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原发性上肢淋巴水肿:一种罕见病理的病例报告

Primary Upper Limb Lymphedema: Case Report of a Rare Pathology.

作者信息

Mcfarlane Michael Ec

机构信息

Consultant General Surgeon and Lecturer in Surgery in the Department of Surgery, Radiology, Anaesthetics and Intensive Care at the University of the West Indies in Mona, Jamaica.

出版信息

Perm J. 2017;21:16-010. doi: 10.7812/TPP/16-010. Epub 2016 Nov 9.

DOI:10.7812/TPP/16-010
PMID:28080951
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5224806/
Abstract

INTRODUCTION

Lymphedema is characterized by a defect in the lymphatic system that causes limb swelling. Impaired uptake and transport of lymphatic fluid through lymphatic vessels causes accumulation of protein-rich fluid in the interstitial spaces, which leads to swelling of the limb. Primary lymphedema often presents at birth. The rare cases that arise after age 35 years are described as lymphedema tarda. The great majority of patients with lymphedema have swelling of the lower limbs-upper limb lymphedema is a rare disorder.

CASE PRESENTATION

An 84-year-old woman presented with a 3-year history of unilateral swelling of the right upper limb. There were no constitutional symptoms and no evidence of lymphadenopathy or systemic disease. Blood tests, carcinoembryonic antigen test, computed tomography scans, and venous Doppler ultrasound were all normal. The diagnosis was primary upper limb lymphedema.

DISCUSSION

The swelling that occurs in upper limb lymphedema is permanent and usually extends to the hand. About one-third of patients with this condition also present with lower limb lymphedema. Thorough investigations are warranted in cases of unilateral upper limb lymphedema to rule out occult malignancy and systemic disease.

摘要

引言

淋巴水肿的特征是淋巴系统存在缺陷,导致肢体肿胀。淋巴液通过淋巴管的摄取和运输受损,导致富含蛋白质的液体在间质间隙中积聚,从而导致肢体肿胀。原发性淋巴水肿通常在出生时出现。35岁以后出现的罕见病例被称为迟发性淋巴水肿。绝大多数淋巴水肿患者下肢肿胀,上肢淋巴水肿是一种罕见的疾病。

病例报告

一名84岁女性,有右侧上肢单侧肿胀3年的病史。无全身症状,无淋巴结病或全身性疾病的证据。血液检查、癌胚抗原检测、计算机断层扫描和静脉多普勒超声均正常。诊断为原发性上肢淋巴水肿。

讨论

上肢淋巴水肿出现的肿胀是永久性的,通常会延伸至手部。约三分之一患有这种疾病的患者也会出现下肢淋巴水肿。对于单侧上肢淋巴水肿病例,有必要进行全面检查以排除隐匿性恶性肿瘤和全身性疾病。