Localized amyloidosis: A survey of 35 French cases
Localized amyloidosis: A survey of 35 French cases
复制标题
DOI：
10.1080/13506120500351174
复制
发表时间：
2005-12-01
影响因子：
5.5
通讯作者：
Roblot, P
中科院分区：
文献类型：
--
作者：
Paccalin, M;Hachulla, E;Roblot, P
由于局部淀粉样变的预后仍不清楚，我们进行了一项调查，以确定这种疾病的特点和过程。分析了35例喉（14例）、气管支气管（10例）、结肠（1例）或下尿路淀粉样变性（10例）患者的图表。诊断时的平均年龄为52.7 ± 12岁（范围33-73岁）。15例患者的淀粉样蛋白类型为淀粉样轻链（AL）。所有患者均接受了额外的活检（副唾液腺、直肠、脂肪垫和骨髓穿刺），以排除全身性疾病。症状治疗包括内镜切除和激光治疗。秋水仙碱和化疗与泼尼松和美法仑处方有限的成功。在平均6.1 ± 5.3年的随访期间，没有患者发生系统性淀粉样变性。报告了6例死亡病例，其中1例因致命的气道出血而死亡。我们建议应更常规地进行免疫标记研究。在我们的调查中，局部淀粉样蛋白沉积没有发展成系统性疾病的风险。然而，局部进化可能危及生命。这类患者应转诊到专科中心进行进一步评估。管理需要密切随访，以排除复发，并确定适当的对症治疗。
Since the prognosis of localized amyloidosis remains unclear, we conducted a survey to define the characteristics and the course of this disease. The charts of 35 patients with either laryngeal (14 patients), tracheobronchial (10 patients), colonic (1 patient), or lower urinary tract amyloidosis (10 patients) were analyzed. The average age at diagnosis was 52.7 +/- 12 years (range 33-73 years). The amyloid protein type was specified to be amyloid light chain (AL) in 15 cases. All patients had undergone additional biopsies (accessory salivary glands, rectal, fat pad and bone marrow aspirates) to rule out a systemic disease. Symptomatic treatments included endoscopic excision and laser therapy. Colchicine and chemotherapy with prednisone and melphalan were prescribed with limited success. During a mean follow-up period of 6.1 +/- 5.3 years no patient developed a systemic form of amyloidosis. Six deaths were reported, one related to the disease because of a fatal airway hemorrhage.We suggest that immunolabeling studies should be more routinely performed. There was no risk of developing a systemic disease from local amyloid deposits in our survey. However, local evolution can be life-threatening. Such patients should be referred to specialist centers for further evaluation. Management requires close follow-up to exclude recurrence and to determine the appropriate symptomatic treatment.