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[获得性血管性血友病]

[Acquired von Willebrand's disease].

作者信息

Berentsen S, Hammerstrøm J

机构信息

Medisinsk avedeling, Regionsykehuset i Trondheim.

出版信息

Tidsskr Nor Laegeforen. 1993 May 10;113(12):1463-5.

PMID:8332973
Abstract

We describe a patient with acquired von Willebrand's disease and Waldenström's macroglobulinemia. He suffered from an acquired tendency to bleed. The laboratory findings were identical to those of congenital von Willebrand's disease. The acquired form of this bleeding disorder is seen in association with immunologically active B cell lymphomas, certain other malignant tumours, and autoimmune diseases. The incidence and prevalence are unknown, but this disorder is probably uncommon. We discuss possible pathogenetic mechanisms. Acquired von Willebrand's disease should be considered in patients with an acquired tendency to bleed, especially if the patient also has a lymphoproliferative, neoplastic, or autoimmune disease.

摘要

我们描述了一位患有获得性血管性血友病和华氏巨球蛋白血症的患者。他有后天性出血倾向。实验室检查结果与先天性血管性血友病相同。这种出血性疾病的获得性形式与免疫活性B细胞淋巴瘤、某些其他恶性肿瘤以及自身免疫性疾病有关。其发病率和患病率尚不清楚,但这种疾病可能并不常见。我们讨论了可能的发病机制。对于有后天性出血倾向的患者,尤其是同时患有淋巴增殖性、肿瘤性或自身免疫性疾病的患者,应考虑获得性血管性血友病。

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