Lillian and Henry M. Stratton-Hans Popper Department of Pathology.
Hepatol Res. 2009 Jun;39(6):577-84. doi: 10.1111/j.1872-034X.2008.00483.x. Epub 2009 Jan 12.
This long-term study aimed to evaluate recurrence and evolution of primary biliary cirrhosis (PBC) after orthotopic liver transplantation (OLT).
We reviewed "blindly" allograft biopsy specimens of women who underwent transplantation for PBC (n = 84), and women who received a transplant for chronic hepatitis C virus infection (CHCV ) (n = 108). All needle liver biopsy specimens obtained more than 6 months post-OLT were examined, including 83 specimens from 44 PBC patients and 152 specimens from 58 CHCV patients.
Granulomatous destructive cholangitis was found in five biopsies from four PBC patients (P = 0.0048). Non-necrotizing epithelioid cell granulomas were present in four biopsies from four PBC patients, and in two biopsies from one CHCV patient. Piecemeal necrosis (P = 0.0002), lobular necroinflammatory activity (P < 0.0001), steatosis (P < 0.0001) and fibrosis (P < 0.0001) were more prevalent in CHCV patients than PBC patients. Four PBC patients developed histologic evidence of autoimmune hepatitis (AIH), at a mean time of 3.66 years post-OLT. One of these patients had histologic features of AIH/PBC overlap syndrome. All four patients developed bridging fibrosis (n = 2) or cirrhosis (n = 2). No other PBC patient had evidence of cirrhosis after OLT.
Histologic findings indicative of recurrent PBC were present in 15.9% of the PBC patients undergoing biopsy in this series. However, this group of patients did not suffer significant bile duct loss or fibrosis, as compared to the control group, suggesting that recurrent PBC is a mild or slowly progressive disease. Histologic evidence of AIH was observed in allograft biopsies of some PBC patients.
本长期研究旨在评估原发性胆汁性肝硬化(PBC)患者接受原位肝移植(OLT)后的复发和演变。
我们“盲目”回顾了 84 例接受 PBC 移植和 108 例接受慢性丙型肝炎病毒感染(CHCV)移植的女性患者的肝移植活检标本。检查了所有 OLT 后 6 个月以上获得的针芯肝活检标本,包括 44 例 PBC 患者的 83 份标本和 58 例 CHCV 患者的 152 份标本。
在 4 例 PBC 患者的 5 份活检标本中发现了肉芽肿性破坏性胆管炎(P = 0.0048)。在 4 例 PBC 患者的 4 份活检标本和 1 例 CHCV 患者的 2 份活检标本中发现了非坏死性上皮样细胞肉芽肿。碎片状坏死(P = 0.0002)、小叶坏死性炎症活动(P < 0.0001)、脂肪变性(P < 0.0001)和纤维化(P < 0.0001)在 CHCV 患者中比 PBC 患者更为常见。4 例 PBC 患者在 OLT 后 3.66 年内出现了自身免疫性肝炎(AIH)的组织学证据。其中 1 例患者具有 AIH/PBC 重叠综合征的组织学特征。这 4 例患者均出现桥接纤维化(n = 2)或肝硬化(n = 2)。OLT 后,没有其他 PBC 患者出现肝硬化的证据。
在本系列接受活检的 PBC 患者中,有 15.9%存在提示复发 PBC 的组织学发现。然而,与对照组相比,这组患者并未出现明显的胆管损失或纤维化,提示复发的 PBC 是一种轻度或进展缓慢的疾病。在一些 PBC 患者的移植肝活检标本中观察到 AIH 的组织学证据。