Primary systemic amyloidosis: a case report

Jayashankar CA; D. S. Somasekar; Pavan Kumar Perugu; Santosh KV; Manjunath Nandennavar; Praveen Mathew

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Primary systemic amyloidosis: a case report

机译：原发性系统性淀粉样变性病：一例报告

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Primary amyloidosis is a group of monoclonal plasma cell disorders, characterized by extracellular deposition of immunoglobulin light chain fibrils in multiple organs leading to progressive multiorgan dysfunction. It is a rare disease which usually occurs in elderly persons and has a poor prognosis. We report a 72-year-old male patient with chronic abdominal distension, bilateral pitting pedal edema with nephrotic range proteinuria and amyloid deposition in liver and bone marrow. Immunoelectrophoresis of serum demonstrated the presence of immunoglobulin light chains of the circulating monoclonal protein. He was treated with IV bortezomib and IV dexamethasone.

机译：原发性淀粉样变性是一组单克隆浆细胞疾病，其特征在于免疫球蛋白轻链原纤维在多个器官中的细胞外沉积，导致进行性多器官功能障碍。这是一种罕见的疾病，通常发生在老年人中，预后较差。我们报告了一名72岁的男性患者，患有慢性腹胀，双侧门水肿，肾病范围蛋白尿和淀粉样蛋白沉积在肝脏和骨髓中。血清的免疫电泳表明存在循环单克隆蛋白的免疫球蛋白轻链。他接受静脉注射硼替佐米和地塞米松静脉注射治疗。

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《International Journal of Research in Medical Sciences》 |2014年第2期|共5页
作者
Jayashankar CA; D. S. Somasekar; Pavan Kumar Perugu; Santosh KV; Manjunath Nandennavar; Praveen Mathew;
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中图分类医药、卫生;
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1. Primary Systemic Amyloidosis and High Levels of Angiotensin-Converting Enzyme: Two Case Reports [J] . J.Praena-Segovia, A.Sanchez-Gastaldo, M.Bernabeu-Wittel, Case Reports in Cardiology . 2013,第1期

机译：原发性系统性淀粉样变性病和高水平的血管紧张素转换酶：2例报告
2. Purpuric halo around hemangioma as a clue for primary systemic amyloidosis: Case report and review of literature [J] . Yin-Shuo Chang, Kuo-Hsien Wang, Hsiou-Hsin Tsai, Dermatologica Sinica . 2018,第1期

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3. Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report [J] . Li Tian, Anliu Tang, Xian Zhang, BMC Gastroenterology . 2017,第1期

机译：不完全性肠梗阻和血细胞减少症是多器官受累的原发性系统性AL淀粉样变性病的罕见特征：
4. DEFINITION OF ORGAN INVOLVEMENT AND TREATMENT RESPONSE IN PRIMARY SYSTEMIC AMYLOIDOSIS (AL): A CONSENSUS OPINION FROM THE 10~(TM) INTERNATIONAL SYMPOSIUM ON AMYLOID AND AMYLOIDOSIS [C] . Morie A- Gertz, Ray Comenzo, Rodney H. Falk, International Symposium on Amyloidosis . 2005

机译：原发性全身淀粉样源病（AL）的器官参与和治疗反应的定义：10〜（TM）国际淀粉样蛋白和淀粉样症症的国际研讨会中的共识意见
5. The role of transthyretin cysteine modification in senile systemic amyloidosis. [D] . Kingsbury, Jonathan S. 2007

机译：运甲状腺素蛋白半胱氨酸修饰在老年系统性淀粉样变性中的作用。
6. Primary Systemic Amyloidosis and High Levels of Angiotensin-Converting Enzyme: Two Case Reports [O] . J. Praena-Segovia, A. Sanchez-Gastaldo, M. Bernabeu-Wittel, 2013

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7. Lung adenocarcinoma expressing receptor for advanced glycation end-products with primary systemic AL amyloidosis: a case report and literature review [O] . 2017

机译：晚期糖基化终末产物伴原发性系统性AL淀粉样变性的肺腺癌表达受体：一例病例报告并文献复习

Primary systemic amyloidosis: a case report

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