Mills L R, Mwakyusa D, Milner P F
Department of Pathology, Veterans Administration Medical Center, Augusta, GA 30910.
Arch Pathol Lab Med. 1988 Mar;112(3):290-4.
Liver biopsy results and clinical records from 13 patients with sickle cell anemia were reviewed to assess the relative importance of local ischemia or of factors unrelated to sickling as a cause of their liver disease. Two of the biopsy specimens were normal and one showed cirrhosis. Nine patients had received multiple blood transfusions and nine had cholelithiasis, of whom two also had choledocholithiasis. Seven had both risk factors. Five had lobular cholestasis and four had acute or chronic hepatitis. One biopsy specimen showed changes of the Budd-Chiari syndrome. Another showed clear portal tract changes of large bile duct obstruction but no mechanical blockage of the biliary system; this suggests the thickened bile as postulated by Muirhead. Otherwise the changes observed were those to be expected in a heavily transfused population with a high prevalence of gallstones.
回顾了13例镰状细胞贫血患者的肝活检结果和临床记录，以评估局部缺血或与镰变无关的因素作为其肝脏疾病病因的相对重要性。两份活检标本正常，一份显示为肝硬化。9例患者接受过多次输血，9例有胆石症，其中2例还患有胆总管结石。7例同时存在这两种危险因素。5例有小叶性胆汁淤积，4例有急性或慢性肝炎。一份活检标本显示有布-加综合征的改变。另一份显示肝门区有明显的大胆管梗阻改变，但胆道系统无机械性阻塞；这提示了如缪尔黑德所假设的胆汁增厚。除此之外，观察到的改变是在大量输血且胆结石患病率高的人群中所预期出现的。
