Kahata Kaoru, Hashino Satoshi, Takahata Mutsumi, Fujisawa Fumie, Kondo Takeshi, Kobayashi Sumiko, Fujita Yasuyuki, Shimizu Hiroshi, Imamura Masahiro, Asaka Masahiro
Department of Gastroenterology and Hematology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Acta Haematol. 2008;120(1):14-8. doi: 10.1159/000151510. Epub 2008 Aug 21.
A 22-year-old Japanese man was diagnosed with Sézary syndrome with large cell transformation. His skin lesions persisted after treatment with 7 cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone), psoralen and ultraviolet light A, and total skin electron beam irradiation. He subsequently underwent allogeneic bone marrow transplantation by reduced-intensity conditioning from a human leukocyte antigen-identical unrelated donor. He developed grade II of acute graft-versus-host disease and extensive-type chronic graft-versus-host disease. He has no signs of disease 36 months after the transplantation. The prognosis of patients with advanced stage of mycosis fungoides or Sézary syndrome is very poor. Allogeneic hematopoietic stem cell transplantation, especially by reduced-intensity conditioning, is expected to become a curative treatment option, and graft-versus-tumor effect might play a critical role for sustained remission.
一名22岁的日本男性被诊断为蕈样肉芽肿综合征伴大细胞转化。在用7个周期的CHOP（环磷酰胺、阿霉素、长春新碱和泼尼松）、补骨脂素和紫外线A以及全身皮肤电子束照射治疗后，他的皮肤病变仍持续存在。随后，他接受了来自人类白细胞抗原匹配的无关供体的低强度预处理的异基因骨髓移植。他出现了Ⅱ级急性移植物抗宿主病和广泛型慢性移植物抗宿主病。移植后36个月他没有疾病迹象。蕈样肉芽肿或蕈样肉芽肿综合征晚期患者的预后非常差。异基因造血干细胞移植，尤其是低强度预处理的移植，有望成为一种治愈性治疗选择，移植物抗肿瘤效应可能对持续缓解起关键作用。
