| 原发性肝淀粉样变1例报告并文献回顾 |
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| 引用本文： | 唐郑一,柳思琪,邵雪,张传辉,金珍婧,杨岚岚.原发性肝淀粉样变1例报告并文献回顾[J].现代肿瘤医学,2020,0(1):75-78. |
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| 作者姓名： | 唐郑一 柳思琪 邵雪 张传辉 金珍婧 杨岚岚 |
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| 作者单位： | 吉林大学第二医院肝胆胰内科，吉林 长春 130041 |
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| 摘 要： | 目的:报道1例原发性肝淀粉样变病例，提高该病的认识水平，减少误诊误治。方法:分析我院2015年03月诊治并经病理学确诊的肝淀粉样变1例，并结合国内外文献，对本病的临床表现、诊断、治疗及预后进行讨论分析。结果:患者临床表现为右上腹隐痛、腹胀、肝大；既往:1年前因脾破裂于我院肝胆外科行脾切除术；术后病理:（脾）内有较多出血，有血肿形成，周围脾白髓萎缩，红髓间质有玻璃样变性，符合陈旧性脾破裂伴血肿形成。肝功提示:碱性磷酸酶明显升高，白蛋白减低，球蛋白升高，肝脏CT示肝脏体积明显增大，肝脏实质强化不均匀，胆囊结石。肝穿刺组织病理活检:肝细胞索变性坏死，有炎细胞浸润，肝索内肝细胞间有粉染无结构物质，免疫组化染色结果:CD34(-)、刚果红染色(+)。结论:对肝淀粉样变缺乏诊治经验是该病误诊的原因之一，以腹痛、肝大为主要临床表现极易误诊为脂肪肝，肝硬化及原发性肝癌。当临床上遇到肝脏肿大明显伴碱性磷酸酶明显升高且与其他肝功生化指标变化不平行时，尤其合并多器官受累表现者，须警惕肝淀粉样变的可能，确诊的惟一方法是肝穿刺行组织病理检查，但应注意出血等并发症。
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| 关 键 词： | 肝淀粉样变 肝肿大 肝穿刺活检 |
A report for primary hepatic amyloidosis and literature review |
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| Tang Zhengyi,Liu Siqi,Shao Xue,Zhang Chuanhui,Jin Zhenjing,Yang Lanlan.A report for primary hepatic amyloidosis and literature review[J].Journal of Modern Oncology,2020,0(1):75-78. |
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| Authors: | Tang Zhengyi Liu Siqi Shao Xue Zhang Chuanhui Jin Zhenjing Yang Lanlan |
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| Affiliation: | Department of Hepatopancreatobiliary，the Second Hospital，Jilin University，Jilin Changchun 130041，China. |
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| Abstract: | Objective:To report a case of primary hepatic amyloidosis,so as to improve perception of the disease and reduce misdiagnosis and mistherapy of the disease.Methods:Discuss and analyze the clinical manifestations,diagnosis,treatment and prognosis of primary hepatic amyloidosis with a case report diagnosed by pathology of our hospital in April 2015 and review the national and international literature.Results:It was a patient with right upper quadrant pain,abdominal distension and hepatauxe who had gotten splenectomy one year ago in our hepatopancreatobiliary surgery department.The spleen pathology showed old splenic rupture including hemorrhage,hematoma,splenic white pulp atrophy and splenic red pulp hyaline degeneration.Liver function displayed high ALP,low albumin and high globulin.Abdominal CT showed hepatomegaly with liver parenchyma inhomogeneous enhancement and cholecystolithiasis.Hepatic biopsy showed hepatic cords degeneration and necrosis,inflammatory cell infiltration,pink with no structural material between liver cells in hepatic cords.Immunohistochemistry showed CD34(-),Congo red staining(+).Conclusion:Lack of experience of diagnosis and treatment of Primary Hepatic Amyloidosis is one of the reasons of misdiagnosis of the disease.The disease characterized by abdominal pain and hepatomegaly is easily misdiagnosed as fatty liver disease,liver cirrhosis or primary liver cancer.We must maintain keen vigilance when we found a patient with hepatomegaly and significantly increased ALP,but unevenness with other liver function biochemical criterion,especially multiple organs involved.The only way to diagnose is liver biopsy,but hemorrhage as complication is severe. |
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| Keywords: | hepatic amyloidosis hepatomegaly liver biopsy |
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