肝移植术后淋巴组织增生性疾病的临床病理分析

Post transplant lymphoproliferative disease of liver: A clinicopathological analysis

目的探讨肝移植术后淋巴组织增生性疾病(PTLD)的临床病理特征。方法对3例肝移植术后PTLD行HE、免疫组织化学染色及TCR、IgH基因重排检测,同时复习其临床资料并随访。结果3例中,2例位于肝门部,1例位于肝动脉旁淋巴结。临床表现为发热和梗阻性黄疸。病理诊断例1为单形性T细胞PTLD的周围T细胞淋巴瘤,例2、例3为单形性B细胞PTLD的淋巴浆细胞样淋巴瘤和弥漫大B细胞淋巴瘤。基因重排检测例1 TCR阳性,例2、例3 IgH阳性。3例EBV免疫组化染色均阳性。行再次移植降低免疫抑制剂用量并配合抗病毒、抗CD20单抗和化疗等治疗,例1、例3分别于术后6个月和4个月死于淋巴瘤复发和肺感染,例2随访11个月无瘤生存。结论PTLD是肝移植术后严重的并发症之一,具有独特的形态和临床特征。病因可能与EB病毒感染和免疫抑制有关,需经病理组织学检查确诊。

Purpose To analyze the clinical and pathological features of liver-localized post transplant lymphoproliferative disease （ PTLD）, and to investigate the clinical significance of this lesion. Methods Three cases of PTLD in our center were reviewed and studied by routine light microscopy, immunohistochemistry and TCR and IgH gene rearrangement analysis. The clinical data and followup information were also reviewed. Results In the three cases, two cases occurred in hepatic portal region and one in portal lymph node. The pathological diagnosis was monomorphic T cell PTLD and mornomorphic B cell PTLD, respectively. The clonal analysis showed TCR gene rearrangement in case 1 and sene rearrangement in case 2 and 3. All of the three cases were positive for EBV immunostaining. After immunosuppressive reduction, antiviral therapy, anti-CD20 antibody treatment and chemotherapy, cases 1 and 3 were died in the 6th month and the 4th month post-transplant and case 3 was still survival in 11 months follow-up without tumor recurrence. Conclusions PTLD is a severe complication of liver transplant with distinctive morphologic and clinical characteristics. The origin of this disease may be associated with Epstein-Barr virus infection and immunosuppressive therapy. It needs the pathologic detection to make a definite diagnosis.