Bisaccia Emil, Palangio Mark, Gonzalez Joselyn
Columbia University College of Physicians and Surgeons, New York, NY, USA.
Transfus Apher Sci. 2011 Oct;45(2):187-90. doi: 10.1016/j.transci.2011.07.005. Epub 2011 Sep 3.
Sclerodermatous chronic graft-versus-host disease (cGVHD) following allogeneic hematopoietic stem cell transplantation (HSCT) in children is difficult to treat and life-threatening. Extracorporeal photochemotherapy (ECP; photopheresis), an immunomodulatory therapy that involves the infusion of autologous peripheral blood leukocytes after ex vivo exposure to the photoactive agent 8-methoxypsoralen and ultraviolet A radiation, is an effective treatment for steroid-refractory cGVHD. After undergoing allogeneic HSCT for pre-B-cell acute lymphoblastic leukemia, a 14-year-old boy developed extensive sclerodermatous cGVHD that was refractory to prednisone, tacrolimus, and sirolimus. ECP was administered over the course of 53 months, during which the skin softened substantially and immunosuppressive therapy was discontinued. This case suggests that long-term ECP is a viable option in children with sclerodermatous cGVHD.
儿童异基因造血干细胞移植(HSCT)后发生的硬皮病样慢性移植物抗宿主病(cGVHD)难以治疗且危及生命。体外光化学疗法(ECP;光分离置换法)是一种免疫调节疗法,包括在体外将自体外周血白细胞暴露于光活性药物8-甲氧基补骨脂素和紫外线A辐射后进行输注,是治疗类固醇难治性cGVHD的有效方法。一名14岁男孩在接受前B细胞急性淋巴细胞白血病的异基因HSCT后,出现了广泛的硬皮病样cGVHD,对泼尼松、他克莫司和西罗莫司均无效。ECP治疗持续了53个月,在此期间皮肤明显软化,免疫抑制治疗停药。该病例表明,长期ECP治疗对于患有硬皮病样cGVHD的儿童是一种可行的选择。