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原发性免疫缺陷病成人患者的回顾性评估

Retrospective evaluation of adults with primary immunodeficiency disease.

作者信息

Özdemir Ebru

机构信息

Division of Allergy and Immunology, Malatya Training and Research Hospital, Malatya, Turkey.

出版信息

Postepy Dermatol Alergol. 2022 Oct;39(5):976-979. doi: 10.5114/ada.2022.120887. Epub 2022 Nov 9.

Abstract

INTRODUCTION

Primary immunodeficiency diseases (PIDs) are a group of heterogeneous disorders that result from one or more immune system abnormalities and have a wide range of clinical manifestations.

AIM

To evaluate the demographic, clinical, and radiological features of adult patients with PID.

MATERIAL AND METHODS

We retrospectively reviewed the data of adult patients with PID who had been receiving immunoglobulin therapy at the adult allergy and immunology outpatient clinic between November 2017 and April 2022.

RESULTS

Of the 23 patients included, 4 (17.4%) were females and 19 (82.6%) were males. The mean age was 38.7 ±16.2 (range: 18-75) years. Time of delay in the diagnosis of immunodeficiency was 14.9 ±15.7 (range: 0.5-49) years. The most common complaint on admission was sinopulmonary infection, and 65% of the patients had bronchiectasis. A total of 6 patients had a history of lymphoma, and 3 of them were diagnosed during the study period. Recurrence of lymphoma was observed in 1 patient.

CONCLUSIONS

Patients with PID frequently have findings related to more than one organ system, and the diagnosis is often delayed in adults. Recognition and increased awareness of these manifestations is essential for early diagnosis and reducing morbidity and mortality.

摘要

引言

原发性免疫缺陷病(PIDs)是一组异质性疾病,由一种或多种免疫系统异常引起,临床表现广泛。

目的

评估成年原发性免疫缺陷病患者的人口统计学、临床和放射学特征。

材料与方法

我们回顾性分析了2017年11月至2022年4月期间在成人过敏与免疫门诊接受免疫球蛋白治疗的成年原发性免疫缺陷病患者的数据。

结果

纳入的23例患者中,4例(17.4%)为女性,19例(82.6%)为男性。平均年龄为38.7±16.2岁(范围:18 - 75岁)。免疫缺陷诊断延迟时间为14.9±15.7年(范围:0.5 - 49年)。入院时最常见的主诉是鼻窦肺部感染,65%的患者有支气管扩张。共有6例患者有淋巴瘤病史,其中3例在研究期间被诊断。1例患者观察到淋巴瘤复发。

结论

原发性免疫缺陷病患者常出现与多个器官系统相关的表现,成人诊断往往延迟。认识并提高对这些表现的认识对于早期诊断以及降低发病率和死亡率至关重要。

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