肝移植术后淋巴组织增生性疾病的诊断和治疗(附1例报告及文献复习)

Diagnosis and treatment of posttransplant lymphoproliferative disease after liver transplantation(a case report and literature review)

目的探讨肝移植术后淋巴组织增生性疾病(posttransplant lymphoproliferative disease,PTLD)的诊断和治疗。方法回顾性分析1例肝移植后PTLD的临床表现、诊断和治疗过程,并复习相关文献。结果 2003年10月至2011年12月在中山大学附属第三医院肝移植中心随访的336例肝移植术后患者中有1例发生PTLD,发生率为0.3%。该例患者的临床表现不典型,主要表现为反复发热、腹部不适、多处淋巴结肿大和肝脏占位病变等,淋巴结活组织检查提示B细胞源性非霍奇金淋巴瘤淋巴瘤,确诊为PTLD。经减少免疫抑制剂用量、抗炎,对症支持治疗无效后死亡。结论肝移植术后PTLD的临床表现不典型,易被误诊或延误诊断,预后甚差。治疗以减少免疫抑制剂用量、抑制淋巴细胞增殖、对症支持治疗为主。

Objective To investigate the diagnosis and treatment of posttransplant lymphoprolifera- tire disease （PTLD） after liver transplantation （LT）. Methods Clinical data of one ease of PTLD, inclu- ding clinical manifestations, diagnosis and treatment, were retrospectively analyzed. And the related literatures were reviewed. Results From October 2003 to December 2011, one case developed PTLD among 336 patients who were followed up after LT in the Third Affiliated Hospital of Sun Yat-sen University. The incidence of PTLD was 0. 3%. Clinical manifestations of the patient weren＇t typical, including frequent fever, abdominal discomfort, multiple lymphadenopathy and focal hepatic lesions. The patient was diagnosed as B cell derived non-Hodgkin＇s disease by pathological examination of the swollen lymph node. Reduction of immunosuppressive dosage, antibiotics and supportive treatment were provided, but the outcome was poor. The patient died finally. Conclusions The clinical manifestations of PTLD is nontypical so that misdiagnosis and missed diagnosis were common, and the prognosis is poor. The main therapy includes immunosuppressant reduction, inhibil：ion of lymphoproliferation and supportive treatment.