原发性气管及支气管淀粉样变一例

患者 ,熊福裕 ,男性 ,81岁 ,住院号 :30 7716 ,入院日期 :2 0 0 1.4.2 8,咯血 40 0 cc/12小时内入院 ,患者不咳嗽无痰 ,无呼吸系症状 ,体检 HR84次 /分 ,律齐 ,心尖区闻及 级 SM,心界向左下扩大 ,杵状指 ,BP12 0 /70 mg,10年前曾有十二指肠球部溃疡合并出血史。胸片示 :右肺中叶炎症伴肺不张 ,CEA8.4ug/ml血常规 :WBC3.9× 10 9/L N6 5 % L2 0 .0 % M13.2 % Hb10 4g/l血沉 5 0 mm/h,CT:右下肺局限实变 ,右下叶背段局限支扩 ,慢支、肝脏多发囊肿 ,B超 :肝脏多发囊肿 ,胆囊多发性小结石 ,胰脾正常。肺功能 :轻度肺容量受限 ,伴中度阻…     

胃肠道原发性淀粉样变1例

病例:患者,女,64岁,因反复腹泻、解脓血便5个月于2002年11月29日入院。患者于入院前5个月出现腹痛,以左下腹为主,并有腹泻,开始为黏液水样便,后出现脓血,便次从3～4次/d逐渐加重至>20次/d,伴发热和口腔黏膜、舌体溃疡。外院肠镜检查示溃疡性结肠炎,但未作病理检查,即予激素、抗感染、止血、止泻和中药灌肠等治疗,因     

肾淀粉样变10例临床与病理分析

淀粉样变病是一种由淀粉样蛋白在组织内沉积引起的全身性疾病,可分为系统性和局灶性2类.系统性淀粉样变性包括免疫球蛋白轻链(AL)、血清淀粉样A物质(AA)、家族性、衰老的系统性淀粉样变性和透析相关的淀粉样变性;局灶性淀粉样变则包括局灶的AL淀粉样变以及与帕金森和快速进展性疾病相关的阿尔海默茨病、克雅病.最常累及肾脏的淀粉样物质是AL、AA及家族性淀粉样变性.据统计,老年及老年前期肾病中,肾淀粉样变占其肾病综合征的9.1％ ～ 15.0％[1].我们对我院收集的10例肾淀粉样变患者的临床及病理资料进行分析.     

原发性呼吸道淀粉样变四例

例 1　 患者女 ,4 9岁。因反复咳嗽 咳痰 咯血 2 0余年 ,喘憋伴声音嘶哑 10年于 1999年入院。患者入院前 7年曾因声音嘶哑于我院行声带息肉摘除术。体检 :声音嘶哑 ,听诊闻及右肺呼吸音较左肺低 ,双肺可闻及呼气性哮鸣音。血常规、肝肾功能、类风湿因子、抗中性粒细胞胞浆抗体、血浆蛋白电泳、免疫球蛋白定量、尿本周蛋白及自身免疫性疾病相关血清学检查均未见异常。反复多次痰脱落细胞学检查及痰找抗酸杆菌均阴性。心电图、肝肾超声检查未见异常。胸部X线示双肺纹理增重、紊乱 ;CT示右肺中叶片状模糊影。肺功能检查示阻塞性通气功能障…     

原发性系统性淀粉样变24例临床病理分析

淀粉样变是器官组织中异常的细胞外蛋白物质沉积所造成的慢性炎症，根据发病原因可分为原发性、继发性、家族遗传性、多发性骨髓瘤伴发、血液透析伴发的淀粉样变；继发性淀粉样变的主要病因是结缔组织病、肿瘤或结核等慢性感染。原发性系统性淀粉样变（PSA）临床罕见，由于其临床表现复杂多样且无特异性。常常被延误诊断。     

原发性淀粉样变病1例

病例患者,女,44岁。因腹胀6年,双下肢水肿2个月入院。2002年患者无明显诱因反复出现腹胀,无胸闷、胸痛、发热。2007年8月患者出现厌油、食欲减退,曾到当地医院行乙肝全套及胸片检查均正常。08年4月腹胀加重,曾多次在医院行B超、CT检查提示肝大。于08年7月厌油、纳差症状加重,     

原发性喉、气管、支气管淀粉样变1例

患者男性，54岁．因活动后咳嗽，气喘7年．声音嘶哑4年，加重2个月，发热3天，于2003年5月29日入院。患者于1996年韧以来常在上楼时咳嗽、胸闷，气促．休息后缓解，尚能胜任一般工作。随后患者症状逐年加重．1999年12月出现声音嘶哑而住院．诊断支气管哮喘并感染、肺结棱，先后给予抗菌、平喘、祛痰、抗结棱等治疗近6个月症状末能完全缓解。近2个月来，     

二甲基亚砜治疗原发性淀粉样变一例

患者男,51岁,因乏力、纳差、多汗、双下肢水肿13年,于l997年6月17日入院.13年前无明显诱因出现乏力、纳差、多汗,伴轻度双下肢水肿.查体:肝肋下7 cm,剑突下10 cm,脾肋下刚触及,舌胖大.肝功 ALT34.2-79 U/L,尿蛋白(?),24小时尿蛋白定量2.16 g,ESR:54 mm/h.肝活检,经刚果红染色诊为“淀粉样变”,骨髓涂片见“散在团块状粉红色无结构物质”.给予强的松45mg/日,服用一月后病情好转停用.10年前开始服用25%二甲基亚砜(Dimethyl Sulfoxide,DM-SO)20 ml, 一日三次.服用4年后乏力、多汗等好转,肝脏缩小(肋下刚扪及,剑突下5     

原发性喉气管支气管淀粉样变一例

患者男性 ,2 4岁 ,因“反复咯血 2年 ,活动后气急 3个月”于 2 0 0 1年 4月 30日入院。患者 1999年 4月无明显诱因出现少量咯血、声音嘶哑 ,电子喉镜检查 :发现左侧声带新生物 ,经激光切除症状改善。 2 0 0 1年 3月咯血症状复发 ,在我院行气管切开 +喉裂开 +新生物摘除术 ,术后病理诊断 :左侧声带淀粉样变性伴潴留囊肿及慢性炎症。今年 1月起患者感觉活动后气急并逐渐加重。 4月 8日因高热、大咯血在外院行纤维支气管镜 (纤支镜 )检查 ,活检病理诊断 :右主支气管慢性炎症 ,其中见淀粉样变和钙盐沉着 ,并有纤维组织增生 ,为进一步治疗收入我…     

原发性支气管淀粉样变1例报告并文献复习

患者男性,62岁,因反复咳嗽咳痰2年,加重伴胸闷气急4月入科.2年前开始反复出现受凉后咳嗽,咳少量白粘痰,4月前咳嗽加重,咳少量白粘痰,伴轻度胸闷、气急,有时有喘鸣,1月来咳嗽、气急进一步加重,不能平卧,自觉无痰血、发热.     

原发性气管淀粉样变一例

@@     

Primary tracheobronchial amyloidosis: a series of 3 cases

Primary tracheobronchial amyloidosis is a form of localized pulmonary amyloidosis, characterised by the deposition of AL-amyloid in trachea and bronchi. It is a rare and slowly progressive disease, usually requiring repeated endoscopic treatment. In this case series we describe symptoms, diagnostic and therapeutic procedures, radiological findings and pulmonary function testing in 3 cases of different presentation and severity. Two patients were treated by endoscopic debulking and stent placement during rigid bronchoscopy, both with excellent clinical and functional results. In one of these patients regular endoscopic and clinical control exams were performed in the 5 years following the initial treatment, showing stable disease, requiring no further therapeutic intervention until today.     

Primary systemic amyloidosis: multivariate analysis for prognostic factors in 168 cases

One hundred sixty-eight patients with primary systemic amyloidosis (AL) were identified. Median survival after diagnosis was 12 months and ranged from 4 months for patients presenting with congestive heart failure to 50 months for those presenting with peripheral neuropathy only. Utilizing the proportional-hazards model in a stepwise multivariate fashion to evaluate the simultaneous influence of putative risk factors as of diagnosis revealed that congestive heart failure, urine light chain, hepatomegaly, and multiple myeloma were the major factors adversely affecting survival during the first year after diagnosis. Serum creatinine, multiple myeloma, orthostatic hypotension, and monoclonal serum protein were the most important variables adversely affecting survival for patients surviving 1 year. These models were used to categorize patients according to the variables in the models into low-, moderate-, and high-risk groups for the first year after diagnosis and separately for subsequent years. The influence of these variables on survival is important in stratification of patients randomized to prospective clinical trials.     

Primary gastrointestinal lymphoma: a clinicopathological study of 58 cases.

BACKGROUND. Primary non Hodgkin's lymphoma (NHL) of the gastrointestinal tract (GI) is the most frequent extranodal lymphoma accounting for approximately 40% of all extranodal primary NHL. The role of surgery and other treatment modalities in the management of these patients is still controversial. PATIENTS AND METHODS. We reviewed the records of 68 patients with primary GI-NHL. Ten patients had incomplete records and were excluded from further evaluation. The records of 58 patients were considered, and all were available for analysis and follow-up. RESULTS. The most frequent site of involvement was the stomach (47 patients), followed by ileum (7 patients), large bowel (3 patients) and duodenum (1 patient). Malignant lymphomas of follicular center cell origin represented the most prevalent histologic types, accounting for 58% (34 of 58) of all cases. Stage, evaluated according to the criteria of Musshoff, was Ie in 15 cases, IIe in 16, IIIe in 7, and IV in the remaining 20 cases. The median survival for the entire group of 58 patients was 54 months, with 46% of patients surviving at 5 years. The median survival was 71 months for patients in stage I-II, 60 for patients in stage III, and 25 for patients in stage IV (p = 0.016). Moreover, we found significantly improved survival in patients undergoing surgical tumor resection (p = 0.003). CONCLUSIONS. Even if at the present time the optimal management of primary GI-NHL is difficult to assess, our data suggest that it is prudent to advise resection followed by adjuvant CT in most patients, whereas CT alone should be considered only when surgery cannot be performed.     

Primary hepatic amyloidosis: a mini literature review and five cases report

Primary hepatic amyloidosis (PHA) is characterized by abnormal deposition of monoclonal immunoglobulin light chains (AL) in the liver. This rare condition is frequently undiagnosed or misdiagnosed and can be associated with poor prognosis. At present, the precise pathogenesis is not fully understood. Despite that hepatomegaly and elevated alkaline phosphatase (ALP) are present in most patients with PHA, no specific clinical markers have been identified. Staining of hepatic tissues with Congo Red is often regarded as the "gold standard". Pharmacological therapy should aim to rapidly reduce the supply of misfolded amyloidoge- nic AL. High-dose intravenous melphalan (HDM) and autologous stem cell transplantation (ASCT) appear to be the most appropriate therapy but controversies still exist.