Liang Hong-Yin, Xie Xiao-Dong, Jing Guang-Xu, Wang Meng, Yu Yang, Cui Jian-Feng
Department of General Surgery, General Hospital of Western Theater Command, Chengdu 410000, Sichuan Province, China.
The Southwest Medical University, Luzhou 410000, Sichuan Province, China.
World J Clin Cases. 2021 Nov 16;9(32):10046-10051. doi: 10.12998/wjcc.v9.i32.10046.
Jaundice is a major manifestation of posthepatectomy liver failure, a feared complication after hepatic resection. Herein, we report a case of posthepatectomy jaundice that was not caused by liver failure but by paroxysmal nocturnal hemoglobinuria (PNH)-induced hemolysis.
A 56-year-old woman underwent right hepatectomy and biliary tract exploration surgery due to hepatic duct stones. Prior to surgery, the patient was mildly anemic. The direct antiglobulin test was negative. A bone marrow biopsy showed mild histiocyte hyperplasia. After surgery, the patient suffered a progressive increase in serum bilirubin. Meanwhile, the patient developed hemolytic symptoms after blood transfusion. She was ultimately diagnosed with PNH. PNH is a rare bone marrow failure disorder that manifests as complement-dependent intravascular hemolysis with varying severity. After steroid treatment, the patient's jaundice gradually decreased, and the patient was discharged on the 35 postoperative day.
PNH-induced hemolysis is a rare cause of posthepatectomy jaundice. It should be suspected in patients having posthepatectomy hyperbilirubinemia without other signs of liver failure. Steroid therapy can be considered for the treatment of PNH in such cases.
黄疸是肝切除术后肝衰竭的主要表现,是肝切除术后令人担忧的并发症。在此,我们报告一例肝切除术后黄疸,其并非由肝衰竭引起,而是由阵发性夜间血红蛋白尿(PNH)诱导的溶血所致。
一名56岁女性因肝内胆管结石接受了右肝切除术和胆道探查术。术前,患者有轻度贫血。直接抗球蛋白试验为阴性。骨髓活检显示轻度组织细胞增生。术后,患者血清胆红素进行性升高。同时,患者输血后出现溶血症状。她最终被诊断为PNH。PNH是一种罕见的骨髓衰竭性疾病,表现为程度不同的补体依赖性血管内溶血。经类固醇治疗后,患者黄疸逐渐减轻,并于术后第35天出院。
PNH诱导的溶血是肝切除术后黄疸的罕见原因。对于肝切除术后出现高胆红素血症且无其他肝衰竭迹象的患者应怀疑此病。在此类病例中,可考虑使用类固醇疗法治疗PNH。