呼吸系统原发性淀粉样变9例临床病理分析

Primary amyloidosis of the respiratory system:a clinicopathological analysis of 9 cases

目的探讨呼吸系统原发性淀粉样变的临床特点及病理特征,为临床诊治提供依据。方法回顾性分析该院2014-01~2019-12收治的9例原发性淀粉样变患者的临床资料,总结其临床特点、病理特征及其与症状表现、预后的关联情况。结果9例患者中,男3例,女6例,中位年龄65岁。6例呼吸道病变呈局灶性,3例病变呈弥漫性分布。苏木精-伊红(HE)染色结果显示粉样沉积物呈粉红色、无定形结构,有特征性裂纹。刚果红染色显示淀粉样物呈砖红色,偏振光显微镜下显示特征性“苹果绿”双折光。免疫组化染色提示,沉积物周围浆细胞限制性表达免疫球蛋白轻链。结论呼吸系统原发性淀粉样变临床表现和辅助检查缺乏特异性,预后与病变类型有关。病理活检是确诊该病的可靠方法。

Objective To explore the clinical and pathological features of primary amyloidosis of the respiratory system,and to provide evidence for clinical diagnosis and treatment.Methods The clinical data of 9 patients with primary amyloidosis in our hospital from January 2014 to December 2019 were retrospectively analyzed.The clinical and pathological characteristics,as well as the correlation between these characteristics and symptoms and prognosis were summarized.Results Of the 9 cases,3 cases were male and 6 cases were female,with a median age of 65 years.Six cases of respiratory tract lesions were focal and 3 cases were diffusely distributed.The results of hematoxylin-eosin(HE)staining showed that the powder-like sediments were pink with amorphous structures and characteristic cracks.Congo red staining showed that the amyloid was brick red,and the characteristic“apple green”birefringence was displayed under a polarized light microscope.Immunohistochemical staining suggested that plasma cells around the sediment restrictedly expressed immunoglobulin light chains.Conclusion The clinical manifestations and auxiliary examinations of primary amyloidosis of the respiratory system lack specificity,and the prognosis is related to the type of the lesions.Pathological biopsy is a reliable diagnostic method to confirm the disease.