| 卢 蓉,许 辉,唐文彬,李晓照.表现为腹部淀粉样瘤及多系统功能异常的AA型系统性淀粉样变性1例暨文献复习[J].,2019,19(6):1074-1077 |
| 表现为腹部淀粉样瘤及多系统功能异常的AA型系统性淀粉样变性1例暨文献复习 |
| AA Systemic Amyloidosis Presenting as Abdominal Amyloidoma and Multiple Systemic Dysfunction: a Case Report and Literature |
| 投稿时间：2018-07-07 修订日期：2018-07-31 |
| DOI：10.13241/j.cnki.pmb.2019.06.015 |
| 中文关键词: 淀粉样瘤 淀粉样变性 结核 |
| 英文关键词: Amyloidoma Amyloidosis Tuberculosis |
| 基金项目:国家自然科学基金项目(81270786) |
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| 中文摘要: |
| 摘要 目的：报道1例AA型系统性淀粉样变性患者的临床资料和病理资料，分析该病例特殊表现，总结淀粉样变性常见的临床表现及诊治要点。方法：回顾性分析1例表现为腹部淀粉样瘤及多系统功能异常的AA型系统性淀粉样变性患者的临床资料及诊治过程，结合该例患者的临床资料及诊治过程进行文献复习，明确淀粉样变性的临床特点、诊治要点。结果：该例患者为一名62岁女性，自2012年开始相继出现四肢麻木、贫血、食欲下降、腹泻、水肿、蛋白尿及肾功能不全等多系统病变的症状。既往有“肺结核、胸椎结核”病史。病程中检查发现：重度缺铁性贫血，肾病综合征，血肌酐258 μmol/L，球蛋白升高；血沉快，血轻链κ/λ比值为1.39，其他免疫学检查均阴性；骨髓细胞学检查未见异常增殖浆细胞；影像学示左下腹部一卵圆形大肿块，腹膜后多发淋巴结肿大；结肠镜检诊断为糜烂性结肠炎；腹部肿块、结肠粘膜及肾脏穿刺病理最终确诊考虑为结核感染导致AA型系统性淀粉样变性。结论：系统性淀粉样变性临床表现多样、缺乏特异性，因此常常被误诊或漏诊。本病例患者除了多系统受累的临床表现外，更为特殊的是在患者的腹部出现了一淀粉样物质所构成的淀粉样瘤，这种少见的临床表现很容易被误诊为肿瘤而延误诊治。因此，及时对该类患者进行组织活检，通过病理学及特殊染色将有助于系统性淀粉样变性的早期诊断，减少及延缓并发症的发生。 |
| 英文摘要: |
| ABSTRACT Objective: To report the clinical data and pathological data of one case of AA systemic amyloidosis, and analyze the special phenomenon about this case, summarizing the common clinical manifestations and key points for the diagnosis and treatment of amyloidosis. Methods: Retrospective analysis of a case of AA systemic amyloidosis, which presenting as abdominal amyloidoma and multiple systemic dysfunction, about its clinical data and diagnosis and treatment process. Reviewed the literature in combination with her clinical data and diagnosis and treatment process, to clarify the clinical characteristics, key points of diagnosis and treatment. Results: This case report a 62-year-old Chinese woman, who developed symptoms of numbness, anemia, loss of appetite, diarrhea, edema, proteinuria and renal insufficiency one after another since 2012. And she had a history of pulmonary tuberculosis and thoracic tuberculosis. She was found to have severe iron deficiency anemia, nephrotic syndrome, creatinine was 258 μmol/L, globulin increased, rapid blood sedimentation, the ratio of immunoglobulin light chain κ/λ was 1.39, and other immunological tests were all negative during the course of the examination. There was no abnormal proliferating plasma cells were found in bone marrow cytology. Imaging examination revealed an oval large mass in her left lower abdomen with multiple enlarged lymph nodes after the peritoneum. Colonoscopy for the diagnosis of erosive colitis. Combining with her pathological results of abdominal mass, colonic mucosa and renal biopsy, her finally diagnosis was AA systemic amyloidosis secondary to tuberculosis infection. Conclusion: The clinical manifestation of systemic amyloidosis are varied and lack of specificity, therefore, it is often misdiagnosed or missed. In addition to involve multiple system, this case is more special for founding amyloidoma in the patient abdomen, and this rare clinical manifestation is easily misdiagnosed as a tumor and affects treatment. Therefore, tissue biopsy should be performed in time for this type of patient, and pathological and special staining will help early diagnosis of systemic amyloidosis, reduce and delay the occurrence of complications. |
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