PDF(Pubmed)
Abstract：
Caroli disease is a rare congenital malformation that predisposes to segmental cystic dilatation of the intrahepatic bile ducts. Banti syndrome is characterized by persistent splenomegaly due to chronic congestion, resulting in a low hematocrit and ultimately leading to pancytopenia. In this report, we describe a 29-year-old woman who presented with a >20-year history of hepatitis B surface antigen positivity and a >1-year history of recurrent fatigue and malaise. On examination, the patient had abdominal distension with marked splenomegaly (7 cm below the ribs) and ascites with tenderness of the abdominal muscles to palpation. A complete blood count showed a low white blood cell count, red blood cell count, and hemoglobin concentration. During the course of treatment, the patient developed multiple symptoms of pancytopenia and concomitant splenomegaly, and she was discharged after total splenectomy with good recovery. The combination of Banti syndrome and Caroli disease results in severe symptoms of portal hypertension.
摘要：
Caroli病是一种罕见的先天性畸形，易导致肝内胆管的节段性囊性扩张。Banti综合征的特征是由于慢性充血引起的持续性脾肿大，导致血细胞比容低，最终导致全血细胞减少症。在这份报告中,我们描述了一名29岁的女性，其乙型肝炎表面抗原阳性病史>20年，复发性疲劳和不适病史>1年。在检查中，患者腹胀伴明显脾肿大（肋骨下7cm），腹水伴腹部肌肉触诊压痛。全血细胞计数显示白细胞计数低,红细胞计数,和血红蛋白浓度。在治疗过程中,患者出现了多种全血细胞减少和合并脾肿大的症状，全脾切除术后她出院，恢复良好。Banti综合征和Caroli病的组合导致严重的门静脉高压症状。
