Park Miguel A, Mueller Paul S, Kyle Robert A, Larson Dirk R, Plevak Matthew F, Gertz Morie A
Division of General Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Medicine (Baltimore). 2003 Sep;82(5):291-8. doi: 10.1097/01.md.0000091183.93122.c7.
The liver is a common site of amyloid deposition in primary systemic amyloidosis. We reviewed the clinical features and natural history of patients with primary systemic amyloidosis and biopsy-proven hepatic involvement who were evaluated at Mayo Clinic from January 1, 1975, to December 31, 1997. The median age of the study group (68 men; 30 women) was 58.5 years. Seventy-one patients (72%) had involuntary weight loss. Hepatomegaly was found in 79 patients (81%). Eighty-two patients (89%) had proteinuria, and 81 patients (86%) had elevated serum alkaline phosphatase levels. Seventy-six patients (83%) had either a serum or urine monoclonal protein. Before liver biopsy, clinicians considered amyloidosis in the differential diagnosis for only 14 patients (26%). None of our patients experienced hepatic rupture or death due to liver biopsy, and only 4 (4%) bled after liver biopsy. The median survival of the 98 patients was 8.5 months. Predictors of a poor prognosis were congestive heart failure, elevated concentrations of bilirubin, and a platelet count greater than 500 x 109/L. In conclusion, clinicians should consider the diagnosis of primary hepatic amyloidosis in patients who present with involuntary weight loss or hepatomegaly. Other clues to the diagnosis include an unexplained elevated serum alkaline phosphatase level, proteinuria, and evidence for hyposplenism (for example, Howell-Jolly bodies on peripheral blood smear). Liver biopsy was safe. Some patients benefit from systemic chemotherapy.
肝脏是原发性系统性淀粉样变性中淀粉样蛋白沉积的常见部位。我们回顾了1975年1月1日至1997年12月31日在梅奥诊所接受评估的原发性系统性淀粉样变性且经活检证实有肝脏受累患者的临床特征和自然病程。研究组（68名男性；30名女性）的中位年龄为58.5岁。71名患者（72%）出现非自愿体重减轻。79名患者（81%）发现肝肿大。82名患者（89%）有蛋白尿，81名患者（86%）血清碱性磷酸酶水平升高。76名患者（83%）血清或尿液中有单克隆蛋白。在肝脏活检前，临床医生仅将14名患者（26%）的鉴别诊断考虑为淀粉样变性。我们的患者均未因肝脏活检发生肝破裂或死亡，只有4名患者（4%）在肝脏活检后出血。98名患者的中位生存期为8.5个月。预后不良的预测因素是充血性心力衰竭、胆红素浓度升高和血小板计数大于500×10⁹/L。总之，临床医生应考虑对出现非自愿体重减轻或肝肿大的患者进行原发性肝淀粉样变性的诊断。其他诊断线索包括不明原因的血清碱性磷酸酶水平升高、蛋白尿和脾功能减退的证据（例如，外周血涂片上的豪-乔小体）。肝脏活检是安全的。一些患者从全身化疗中获益。
