获得性血管性血友病(AvWS)

Q4 Medicine Vnitrni lekarstvi Pub Date : 2022-06-22 DOI:10.36290/vnl.2022.055
Petr Drenko, Petra Přenosilová, Alena Lavičková
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引用次数: 0

摘要

获得性血管性血友病(AvWS)是由血管性血友病因子(vWF)功能障碍引起的一种罕见的出血性疾病。临床表现和出血强度的差异由vWF疾病的多样性给出。AvWS通常发生在没有出血症状的个人或家族史的成人患者中,并与潜在疾病有关。近年来,vWF功能障碍最常见的原因是心血管、自身免疫、淋巴增生性或骨髓增生性合并症。AvWS的真实患病率尚不清楚,因为许多病例可能在临床上或实验室中未被诊断出来。因此,在上述特定患者人群中可以预期较高的发病率。我们报告了一例90岁的患者,首次出现出血表现和新诊断的vWF功能障碍。通过对该病例的描述,我们将尝试介绍AvWS的诊断和治疗方案,包括其陷阱,并提请注意考虑AvWS的情况。
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Acquired von Willebrand syndrome (AvWS).
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.
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来源期刊
Vnitrni lekarstvi
Vnitrni lekarstvi Medicine-Internal Medicine
CiteScore
0.50
自引率
0.00%
发文量
104
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.
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