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目的分析原发性肝淀粉样变的临床表现和诊治经过,旨在提高对该病的认识和诊治水平。方法对北京协和医院的4例和国内1978年以来报道的6例肝淀粉样变进行回顾性总结。结果(1)临床表现主要包括:肝大(90%),其中肋下平均(6·9±4·3)cm,剑下平均(9·1±3·3)cm,腹胀(80%),移动性浊音(60%),纳差(50%),乏力(40%),下肢水肿(40%),消瘦(40%),腹痛(30%)等。(2)实验室检查主要表现为碱性磷酸酶明显升高(100%),平均为(558·3±517·2)U/L,其他肝功能受损较轻,单克隆蛋白检查阳性率为85·7%(6/7)。(3)肝淀粉样变的惟一确诊手段是肝穿刺行组织病理检查,淀粉样物质沉积主要部位在窦状隙和间质4例(57·1%),沉积在血管壁的2例(28·6%);肝穿合并出血1例。(4)治疗与预后:本组患者3例给予MP方案(马法兰+泼尼松)进行化疗,1例患者给予反应停+α-干扰素(α-IFN)治疗,其中2例好转,2例自动出院,其他主要以对症治疗为主;死亡4例,死亡原因为肝功能衰竭、并发感染等。结论单克隆蛋白升高、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,提示考虑肝淀粉样变。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。目前治疗仍多采用MP方案(马法兰+泼尼松),预后较差。
Objective To analyze the clinical manifestations and diagnosis and treatment of primary hepatic amyloidosis and to improve the understanding and diagnosis and treatment of the disease. Methods Four cases of Peking Union Medical College Hospital and 6 cases of hepatic amyloidosis reported in China since 1978 were retrospectively reviewed. Results (1) The main clinical manifestations included: liver enlargement (90%), average costal approach (6.9 ± 4.3cm), average sphincter (9.1 ± 3.3cm), abdominal distension (80%), , Dullness of movement (60%), anorexia (50%), weakness (40%), lower extremity edema (40%), weight loss (40%), abdominal pain (30%) and so on. (2) Laboratory tests showed that alkaline phosphatase was significantly increased (100%), with an average of (558.3 ± 517.2) U / L, with less impairment of other liver functions. The positive rate of monoclonal protein test 85.7% (6/7). (3) The only diagnostic method for hepatic amyloidosis was histopathological examination of hepatic puncture. There were 4 cases (57.1%) of the main parts of amyloid deposition in the sinusoid and interstitium, 2 cases (28 · 6%); liver penetration and bleeding in 1 case. (4) treatment and prognosis: 3 patients in this group were given MP regimen (melphalan + prednisone) for chemotherapy, and 1 patient was treated with alpha-interferon (IFN-α), of which 2 patients improved and 2 patients Automatic discharge, the other mainly symptomatic treatment-based; death in 4 cases, the cause of death for liver failure, concurrent infections. Conclusion Monoclonal protein is elevated, liver enlargement is obviously associated with alkaline phosphatase and is not parallel with other liver biochemical indicators, suggesting that hepatic amyloidosis is considered. The only way to confirm the diagnosis of liver biopsy histopathology, but should pay attention to complications such as bleeding. The current treatment is still more use of MP regimen (melphalan + prednisone), the prognosis is poor.