以乳糜胸作为首发表现的原发性系统性淀粉样变性一例并文献复习_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

蒲银 , 龚胜兰 , 谢玲俐 ,  毛辉

四川大学华西医院呼吸与危重症医学科（四川成都 610041）;

Corresponding author：

毛辉, Email: merrymh@126.com

Keywords：

胸膜; 淀粉样变; 乳糜胸; 胸腔积液

DOI：

10.7507/1671-6205.201910053

Video：

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目的探讨原发性系统性淀粉样变性的临床表现、诊断、治疗及预后，提高对累及胸膜的原发性系统性淀粉样变性的临床认识。方法报道四川大学华西医院收治的 1 例以乳糜胸作为首发表现的原发性系统性淀粉样变性患者，回顾性分析国内外文献。以“Amyloidosis，pleura”为检索词，在 PubMed 数据库检索，检索时间为 1968 年 12 月至 2019 年 9 月，共检测到相关英文文献 57 篇，报道 18 例患者。以“淀粉样变，胸膜”为检索词，在中国期刊网全文数据库检索，检索时间为 1981 年 3 月至 2019 年 9 月，共检索到相关中文文献 12 篇，报道 7 例患者。结果 58 岁男性患者，临床表现为间断性背痛。查体：胸廓对称，双侧肩胛下角第 7 肋间各有一引流管，引流出乳白色液体。双下肺叩诊呈浊音，双下肺呼吸音低至消失，心脏及腹部查体未见异常。血清免疫固定电泳示可疑 IgA κ 型 M 蛋白条带，血清游离 κ 轻链检测 745 mg/L（参考值 6.7～22.4 mg/L）。胸部 CT 示双侧胸腔积液，双肺门淋巴结增大，腹部增强 CT 示胃壁广泛增厚伴腹腔、腹膜后广泛淋巴结增大，经多次胃镜行胃黏膜组织活检，胃组织刚果红染色阳性，胸腔积液流式细胞学见克隆性浆细胞，骨髓流式细胞学见 4% 克隆性浆细胞，确诊为原发性系统性淀粉样变性。在上述数据库中共检索 69 篇文献，经筛选除外资料不详后，有 18 篇文献 20 例患者明确诊断为胸膜淀粉样变性，符合检索条件。此类患者临床表现及影像学均无特异性，确诊依据胸膜活检刚果红染色阳性或胸腔积液查见淀粉样蛋白。结论原发性系统性淀粉样变性累及胸膜，临床表现及影像学缺乏特征性，与肿瘤胸膜转移、胸膜间皮瘤等疾病易混淆，但多数患者血清中会出现单克隆性免疫球蛋白增多，其诊断主要依靠活检组织病理学检查。治疗方案仍以化疗为主。

Citation： 蒲银, 龚胜兰, 谢玲俐, 毛辉. 以乳糜胸作为首发表现的原发性系统性淀粉样变性一例并文献复习. Chinese Journal of Respiratory and Critical Care Medicine, 2020, 19(5): 500-504. doi: 10.7507/1671-6205.201910053 Copy

1.	Smith FB, Brown RB, Maguire G, et al. Localized pleural microdeposition of type A amyloid in a patient with rheumatoid pleuritis. Histologic distinction from pleural involvement in systemic amyloidosis. Am J Clin Pathol, 1993, 99(3): 261-264.
2.	曹伟标, 朱元珏, 高艳, 等. 淀粉样变伴胸腔积液二例报告. 中国医学科学院学报, 1994, 16(5): 401-402.
3.	Bontemps F, Tillie-Leblond I, Coppin MC, et al. Pleural amyloidosis: thoracoscopic aspects. Eur Respir J, 1995, 8(6): 1025-1027.
4.	Meeus G, Ponette J, Delabie J, et al. Immunoglobulin related amyloidosis presenting as isolated lymph node and pulmonary involvement. Leuk Lymphoma, 2000, 38(3-4): 423-427.
5.	Mansalis KA, Klein DA, Demartini SD, et al. Pleural findings in a patient with persistent pulmonary effusions from systemic amyloidosis. Amyloid, 2011, 18(1): 29-31.
6.	Adams AL, Castro CY, Singh SP, et al. Pleural amyloidosis mimicking mesothelioma: a clinicopathologic study of two cases. Ann Diagn Pathol, 2001, 5(4): 229-232.
7.	王志虹, 林江涛. 肺及胸膜淀粉样变长期误诊一例. 中华医学杂志, 2002, 82(4): 243.
8.	Roux CH, Breuil V, Brocq O, et al. Pleural amyloidosis as the first sign of IgD multiple myeloma. Clin Rheumatol, 2005, 24(3): 294-295.
9.	耑冰, 陈绍平, 周继庸, 等. 胸膜淀粉样变长期误诊一例. 中国呼吸与危重监护杂志, 2006, 5(5): 386-387.
10.	张勇. 胸膜淀粉样变 1 例. 潍坊医学院学报, 2008, 30(1): 35.
11.	Aichaouia C, Ben Meftah MR, M'hamdi S, et al. Pleural amyloidosis attesting to generalised amyloidosis. Rev Pneumol Clin, 2010, 66(3): 204-208.
12.	黄静, 张天托, 周凤丽, 等. 胸膜淀粉样变 1 例并文献复习. 国际呼吸杂志, 2012, 32(14): 1066-1069.
13.	Coolbear F, Bilawich AM, Tongson J, et al. Pleural amyloidosis imitating pleural malignancy. Respir Med Case Rep, 2017, 6(20): 195-197.
14.	Manur R, Lamzabi I. Amyloidosis diagnosed in cytology specimen of pleural effusion: a case report. Diagn Cytopathol, 2018, 46(6): 522-524.
15.	Kunihiro Y, Onoda H, Aoe K, et al. A case of localized pleural amyloidosis: multimodality imaging features. J Thorac Imaging, 2018, 33(6): 54-56.
16.	Kanno Y, Furuya N, Okamoto M, et al. Narrow-band imaging thoracoscopy in pleural amyloidosis. Respirol Case Rep, 2018, 6(3): e00305.
17.	Xiong J, Ren Y, Li H, et al. First case of pleural amyloidosis in systemic erythematosus: report and literature review. Z Rheumatol, 2018, 77(9): 841-843.
18.	Dai Y, Liu C, Chen J, et al. Pleural amyloidosis with recurrent pleural effusion and pulmonary embolism: a case report. Medicine (Baltimore), 2019, 98(3): e14151.
19.	Aylwin AC, Gishen P, Copley SJ. Imaging appearance of thoracic amyloidosis. Thorac Imaging, 2005, 20(1): 41-46.
20.	Cordier JF, Loire R, Brune J. Amyloidosis of the lower respiratory tract. Clinical and pathologic features in a series of 21 patients. Chest, 1986, 90(6): 821-830.
21.	Wang ZJ, Reddy GP, Gotway MB, et al. Malignant pleural mesothelioma: evaluation with CT, MR imaging, and PET. Radiographics, 2004, 24(1): 105-119.
22.	王棪嫒, 路瑾. 原发系统性淀粉样变性的诊疗进展. 临床血液学杂志, 2016, 29(7): 544-546.
23.	Hassan W, Al-Sergani H, Mourad W, et al. Amyloid heart disease. New frontiers and insights in pathophysiology, diagnosis, and management. Texas Heart Inst J, 2005, 32(2): 178-184.
24.	Patil PD, Cua YM, Farver C, et al. A 54-year-old man with anasarca, dyspnea, and recurrent bilateral pleural effusions. Chest, 2017, 52(2): e39-e44.
25.	Berk JL. Pleural effusions in systemic amyloidosis. Curr Opin Pulm Med, 2005, 1: 324-328.
26.	Lousada I, Comenzo RL, Landau H, et al. Light chain amyloidosis: patient experience survey from the amyloidosis. Research consortium. Adv Ther, 2015, 32(10): 920-928.
27.	Wechalekar AD, Schonland SO, Kastritis E, et al. A European collaborative study of treatment outcomes in 346 patients with cardiac stage Ⅲ AL amyloidosis. Blood, 2013, 121: 3420-3427.
28.	Gertz MA. Immunoglobulin light chain amyloidosis: 2016 update on diagnosis, prognosis, and treatment. Hematology, 2016, 91: 948-956.
29.	Quarta CC, Gonzalez-Lopez E, Gilbertson JA, et al. Diagnostic sensitivity of abdominal fat aspiration in cardiac amyloidosis. European Heart Journal, 2017, 38: 1905-1908.
30.	Muchtar E, Dispenzieri A, Lacy MQ, et al. Overuse of organ biopsies in immunoglobulin light chain amyloidosis (AL): the consequence of failure of early recognition. Ann Med, 2017, 49(7): 545-551.
31.	中国抗癌协会血液肿瘤专业委员会, 中华医学会血液分会白血病淋巴瘤学组. 原发性轻链型淀粉样变的诊断和治疗中国专家共识. 中华血液学杂志, 2016, 37(9): 742-746.
32.	Nakano T, Endo S, Tetsuka K, et al. Asymptomatic localized pleural amyloidosis mimicking malignant pleural mesothelioma: report of a case. J Thorac Dis, 2016, 8(1): E157-E160.
33.	中国系统性淀粉样变性协作组, 国家肾脏疾病临床医学研究中心. 系统性轻链型淀粉样变性诊断和治疗指南. 中华医学杂志, 2016, 96(44): 3540-3548.
34.	黄栩芾, 冯俊, 张聪丽, 等. 梅奥分期系统在中国原发性轻链型淀粉样变患者中的临床应用价值. 中华血液学杂志, 2016, 37(4): 278-282.
35.	D'Souza A, Dispenzieri A, Wirk B, et al. Improved outcomes after autologous hematopoietic cell transplantation for light chain amyloidosis: a center for international blood and marrow transplant research study. J Clin Oncol, 2015, 33(32): 3741-3749.
36.	Landau H, Smith M, Landry C, et al. Long-term event-free and overall survival after risk-adapted melphalan and SCT for systemic light chain amyloidosis. Leukemia, 2017, 31(1): 136-142.
37.	Palladini G, Perfetti V, Obici L, et al. Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation. Blood, 2004, 103(8): 2936-2938.
38.	Palladini G, Russo P, Nuvolone M, et al. Treatment with oral melphalan plus dexamethasone produces long-term remissions in AL amyloidosis. Blood, 2007, 110(2): 787-788.
39.	Kastritis E, Leleu X, Arnulf B, et al. A randomized phase Ⅲ trial of melphalan and dexamethasone (MDex) versus bortezomib, melphalan and dexamethasone (BMDex) for untreated patients with AL amyloidosis. Blood, 2016, 128(22): 646.
40.	Palladini G, Milani P, Foli A, et al. Melphalan and dexamethasone with or without bortezomib in newly diagnosed AL amyloidosis: a matched casecontrol study on 174 patients. Leukemia, 2014, 28(12): 2311-22316.
41.	Palladini G, Milani P, Merlini G. Novel strategies for the diagnosis and treatment of cardiac amyloidosis. Expert Rev Cardiovasc Ther, 2015, 13(11): 1195-1211.
42.	Palladini G, Merlini G. What is new in diagnosis and management of light chain amyloidosis?. Blood, 2016, 128(2): 159-168.
43.	Muchtar E, Gertz MA, Kumar SK, et al. Improved outcomes for newly diagnosed AL amyloidosis between 2000 and 2014: cracking the glass ceiling of early death. Blood, 2017, 129(15): 2111-2119.

1. Smith FB, Brown RB, Maguire G, et al. Localized pleural microdeposition of type A amyloid in a patient with rheumatoid pleuritis. Histologic distinction from pleural involvement in systemic amyloidosis. Am J Clin Pathol, 1993, 99(3): 261-264.
2. 曹伟标, 朱元珏, 高艳, 等. 淀粉样变伴胸腔积液二例报告. 中国医学科学院学报, 1994, 16(5): 401-402.
3. Bontemps F, Tillie-Leblond I, Coppin MC, et al. Pleural amyloidosis: thoracoscopic aspects. Eur Respir J, 1995, 8(6): 1025-1027.
4. Meeus G, Ponette J, Delabie J, et al. Immunoglobulin related amyloidosis presenting as isolated lymph node and pulmonary involvement. Leuk Lymphoma, 2000, 38(3-4): 423-427.
5. Mansalis KA, Klein DA, Demartini SD, et al. Pleural findings in a patient with persistent pulmonary effusions from systemic amyloidosis. Amyloid, 2011, 18(1): 29-31.
6. Adams AL, Castro CY, Singh SP, et al. Pleural amyloidosis mimicking mesothelioma: a clinicopathologic study of two cases. Ann Diagn Pathol, 2001, 5(4): 229-232.
7. 王志虹, 林江涛. 肺及胸膜淀粉样变长期误诊一例. 中华医学杂志, 2002, 82(4): 243.
8. Roux CH, Breuil V, Brocq O, et al. Pleural amyloidosis as the first sign of IgD multiple myeloma. Clin Rheumatol, 2005, 24(3): 294-295.
9. 耑冰, 陈绍平, 周继庸, 等. 胸膜淀粉样变长期误诊一例. 中国呼吸与危重监护杂志, 2006, 5(5): 386-387.
10. 张勇. 胸膜淀粉样变 1 例. 潍坊医学院学报, 2008, 30(1): 35.
11. Aichaouia C, Ben Meftah MR, M'hamdi S, et al. Pleural amyloidosis attesting to generalised amyloidosis. Rev Pneumol Clin, 2010, 66(3): 204-208.
12. 黄静, 张天托, 周凤丽, 等. 胸膜淀粉样变 1 例并文献复习. 国际呼吸杂志, 2012, 32(14): 1066-1069.
13. Coolbear F, Bilawich AM, Tongson J, et al. Pleural amyloidosis imitating pleural malignancy. Respir Med Case Rep, 2017, 6(20): 195-197.
14. Manur R, Lamzabi I. Amyloidosis diagnosed in cytology specimen of pleural effusion: a case report. Diagn Cytopathol, 2018, 46(6): 522-524.
15. Kunihiro Y, Onoda H, Aoe K, et al. A case of localized pleural amyloidosis: multimodality imaging features. J Thorac Imaging, 2018, 33(6): 54-56.
16. Kanno Y, Furuya N, Okamoto M, et al. Narrow-band imaging thoracoscopy in pleural amyloidosis. Respirol Case Rep, 2018, 6(3): e00305.
17. Xiong J, Ren Y, Li H, et al. First case of pleural amyloidosis in systemic erythematosus: report and literature review. Z Rheumatol, 2018, 77(9): 841-843.
18. Dai Y, Liu C, Chen J, et al. Pleural amyloidosis with recurrent pleural effusion and pulmonary embolism: a case report. Medicine (Baltimore), 2019, 98(3): e14151.
19. Aylwin AC, Gishen P, Copley SJ. Imaging appearance of thoracic amyloidosis. Thorac Imaging, 2005, 20(1): 41-46.
20. Cordier JF, Loire R, Brune J. Amyloidosis of the lower respiratory tract. Clinical and pathologic features in a series of 21 patients. Chest, 1986, 90(6): 821-830.
21. Wang ZJ, Reddy GP, Gotway MB, et al. Malignant pleural mesothelioma: evaluation with CT, MR imaging, and PET. Radiographics, 2004, 24(1): 105-119.
22. 王棪嫒, 路瑾. 原发系统性淀粉样变性的诊疗进展. 临床血液学杂志, 2016, 29(7): 544-546.
23. Hassan W, Al-Sergani H, Mourad W, et al. Amyloid heart disease. New frontiers and insights in pathophysiology, diagnosis, and management. Texas Heart Inst J, 2005, 32(2): 178-184.
24. Patil PD, Cua YM, Farver C, et al. A 54-year-old man with anasarca, dyspnea, and recurrent bilateral pleural effusions. Chest, 2017, 52(2): e39-e44.
25. Berk JL. Pleural effusions in systemic amyloidosis. Curr Opin Pulm Med, 2005, 1: 324-328.
26. Lousada I, Comenzo RL, Landau H, et al. Light chain amyloidosis: patient experience survey from the amyloidosis. Research consortium. Adv Ther, 2015, 32(10): 920-928.
27. Wechalekar AD, Schonland SO, Kastritis E, et al. A European collaborative study of treatment outcomes in 346 patients with cardiac stage Ⅲ AL amyloidosis. Blood, 2013, 121: 3420-3427.
28. Gertz MA. Immunoglobulin light chain amyloidosis: 2016 update on diagnosis, prognosis, and treatment. Hematology, 2016, 91: 948-956.
29. Quarta CC, Gonzalez-Lopez E, Gilbertson JA, et al. Diagnostic sensitivity of abdominal fat aspiration in cardiac amyloidosis. European Heart Journal, 2017, 38: 1905-1908.
30. Muchtar E, Dispenzieri A, Lacy MQ, et al. Overuse of organ biopsies in immunoglobulin light chain amyloidosis (AL): the consequence of failure of early recognition. Ann Med, 2017, 49(7): 545-551.
31. 中国抗癌协会血液肿瘤专业委员会, 中华医学会血液分会白血病淋巴瘤学组. 原发性轻链型淀粉样变的诊断和治疗中国专家共识. 中华血液学杂志, 2016, 37(9): 742-746.
32. Nakano T, Endo S, Tetsuka K, et al. Asymptomatic localized pleural amyloidosis mimicking malignant pleural mesothelioma: report of a case. J Thorac Dis, 2016, 8(1): E157-E160.
33. 中国系统性淀粉样变性协作组, 国家肾脏疾病临床医学研究中心. 系统性轻链型淀粉样变性诊断和治疗指南. 中华医学杂志, 2016, 96(44): 3540-3548.
34. 黄栩芾, 冯俊, 张聪丽, 等. 梅奥分期系统在中国原发性轻链型淀粉样变患者中的临床应用价值. 中华血液学杂志, 2016, 37(4): 278-282.
35. D'Souza A, Dispenzieri A, Wirk B, et al. Improved outcomes after autologous hematopoietic cell transplantation for light chain amyloidosis: a center for international blood and marrow transplant research study. J Clin Oncol, 2015, 33(32): 3741-3749.
36. Landau H, Smith M, Landry C, et al. Long-term event-free and overall survival after risk-adapted melphalan and SCT for systemic light chain amyloidosis. Leukemia, 2017, 31(1): 136-142.
37. Palladini G, Perfetti V, Obici L, et al. Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation. Blood, 2004, 103(8): 2936-2938.
38. Palladini G, Russo P, Nuvolone M, et al. Treatment with oral melphalan plus dexamethasone produces long-term remissions in AL amyloidosis. Blood, 2007, 110(2): 787-788.
39. Kastritis E, Leleu X, Arnulf B, et al. A randomized phase Ⅲ trial of melphalan and dexamethasone (MDex) versus bortezomib, melphalan and dexamethasone (BMDex) for untreated patients with AL amyloidosis. Blood, 2016, 128(22): 646.
40. Palladini G, Milani P, Foli A, et al. Melphalan and dexamethasone with or without bortezomib in newly diagnosed AL amyloidosis: a matched casecontrol study on 174 patients. Leukemia, 2014, 28(12): 2311-22316.
41. Palladini G, Milani P, Merlini G. Novel strategies for the diagnosis and treatment of cardiac amyloidosis. Expert Rev Cardiovasc Ther, 2015, 13(11): 1195-1211.
42. Palladini G, Merlini G. What is new in diagnosis and management of light chain amyloidosis?. Blood, 2016, 128(2): 159-168.
43. Muchtar E, Gertz MA, Kumar SK, et al. Improved outcomes for newly diagnosed AL amyloidosis between 2000 and 2014: cracking the glass ceiling of early death. Blood, 2017, 129(15): 2111-2119.

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