异基因造血干细胞移植后淋巴组织增生性疾病3例报告并文献复习

Post-transplantation lymphoproliferative disease after allogeneic hematopoietic stem cell transplantation （3 cases report and literature review）

目的探讨异基因造血干细胞移植后淋巴组织增生性疾病的危险因素、临床表现、早期诊断和治疗方法。方法回顾性分析3例亲属供者人类白细胞抗原（HLA）配型不完全相合异基因造血干细胞移植后发生淋巴组织增生性疾病患者的临床表现、实验室检查、治疗及预后。结果2例患者分别在移植后65d和51d出现高热，抗生素治疗无效，并很快出现全身淋巴结肿大，血清EB病毒（EBV）-DNA分别为3．50×10^8、1．33×10^7拷贝／ml；抗病毒治疗无效分别于移植后80d和67d死亡。另1例移植后52d出现全身淋巴结肿大，不伴发热，血清EBV-DNA为7．24×10^6拷贝／ml，由于诊断早，及时治疗，抗病毒治疗15周后肿大淋巴结消退，血清EBV—DNA转阴。结论移植后淋巴组织增生性疾病是异基因造血干细胞移植后的严重并发症之一，病死率高，临床医师应提高对其的认识，如能早期诊断、早期治疗，可明显改善预后。

Objective To study the risk factors,clinical features, and early diagnosis and treatment of the post-transplantation lymphoproliferative disease （ PTLD ） after allogeneie hematopoietie stem cell transplantation （ allo-HSCT）. Methods 3 patients suffered from PTLD after related HLA-mismatehed HSCT for hematologic malignancies were reported, and the clinical manifestations, laboratory examinations, treatments and outcomes were retrospectively analyzed. Results 2 patients presented with serious fever at 65 and 51 days after allo-HSCT individually,with no reaction to any antibiotics, then rapidly developed generalized lymphoadenopathy. The evaluations of EBV viral load in their peripheral blood were 3.5 ×10^8 eopies/ml and 1.33 ×10^7 eopies/ml,respeetively. They died at 80 and 67 days after HSCT separately. The third patient presented with generalized lymphoadenopathy at 52 days, without fever,whose EBV viral load was 7.24 ×10^6 eopies/ml. He was cured by 15 weeks antiviral therapy at last. Conclusion PTLD is one of the severe complications of allo-HSCT. Overall mortality of PTLD is high, so attention should be paid, and early diagnosis and effective treatment could obviously improve the prognosis.