":null,"url":null,"abstract":"Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder caused by von Willebrand factor (vWF) dysfunction of various types and severities. Clinical manifestations and differences in the intensity of bleeding are then given by the diversity of vWF disorders. AvWS usually occurs in adult patients with a negative personal or family history of bleeding symptoms and is associated with underlying disease. In recent years, vWF dysfunction has been most commonly attributed to cardiovascular, autoimmune, lymphoproliferative or myeloproliferative comorbidities. The true prevalence of AvWS is unknown, since many cases may be clinically or laboratory silent and remain undiagnosed. Thus, a higher incidence can be expected in the above-mentioned specific patient population. We present a case report of a 90year-old patient with the very first episode of bleeding manifestation and newly diagnosed vWF dysfunction. By describing the case, we will try to present the diagnostic and treatment options of AvWS, including their pitfalls, and draw attention to situations in which to think about AvWS.","PeriodicalId":23501,"journal":{"name":"Vnitrni lekarstvi","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Acquired von Willebrand syndrome (AvWS).\",\"authors\":\"Petr Drenko, Petra Přenosilová, Alena Lavičková\",\"doi\":\"10.36290/vnl.2022.055\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder caused by von Willebrand factor (vWF) dysfunction of various types and severities. Clinical manifestations and differences in the intensity of bleeding are then given by the diversity of vWF disorders. AvWS usually occurs in adult patients with a negative personal or family history of bleeding symptoms and is associated with underlying disease. In recent years, vWF dysfunction has been most commonly attributed to cardiovascular, autoimmune, lymphoproliferative or myeloproliferative comorbidities. The true prevalence of AvWS is unknown, since many cases may be clinically or laboratory silent and remain undiagnosed. Thus, a higher incidence can be expected in the above-mentioned specific patient population. We present a case report of a 90year-old patient with the very first episode of bleeding manifestation and newly diagnosed vWF dysfunction. By describing the case, we will try to present the diagnostic and treatment options of AvWS, including their pitfalls, and draw attention to situations in which to think about AvWS.\",\"PeriodicalId\":23501,\"journal\":{\"name\":\"Vnitrni lekarstvi\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-06-22\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Vnitrni lekarstvi\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.36290/vnl.2022.055\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Vnitrni lekarstvi","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.36290/vnl.2022.055","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder caused by von Willebrand factor (vWF) dysfunction of various types and severities. Clinical manifestations and differences in the intensity of bleeding are then given by the diversity of vWF disorders. AvWS usually occurs in adult patients with a negative personal or family history of bleeding symptoms and is associated with underlying disease. In recent years, vWF dysfunction has been most commonly attributed to cardiovascular, autoimmune, lymphoproliferative or myeloproliferative comorbidities. The true prevalence of AvWS is unknown, since many cases may be clinically or laboratory silent and remain undiagnosed. Thus, a higher incidence can be expected in the above-mentioned specific patient population. We present a case report of a 90year-old patient with the very first episode of bleeding manifestation and newly diagnosed vWF dysfunction. By describing the case, we will try to present the diagnostic and treatment options of AvWS, including their pitfalls, and draw attention to situations in which to think about AvWS.
期刊介绍:
Vnitřní lékařství je tiskovým orgánem České internistické společnosti České lékařské společnosti Jana Evangelisty Purkyně a Slovenskej internistickej spoločnosti Slovenskej lekárskej spoločnosti. Je vydáván nepřetržitě od roku 1955. Časopis vychází jako měsíčník, tedy 12krát do roka a podle potřeby jsou v běžném ročníku vydávána jeho suplementa, která jsou obsahově zaměřena k určitému tématu. Tematicky je časopis zaměřen široce na oblast interní medicíny se zvláštní pozorností ke kardiologii, diabetologii a poruchám metabolizmu.