Sugino Noriko, Nakamura Chishiho, Fujii Sumie, Matsui Yusuke, Kaneko Hitomi, Watanabe Mitsumasa, Miura Yasuo, Wakasa Tomoko, Tsudo Mitsuru
Department of Hematology, Osaka Red Cross Hospital.
Rinsho Ketsueki. 2011 Mar;52(3):129-35.
Age-related EBV-associated B-cell lymphoproliferative disorder is a highly aggressive lymphoma, and a standard therapy for this disease has not yet been established. A 58-year-old male was admitted to our hospital because of fever and lymphadenopathy across the whole body. Neck lymph node biopsy showed hemorrhagic and geographic necrosis with Hodgkin-like large cells against a background of small lymphocytes. The large cells were positive for CD30 and EBER. The patient was diagnosed as having age-related EBV-associated B-cell lymphoproliferative disorder. Although there was no response to CHOP therapy, he obtained partial response after 3 courses of DeVIC therapy. Because his lymphoma was highly aggressive and chemotherapy-resistant, he underwent autologous stem cell transplantation with a conditioning regimen including ranimustine, etoposide, cytarabine, and melphalan. After stem cell transplantation and subsequent radiotherapy to the residual lesion, the patient achieved complete remission. This is the first report of successful autologous stem cell transplantation for a patient with age-related EBV-associated B-cell lymphoproliferative disorder.
年龄相关的EB病毒相关B细胞淋巴增殖性疾病是一种侵袭性很强的淋巴瘤,目前尚未确立针对该疾病的标准治疗方法。一名58岁男性因发热和全身淋巴结肿大入住我院。颈部淋巴结活检显示在小淋巴细胞背景下有出血性和地图状坏死,伴有霍奇金样大细胞。大细胞CD30和EBER呈阳性。该患者被诊断为年龄相关的EB病毒相关B细胞淋巴增殖性疾病。尽管CHOP治疗无效,但他在接受3个疗程的DeVIC治疗后获得了部分缓解。由于他的淋巴瘤侵袭性很强且对化疗耐药,他接受了自体干细胞移植,预处理方案包括雷莫司汀、依托泊苷、阿糖胞苷和美法仑。干细胞移植及随后对残留病灶进行放疗后,患者实现完全缓解。这是首例关于年龄相关的EB病毒相关B细胞淋巴增殖性疾病患者自体干细胞移植成功的报告。