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3型血管性血友病患者的结肠癌手术引发肠吻合处血管异常。

Colon cancer surgery in von Willebrand disease type 3 setting triggering vascular abnormalities on bowel anastomosis.

作者信息

Rauch Antoine, Paris Camille, Daniel Mélanie, Branche Julien, Goudemand Jenny, Susen Sophie

机构信息

Hematology and Transfusion Department, Centre Hospitalier Universitaire de Lille, Lille, France.

Gastroenterology Department, Centre Hospitalier Universitaire de Lille, Lille, France.

出版信息

Res Pract Thromb Haemost. 2023 Jun 10;7(5):100277. doi: 10.1016/j.rpth.2023.100277. eCollection 2023 Jul.

Abstract

BACKGROUND

von Willebrand disease (VWD) is associated with vascular malformations in the gastrointestinal tract. This complication, more frequent in VWD types 2A and 3, may be due to abnormal angiogenesis, but the precise mechanism is still unclear. Angiogenesis and inflammation are closely linked and can potentiate each other.

KEY CLINICAL QUESTION

Can colon inflammation in the setting of cancer surgery potentiate angiogenesis in the VWD setting?

CLINICAL APPROACH

A woman with VWD type 3 underwent partial colectomy twice for an adenocarcinoma. After managing the first surgery with a plasma-derived von Willebrand factor (VWF) concentrate (Wilfactin; LFB), refractory gastrointestinal bleeding occurred from neovessels on bowel anastomosis. After a multidisciplinary discussion, a second surgery was undertaken with a recombinant VWF concentrate (Veyvondi; Takeda Pharmaceuticals). Pathologic neovessels were again observed on the new anastomosis.

CONCLUSION

Colectomy was complicated twice by pathologic neovessels on bowel anastomosis in 2 distinct procedures managed either with plasma-derived VWF or with recombinant VWF.

摘要

背景

血管性血友病(VWD)与胃肠道血管畸形有关。这种并发症在2A型和3型VWD中更为常见,可能是由于异常血管生成所致,但确切机制仍不清楚。血管生成与炎症密切相关,且可相互促进。

关键临床问题

癌症手术背景下的结肠炎症能否在VWD背景下促进血管生成?

临床方法

一名3型VWD女性因腺癌接受了两次部分结肠切除术。在首次手术中使用血浆源性血管性血友病因子(VWF)浓缩物(Wilfactin;LFB)处理后,肠吻合口新生血管出现难治性胃肠道出血。经过多学科讨论,第二次手术使用重组VWF浓缩物(Veyvondi;武田制药)。在新的吻合口再次观察到病理性新生血管。

结论

在分别使用血浆源性VWF或重组VWF处理的2次不同手术中,结肠切除术后均出现了肠吻合口病理性新生血管的并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/025a/10439390/957bcb745eb3/gr1.jpg

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