Kuriyama Takuro, Kawano Noriaki, Yamashita Kiyoshi, Ueda Akira
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital.
J Clin Exp Hematop. 2014;54(2):149-53. doi: 10.3960/jslrt.54.149.
Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (EBV-PTLD) is a complication of hematopoietic stem cell transplantation (HSCT). Standard initial treatment of patients with EBV-PTLD includes administration of rituximab or dose reduction of a calcineurin inhibitor. We report successful chemotherapeutic treatment of rituximab-resistant EBV-PTLD after HSCT in a patient with severe aplastic anemia (AA). A 38-year-old woman with antithymocyte globulin (ATG)-resistant severe AA received bone marrow transplantation from an unrelated donor (human leukocyte antigen-DR single-locus mismatch). The conditioning regimen included fludarabine, cyclophosphamide, ATG, and total body irradiation, and prophylaxis for graft-versus-host disease consisted of short methotrexate and tacrolimus. Neutrophil engraftment occurred on day 21. Left cervical lymph node swelling was observed after day 45, and analysis of a biopsy specimen revealed EBV-PTLD and a high blood EBV load (56,000 copies). The patient was treated with rituximab 4 times per week, but the lymphadenopathy continued and the blood EBV load increased to 96,000 copies. Half-dose treatment with rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisolone (R-CHOP) was initiated on day 71. After 32 days of treatment with R-CHOP, the patient's neutrophil level was restored to > 0.5 × 10(9)/L and both the lymphadenopathy and the blood EBV load (< 100 copies) were rapidly reduced. Although chemotherapy is not preferred soon after HSCT, it may be an effective strategy for treating patients with rituximab-resistant EBV-PTLD.
爱泼斯坦-巴尔病毒(EBV)相关的移植后淋巴细胞增生性疾病(EBV-PTLD)是造血干细胞移植(HSCT)的一种并发症。EBV-PTLD患者的标准初始治疗包括使用利妥昔单抗或减少钙调神经磷酸酶抑制剂的剂量。我们报告了1例重症再生障碍性贫血(AA)患者HSCT后对利妥昔单抗耐药的EBV-PTLD的成功化疗治疗。一名38岁对抗胸腺细胞球蛋白(ATG)耐药的重症AA女性接受了来自无关供体(人类白细胞抗原-DR单倍型错配)的骨髓移植。预处理方案包括氟达拉滨、环磷酰胺、ATG和全身照射,移植物抗宿主病的预防措施包括短期甲氨蝶呤和他克莫司。中性粒细胞于第21天植入。第45天后观察到左颈部淋巴结肿大,活检标本分析显示为EBV-PTLD且血液EBV载量高(56,000拷贝)。患者每周接受4次利妥昔单抗治疗,但淋巴结病持续存在且血液EBV载量增加至96,000拷贝。在第71天开始使用利妥昔单抗、环磷酰胺、长春新碱、多柔比星和泼尼松龙(R-CHOP)进行半量治疗。R-CHOP治疗32天后,患者的中性粒细胞水平恢复至>0.5×10⁹/L,淋巴结病和血液EBV载量(<100拷贝)均迅速降低。虽然HSCT后不久不首选化疗,但它可能是治疗利妥昔单抗耐药的EBV-PTLD患者的有效策略。