新医学
新醫學
신의학
NEW CHINESE MEDICINE
2014年
12期
841-843
,共3页
腹胀%肝淀粉样变性%病理学检查%刚果红染色
腹脹%肝澱粉樣變性%病理學檢查%剛果紅染色
복창%간정분양변성%병이학검사%강과홍염색
Abdominal distension%Amyloidosis%Pathological examination%Congo red staining
淀粉样变性是以不溶性淀粉样蛋白在血管壁及器官、组织细胞外沉积为特征的一种进行性、预后不良性疾病，可累及心、肝、肾、脾、胃肠、肌肉及皮肤等组织，可分为原发性及继发性。该病早期临床症状隐匿，患者常在发生多脏器损害后就诊，临床上易误诊，诊断主要依赖于病理学检查。原发性淀粉样变性目前无根治方法，主要行对症及支持治疗，常规的治疗方案为MP方案（马法兰联合泼尼松）。该文报道1例原发性肝淀粉样变性患者，其有腹胀、食欲差的症状，肝、脾肿大明显，血碱性磷酸酶及谷胺酰转肽酶升高，有高脂血症、低蛋白血症，蛋白尿明显，蛋白电泳IgA、γ轻链均阳性，IgG、IgM、κ轻链阴性，肝病理学活组织检查及刚果红染色均提示为肝淀粉样变性，因患者无其它基础疾病，故诊断为原发性肝淀粉样变性。确诊后予护肝、调节胃肠功能等处理，患者乏力、食欲差症状较前稍改善，其后患者拒绝接受化学治疗而出院，最终死亡。
澱粉樣變性是以不溶性澱粉樣蛋白在血管壁及器官、組織細胞外沉積為特徵的一種進行性、預後不良性疾病，可纍及心、肝、腎、脾、胃腸、肌肉及皮膚等組織，可分為原髮性及繼髮性。該病早期臨床癥狀隱匿，患者常在髮生多髒器損害後就診，臨床上易誤診，診斷主要依賴于病理學檢查。原髮性澱粉樣變性目前無根治方法，主要行對癥及支持治療，常規的治療方案為MP方案（馬法蘭聯閤潑尼鬆）。該文報道1例原髮性肝澱粉樣變性患者，其有腹脹、食欲差的癥狀，肝、脾腫大明顯，血堿性燐痠酶及穀胺酰轉肽酶升高，有高脂血癥、低蛋白血癥，蛋白尿明顯，蛋白電泳IgA、γ輕鏈均暘性，IgG、IgM、κ輕鏈陰性，肝病理學活組織檢查及剛果紅染色均提示為肝澱粉樣變性，因患者無其它基礎疾病，故診斷為原髮性肝澱粉樣變性。確診後予護肝、調節胃腸功能等處理，患者乏力、食欲差癥狀較前稍改善，其後患者拒絕接受化學治療而齣院，最終死亡。
정분양변성시이불용성정분양단백재혈관벽급기관、조직세포외침적위특정적일충진행성、예후불량성질병，가루급심、간、신、비、위장、기육급피부등조직，가분위원발성급계발성。해병조기림상증상은닉，환자상재발생다장기손해후취진，림상상역오진，진단주요의뢰우병이학검사。원발성정분양변성목전무근치방법，주요행대증급지지치료，상규적치료방안위MP방안（마법란연합발니송）。해문보도1례원발성간정분양변성환자，기유복창、식욕차적증상，간、비종대명현，혈감성린산매급곡알선전태매승고，유고지혈증、저단백혈증，단백뇨명현，단백전영IgA、γ경련균양성，IgG、IgM、κ경련음성，간병이학활조직검사급강과홍염색균제시위간정분양변성，인환자무기타기출질병，고진단위원발성간정분양변성。학진후여호간、조절위장공능등처리，환자핍력、식욕차증상교전초개선，기후환자거절접수화학치료이출원，최종사망。
Amyloidosis is a progressive disease with poor prognosis,which is characterized as insolu-ble amyloid proteins deposited in vascular walls,organs or tissues,involving in heart,liver,kidney,spleen, stomach and intestine,muscle and skin,etc. It can be divided into primary and secondary amyloidosis. Early clinical symptoms of amyloidosis are reclusive. The patients constantly present with damages of multiple organs upon admission. It is likely to misdiagnose amyloidosis and the diagnosis mainly depends upon pathological ex-amination. No radical treatment of primary amyloidosis is available at present. Symptomatic and supportive therapies are mainly adopted in clinical settings. Combined treatment of melphalan and prednisone is a conven-tional approach. In this article,we reported one case of primary hepatic amyloidosis presenting with abdominal distension,poor appetite,significant hepatosplenomegaly,elevated levels of serum alkaline phosphatase and glutamyltranspeptide,hyperlipidemia hypoalbuminemia,severe albuminuria,positive IgA andγ-light chain and negative IgG,IgM and κ-light chain by protein electrophoresis. Liver pathological biopsy and Congo red stai-ning comfirmed the diagnosis of amyloidosis. Since the patient had no alternative primary diseases,he was eventually diagnosed with primary hepatic amyloidosis. The patient received treatment to protect the liver and regulate gastrointestinal function. The symptoms of hypodynamia and poor appetite were slightly alleviated. Subsequently,the patient refused to receive chemotherapy and was discharged. The patient died finally.
