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A Pediatric Case of Sclerodermatous Graft-Versus-Host Disease Responsive to Ultraviolet A1 Phototherapy

机译:紫外线A1光疗反应的小儿皮硬化移植物抗宿主病

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摘要

Graft-versus-host disease (GVHD) is one of the major complications after hematopoietic stem cell transplantation and is responsible for post-therapeutic morbidity, mortality, and poor quality of life of recipients. Sclerodermatous graft-versus-host disease (sGVHD) is a rare variant of chronic GVHD characterized by deposition of collagen in the skin and other soft tissues and resulting in loss of range of motion and functional capabilities. Treatment of sGVHD is challenging and largely limited by systemic side effects. Ultraviolet A1 phototherapy has been reported to be effective in connective tissue disorders, including sGVHD. We report a case of sGVHD in a 15-year-old girl that was resistant to traditional therapy but showed improvement in cutaneous symptoms with ultraviolet A1 phototherapy three times a week for 6 weeks (10 J/cm(2) single dose, 180 J/cm(2) cumulative dose).
机译:移植物抗宿主病(GVHD)是造血干细胞移植后的主要并发症之一,是导致治疗后发病率,死亡率和受体生活质量差的原因。硬皮移植物抗宿主病(sGVHD)是一种慢性GVHD的罕见变体,其特征是胶原蛋白沉积在皮肤和其他软组织中,导致运动范围和功能能力丧失。 sGVHD的治疗具有挑战性,并且在很大程度上受到全身副作用的限制。据报道,紫外线A1光疗法可有效治疗结缔组织疾病,包括sGVHD。我们报告了一个15岁女孩的sGVHD病例,该女孩对传统疗法有抵抗力,但紫外线A1光疗一周六次(每次10 J / cm(2)单剂量,180 J)显示出改善的皮肤症状/ cm(2)累积剂量)。

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