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一个异常多中心卡斯特曼病病例与二级自身免疫性中性质衰竭

Toshiki Morimoto¹, Takako Kawaguchi¹, Kei Yamasaki¹

¹Department of Respiratory Medicine University of Occupational and Environmental Health, Japan Kitakyushu Japan.

Respirology case reports

|December 21, 2023

概括

异常多中心的卡斯特勒曼异常多中心的卡斯特勒曼

科学领域:

血液学血液学血液学
肺部病理学肺部病理学
免疫学免疫学免疫学

背景情况:

异形多中心卡斯特曼病 (iMCD) 是一种罕见的淋巴增殖性疾病.
血质并发症在IMCD中很常见,但以前没有报告过自身免疫性中性质衰竭 (AIN).
这一案例突出显示了一种罕见的iMCD表现.

研究的目的:

报告一个因二次AIN而复杂的iMCD新病例.
强调研究iMCD中性衰竭患者抗中性粒细胞抗体的重要性.

主要方法:

一个73岁的日本妇女的案例报告.
临床表现包括生产性咳和呼吸障碍.
诊断工作包括胸部计算机断层扫描 (CT),手术肺活检和淋巴结活检.

主要成果:

患者呈现了中性衰竭,肺网膜不透明度和淋巴腺病在胸部CT上.
活检证实了异常多中心卡斯特曼病 (iMCD) 与二级自身免疫中性质衰竭 (AIN).
这代表了第一个报告的因AIN而复杂的iMCD病例.

结论:

异常多中心性卡斯特曼病可以伴随或导致自身免疫性中性质衰竭.
测量抗中性粒细胞抗体对于患有IMCD和中性粒细胞衰竭的患者至关重要.
在iMCD中早期诊断和管理AIN可以改善患者的治疗结果.

关键词:

抗中性粒细胞抗体的抗体.自身免疫性中性的发生.血液学疾病血液学疾病异常多中心的卡斯特曼病.

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