中华结核和呼吸杂志
中華結覈和呼吸雜誌
중화결핵화호흡잡지
Chinese Journal of Tuberculosis and Respiratory Diseases
2013年
2期
88-93
,共6页
牟向东%熊焰%陈建%章巍%邱志祥%胡艳%刘莹%丛铁川%高莉%任雅丽%王广发
牟嚮東%熊燄%陳建%章巍%邱誌祥%鬍豔%劉瑩%叢鐵川%高莉%任雅麗%王廣髮
모향동%웅염%진건%장외%구지상%호염%류형%총철천%고리%임아려%왕엄발
淀粉样变性%呼吸系统%造血干细胞移植
澱粉樣變性%呼吸繫統%造血榦細胞移植
정분양변성%호흡계통%조혈간세포이식
Amyloidosis%Respiratory system%Hematopoietic stem cell transplantation
目的 探讨呼吸系统淀粉样变性的临床表现、诊断和治疗方法.方法 回顾性分析2002年1月至2012年1月在北京大学第一医院住院的11例呼吸系统淀粉样变性患者的临床资料,并结合文献进行复习.结果 10年间病理确诊为淀粉样变性的住院患者共250例,占同期住院患者总数的0.06％ (250/389 402)；病理确诊为呼吸系统淀粉样变性11例,占淀粉样变性总例数的4.4％(11/250),其中淀粉样物相关蛋白A型(AA型)4例,免疫球蛋白轻链型(AL型)7例.临床主要表现为声嘶、咳嗽、呼吸困难等.4例AA型患者为单侧喉部局限性病变,手术切除后无复发.7例AL型患者中,2例累及双侧喉部,手术切除后多次复发；4例弥漫累及气管、支气管,影像学表现为气道黏膜弥漫性增厚、管腔狭窄、突向腔内的结节及气道壁的钙化,内窥镜表现为气道黏膜肥厚、充血、水肿、隆起及管腔狭窄；3例累及肺部,2例为双肺弥漫性间质样病变,另1例为肺内孤立性肿块,伴肺外受累.7例AL型患者中,3例接受化疗和(或)放疗,3例行外科手术,2例行自体造血干细胞移植,2例行支气管镜下介入治疗；随访3年,4例存活,2例死亡,1例失访.结论 呼吸系统淀粉样变性临床罕见,可分为AA型和AL型.AA型常为局限性病变,手术切除效果好；AL型常为弥漫性病变,易复发、预后差,治疗措施包括外科手术、介入治疗、放化疗及自体造血干细胞移植等.
目的 探討呼吸繫統澱粉樣變性的臨床錶現、診斷和治療方法.方法 迴顧性分析2002年1月至2012年1月在北京大學第一醫院住院的11例呼吸繫統澱粉樣變性患者的臨床資料,併結閤文獻進行複習.結果 10年間病理確診為澱粉樣變性的住院患者共250例,佔同期住院患者總數的0.06％ (250/389 402)；病理確診為呼吸繫統澱粉樣變性11例,佔澱粉樣變性總例數的4.4％(11/250),其中澱粉樣物相關蛋白A型(AA型)4例,免疫毬蛋白輕鏈型(AL型)7例.臨床主要錶現為聲嘶、咳嗽、呼吸睏難等.4例AA型患者為單側喉部跼限性病變,手術切除後無複髮.7例AL型患者中,2例纍及雙側喉部,手術切除後多次複髮；4例瀰漫纍及氣管、支氣管,影像學錶現為氣道黏膜瀰漫性增厚、管腔狹窄、突嚮腔內的結節及氣道壁的鈣化,內窺鏡錶現為氣道黏膜肥厚、充血、水腫、隆起及管腔狹窄；3例纍及肺部,2例為雙肺瀰漫性間質樣病變,另1例為肺內孤立性腫塊,伴肺外受纍.7例AL型患者中,3例接受化療和(或)放療,3例行外科手術,2例行自體造血榦細胞移植,2例行支氣管鏡下介入治療；隨訪3年,4例存活,2例死亡,1例失訪.結論 呼吸繫統澱粉樣變性臨床罕見,可分為AA型和AL型.AA型常為跼限性病變,手術切除效果好；AL型常為瀰漫性病變,易複髮、預後差,治療措施包括外科手術、介入治療、放化療及自體造血榦細胞移植等.
목적 탐토호흡계통정분양변성적림상표현、진단화치료방법.방법 회고성분석2002년1월지2012년1월재북경대학제일의원주원적11례호흡계통정분양변성환자적림상자료,병결합문헌진행복습.결과 10년간병리학진위정분양변성적주원환자공250례,점동기주원환자총수적0.06％ (250/389 402)；병리학진위호흡계통정분양변성11례,점정분양변성총례수적4.4％(11/250),기중정분양물상관단백A형(AA형)4례,면역구단백경련형(AL형)7례.림상주요표현위성시、해수、호흡곤난등.4례AA형환자위단측후부국한성병변,수술절제후무복발.7례AL형환자중,2례루급쌍측후부,수술절제후다차복발；4례미만루급기관、지기관,영상학표현위기도점막미만성증후、관강협착、돌향강내적결절급기도벽적개화,내규경표현위기도점막비후、충혈、수종、륭기급관강협착；3례루급폐부,2례위쌍폐미만성간질양병변,령1례위폐내고립성종괴,반폐외수루.7례AL형환자중,3례접수화료화(혹)방료,3례행외과수술,2례행자체조혈간세포이식,2례행지기관경하개입치료；수방3년,4례존활,2례사망,1례실방.결론 호흡계통정분양변성림상한견,가분위AA형화AL형.AA형상위국한성병변,수술절제효과호；AL형상위미만성병변,역복발、예후차,치료조시포괄외과수술、개입치료、방화료급자체조혈간세포이식등.
Objective To investigate the clinical manifestation,diagnosis and treatment of respiratory amyloidosis.Methods Data of 11 patients with respiratory amyloidosis diagnosed by biopsy in Peking University First Hospital from January 2002 to January 2012 were analyzed,and the related literatures were reviewed.Results In the last decade,250 of 389 402 hospitalized patients were pathologically diagnosed as having amyloidosis,and 11 cases were pathologically confirmed to be respiratory amyloidosis.In these 11 patients,4 cases were with serum amloid A (AA) amyloidosis and 7 with light-chain (AL) amyloidosis.The main clinical manifestations included hoarseness,cough and dyspnea.In 4 cases with AA type unilateral larynx was involved and there was no recurrence after surgical resection.Of 7 cases with AL type,2 cases had involvement of bilateral larynxes and both relapsed after surgery.Diffuse involvement of trachea and bronchi was found in 4 cases,and the chest CT scans showed diffuse thickening and local calcification of the airway wall,bronchial stenosis and nodules protruding into the lumen.Bronchoscopy showed airway mucosal hypertrophy,hyperemia,edema and bronchial stenosis.Lung involvement was found in 3 cases,2 of which presented with diffuse pulmonary interstitial infiltrates,and another case presented with solitary pulmonary mass and extrapulmonary lesions.Of the 7 cases with AL type,3 cases were treated by chemotherapy and/or radiotherapy,3 received surgery,2 underwent autologous hematopoietic stem cell transplantation,and 2 underwent bronchoscopic interventional therapy.Within 3 years of follow-up,4 patients were alive,2 dead and 1 lost to follow up.Conclusions Respiratory amyloidosis,which can be divided into AA and AL types,is clinically rare.Patients with AA type usually present with local lesions,which can be cured by surgery,while patients with AL type often present with diffuse lesions and require integrated therapies including surgery,interventional treatment,chemotherapy,radiotherapy,and autologous hematopoietic stem cell transplantation.
