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脾边缘区淋巴瘤所致获得性C1抑制物缺乏症:病例报告

Acquired C1-inhibitor deficiency due to splenic marginal zone lymhoma: Case Report.

作者信息

Verschueren Jolien, Schrijvers Rik, Goffin Karolien E, Put Natalie, Tousseyn Thomas, Dierickx Daan, Gheysens Olivier

机构信息

Nuclear Medicine and Molecular Imaging, University Hospitals Leuven, Leuven, Belgium.

General Internal Medicine, University Hospitals Leuven, Leuven, Belgium.

出版信息

Acta Clin Belg. 2021 Oct;76(5):402-405. doi: 10.1080/17843286.2020.1746552. Epub 2020 Mar 31.

DOI:10.1080/17843286.2020.1746552
PMID:32228367
Abstract

We present the case of a 67-year-old woman who suffered recurrent episodes of angioedema of the face and larynx. After thorough biochemical investigations, an acquired deficiency of C1-INH was suspected. To evaluate a potential underlying malignancy, a whole-body FDG-PET/CT was performed and showed solely a marked splenomegaly pointing towards a splenic marginal zone lymphoma, which was confirmed by pathological examination.With this case, we discuss the pathophysiology, diagnosis and management of recurrent acquired angioedema attacks as the first presentation of an underlying lymphoproliferative disease.

摘要

我们报告了一例67岁女性患者,她反复出现面部和喉部血管性水肿。经过全面的生化检查,怀疑是获得性C1-INH缺乏症。为了评估潜在的潜在恶性肿瘤,进行了全身FDG-PET/CT检查,结果仅显示明显的脾肿大,提示为脾边缘区淋巴瘤,病理检查证实了这一点。通过这个病例,我们讨论了复发性获得性血管性水肿发作作为潜在淋巴增殖性疾病的首发表现的病理生理学、诊断和管理。

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