一例以胸闷气短为首发症状的系统性轻链（AL）型淀粉样变性个案报道

轻链型(AL)系统样变性是由单克隆免疫球蛋白轻链错误折叠形成淀粉样蛋白,沉积于气管,造成组织结构破坏、气管功能障碍并进行性进展的疾病,主要与克隆性浆细胞异常增殖有关。免疫球蛋白轻链（AL）型淀粉样变性是浆细胞病的一种罕见类型,个体差异大,疾病的首发起病症状不一,且舌体肥大和眶周紫癜是AL型淀粉样变性的特异性表现3]。这是我们报道这例以胸闷气短为首发症状的免疫球蛋白轻链（AL)型淀粉样变性的主要原因。病例报告：我们报告一例59岁女性患者,以活动后乏力、气短4年余,双下肢水肿,夜间不能平卧起病,患者病史多年,且一直以“胸闷气短”为主要症状,并伴有舌体肥大,在按照心内科常规治疗情况下未见好转,经过多学科会诊及讨论,建议行舌体活检及骨髓穿刺考虑为系统性轻链型（AL）型淀粉样变性。明确诊断后启动化疗,现患者胸闷气短症状明显好转,且舌体较前缩小。结论:以“胸闷气短”为首发症状,且症状反复,心内科治疗效果欠佳,并伴有多系统累及,应考虑该病的诊断且尽快行相关的专科检查,主要依靠组织病理学检查。

A case report of systemic light chain (AL) amyloidosis with chest tightness and shortness of breath as the initial symptom

Light chain systemic-like degeneration is a disease in which amyloid protein is deposited in the trachea by misfolding of monoclonal immunoglobulin light chains, resulting in tissue structure destruction, trachea dysfunction and progressive progression. It is mainly related to the abnormal proliferation of clonal plasma cells. Immunoglobulin light chain (AL) amyloidosis is a rare type of plasmacytosis, which varies greatly among individuals and has different symptoms at the first onset. Tongue hypertrophy and periorbital purpura are the specific manifestations of AL amyloidosis 3]. This is the main reason why we report this case of immunoglobulin light chain (AL) amyloidosis with chest tightness and shortness of breath as the first symptom. Case report: We report a case of a 59-year-old female patients to activity after fatigue, shortness of breath more than 4 years, double leg edema, cannot lay down at night have disease, patients with history of many years, and has been "chest condition" as the main symptoms, accompanied by the tongue body hypertrophy, in accordance with heart medicine routine treatment before cases get better, after multidisciplinary consultation and discussion, Tongue biopsy and bone marrow aspiration are recommended for systemic light chain (AL) amyloidosis. After the diagnosis was confirmed, chemotherapy was started. The symptoms of chest tightness and shortness of breath were significantly improved, and the tongue body was smaller than before. Conclusion: The first symptom is "chest shortness of breath", and the symptoms are repeated. The treatment effect of cardiology department is not good, and it is accompanied by multiple system involvement. The diagnosis of this disease should be considered and relevant specialist examination should be performed as soon as possible, mainly relying on histopathological examination.