Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms
Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms
复制标题
DOI：
10.2169/internalmedicine.53.0898
复制
发表时间：
2014-01-01
影响因子：
1.2
通讯作者：
Ikeda, Shu-ichi
中科院分区：
文献类型：
--
作者：
Matsuda, Masayuki;Katoh, Nagaaki;Ikeda, Shu-ichi
目的回顾性分析日本全身性AL淀粉样变诊断时的临床表现。方法回顾230例来我院就诊并确诊为AL淀粉样变的日本患者的病历资料，筛选出系统性淀粉样变患者。分析全身性AL淀粉样变诊断的临床资料，包括实验室和影像学结果。结果122例患者入组，平均年龄58.7±9.5岁。173例患者进行免疫固定或免疫电泳，其中144例血清和/或尿液m蛋白阳性（kappa lambda = 30:14 4）。诊断时的主要临床表现为蛋白尿和/或肾功能不全（54.0%）、充血性心力衰竭（24.8%）、周围神经病变（10.4%）、肝肿大（7.9%）和心律失常（5.0%）。其余患者出现异常表现，如孤立性肿瘤、淋巴结病变、胃肠道出血、肠假性梗阻、出血倾向和多关节痛。计算机断层扫描显示肠扩张伴明显的肠壁增厚，内窥镜显示多发性结节病变伴黏膜脆性，是全身性AL淀粉样变性的特征性表现。结论日本全身性AL淀粉样变性患者的临床表现与美国和欧洲国家先前报道的相似；然而，一些患有此病的患者会出现不常见的症状。在对这些症状进行鉴别诊断时，进行系统性AL淀粉样变的实验室和组织学检查是必要的。
Objective To retrospectively investigate the clinical manifestations at diagnosis in Japanese patients with systemic AL amyloidosis.Methods We reviewed the medical records of 230 Japanese patients who had visited our hospital and been diagnosed with AL amyloidosis, and abstracted those with the systemic type. The clinical data at diagnosis of systemic AL amyloidosis, including laboratory and imaging findings, were analyzed.Results Two hundred and two patients (mean, 58.7 +/- 9.5 years) were enrolled in this study. Immunofixation or immunoelectrophoresis was performed in 173 patients, 144 of whom were positive for M-protein in the serum and/or urine (kappa lambda = 30: 114). The primary clinical manifestations at diagnosis were proteinuria and/or renal dysfunction (54.0%), congestive heart failure (24.8%), peripheral neuropathy (10.4%), hepatomegaly (7.9%) and arrhythmia (5.0%). The remaining patients developed unusual manifestations, such as solitary tumor, lymphadenopathy, gastrointestinal bleeding, intestinal pseudoobstruction, hemorrhagic tendencies and polyarthralgia. Dilatation of the intestine with marked thickening of the gastrointestinal wall on computed tomography and multiple nodular lesions with associated mucosal friability on endoscopy are characteristic findings of systemic AL amyloidosis.Conclusion The clinical pictures of Japanese patients with systemic AL amyloidosis are similar to those previously reported from the US and European nations; however, some patients with this disease develop uncommon symptoms. Conducting laboratory and histological examinations for systemic AL amyloidosis is necessary when making a differential diagnosis of these symptoms.