Graduate Medical Sciences Division, Boston University School of Medicine, Boston, Massachusetts, USA.
Amyloid. 2009;16(2):103-7. doi: 10.1080/13506120902879574.
A 55-year-old woman with primary Immunoglobulin light chain (AL) systemic amyloidosis died due to spontaneous rupture of her liver following treatment with high-dose melphalan and autologous stem cell transplant (HDM/SCT). She was first diagnosed after developing nephrotic-range proteinuria. Spontaneous rupture of her liver occurred 10 days after treatment with HDM/SCT and was complicated by septic shock. She was not eligible for surgical intervention and died shortly after. Amyloid fibrils were extracted from the autopsied liver sample (05-135L) and the biochemical nature of the fibrils was analyzed using electrophoretic and immunohistochemical techniques. Our testing showed that the fibrils were composed of immunoglobulin lambda light chains that were not glycosylated. While the liver is often involved in AL amyloidosis, this is the first documented case of a spontaneous hepatic rupture in a patient during treatment with HDM/SCT. A literature review of spontaneous liver rupture in patients with amyloidosis is presented.
一位 55 岁女性因原发性免疫球蛋白轻链（AL）系统性淀粉样变性症接受大剂量美法仑和自体干细胞移植（HDM/SCT）治疗后，肝脏自发性破裂死亡。她最初在出现肾病范围蛋白尿后被诊断为该病。在接受 HDM/SCT 治疗 10 天后，她的肝脏发生自发性破裂，并伴有感染性休克。她不符合手术干预的条件，并在不久后死亡。从尸检肝脏样本（05-135L）中提取淀粉样纤维，并使用电泳和免疫组织化学技术分析纤维的生化性质。我们的检测结果显示，纤维由未糖基化的免疫球蛋白 lambda 轻链组成。虽然肝脏通常会受累于 AL 淀粉样变性症，但这是在接受 HDM/SCT 治疗期间，患者肝脏自发性破裂的首例记录病例。本文还对淀粉样变性症患者的自发性肝破裂进行了文献复习。
