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目的 探讨轻链型系统性淀粉样变的临床表现、诊断、鉴别诊断及治疗、预后，提高对系统性淀粉样变的临床认识。方法 报道2022年武汉市第四医院肿瘤内科收治的1例以舌损害为首发表现的累及多器官的系统性淀粉样变患者，并对该疾病相关临床内容进行分析。结果 64岁男性患者，临床表现为双颊及舌部发硬。行一系列相关检查确诊为“舌淀粉样变性”，后予以沙利度胺+泼尼松联合治疗。结论 淀粉样变性的临床表现多样，可累及多个器官，总体预后差。大部分临床表现无特异性，但舌体肥大和眶周紫癜是轻链型淀粉样变性较为特异的临床表现。临床上常需与M蛋白血症，如多发性骨髓瘤相鉴别。诊断上主要依靠活检组织病理学检查。治疗方案以化疗为主。
Abstract：Objective To explore the clinical manifestation,diagnosis,differential diagnosis,treatment,and prognosis of light chain systemic amyloidosis,and to improve the clinical understanding of systemic amyloidosis. Methods A case of systemic amyloidosis involving multiple organs with tongue damage as the first manifestation in the Department of Oncology of the Fourth Hospital of Wuhan in 2022 was reported,and the related clinical contents of the disease were analyzed. Results A 64-year-old male patient presented with stiff cheeks and tongue. Tongue amyloidosis was diagnosed by a series of related examinations and then treated with thalidomide and prednisone. Conclusion Amyloidosis has a variety of clinical manifestations and can involve multiple organs. The prognosis is poor. Most clinical manifestations are not specific,but tongue hypertrophy and periorbital purpura are more specific clinical manifestations of light chain amyloidosis. Clinically,it is often differentiated from M-proteinemia,such as multiple myeloma. The diagnosis mainly depends on the pathological examination of biopsy tissue. Chemotherapy is the main treatment.
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基本信息:
DOI：10.16389/j.cnki.cn42-1737/n.2022.06.010
中图分类号:R597.2
引用信息:
[1]邓子祥,李敏,桂华伟等.以舌损害为首发表现的1例系统性轻链型淀粉样变并临床分析[J].江汉大学学报(自然科学版),2022,50(06):86-90.DOI:10.16389/j.cnki.cn42-1737/n.2022.06.010.
基金信息: