Harrison R F, Hawkins P N, Roche W R, MacMahon R F, Hubscher S G, Buckels J A
Department of Pathology, University of Birmingham.
Gut. 1996 Jan;38(1):151-2. doi: 10.1136/gut.38.1.151.
The first case of amyloidosis is reported in which spontaneous massive hepatic haemorrhage necessitated emergency liver transplantation. Liver transplantation, as a treatment for a failing liver due to amyloidosis has not been previously reported. At transplantation, the liver tissue was uncharacteristically friable, although the subsequent vascular and biliary anastomoses were uncomplicated. Histological examination of the liver showed a surprisingly modest amount of amyloid, which was shown immunohistochemically to be derived from lysozyme, and a striking absence of reticulin staining. Both the patient's father and paternal grandfather had died from spontaneous hepatic haemorrhage, and histological review of their liver tissue showed similarly modest deposition of lysozyme-derived amyloid associated with loss of reticulin staining. In each case the quantity of amyloid was far less than would be expected to interfere with the mechanical integrity of the liver. This is the only report of hepatic disintegration associated with absence of reticulin staining, and it is probable that the mechanism represents a novel secondary effect of the amyloid deposits in the livers of this family.
首例淀粉样变性病例被报道，该病例中自发性大量肝出血使得紧急肝移植成为必要。肝移植作为治疗因淀粉样变性导致肝功能衰竭的方法此前尚未见报道。移植时，肝脏组织异常脆弱，不过随后的血管和胆管吻合未出现并发症。肝脏的组织学检查显示淀粉样物质的量出人意料地少，免疫组织化学显示其来源于溶菌酶，且网状纤维染色显著缺失。患者的父亲和祖父均死于自发性肝出血，对他们肝脏组织的组织学复查显示，同样存在少量源自溶菌酶的淀粉样物质沉积，并伴有网状纤维染色缺失。在每个病例中，淀粉样物质的量远低于预期会干扰肝脏机械完整性的量。这是唯一一篇关于与网状纤维染色缺失相关的肝脏崩解的报道，很可能该机制代表了这个家族肝脏中淀粉样沉积物的一种新的继发效应。
