Copperman Thomas S, Truong Minh Tam, Berk John L, Sobel Rachel K
a Department of Ophthalmology , Boston University, Boston Medical Center , Boston , MA , USA.
b Department of Radiation Oncology , Boston University, Boston Medical Center , Boston , MA , USA.
Orbit. 2019 Jun;38(3):210-216. doi: 10.1080/01676830.2018.1483407. Epub 2018 Jun 11.
: To evaluate the effectiveness of low dose external beam radiation therapy to halt progression of localized periocular light chain (AL) amyloidosis, a clonal plasma cell disorder. : This is a retrospective review of patients referred to a tertiary care center for external beam radiation treatment of biopsy proven localized periocular light chain amyloidosis. The primary outcome measure was clinical disease stability at one year following radiation therapy as evidenced by slit lamp exam and external photography. Pre and post radiation MRI imaging of the affected area were also used as a means to monitor disease progression. : Four symptomatic patients with localized periocular AL amyloidosis received external beam radiation therapy ranging from 20-30 Gy fractioned over 10-20 fractions. Three of the four patients had prior surgical debulking with or without ptosis repair. Amyloid deposition did not progress in any patient at one year. Further follow-up of two patients revealed amyloid progression at two years post radiation. : External beam radiation therapy for localized periocular AL amyloidosis demonstrated efficacy at halting disease progression at one year; however, the long-term efficacy is unknown. Monitoring of periocular amyloid is best achieved with slit lamp exam and external photography as opposed to MRI.
为评估低剂量外照射放疗对阻止局限性眼周轻链(AL)淀粉样变性(一种克隆性浆细胞疾病)进展的有效性。
这是一项对转诊至三级医疗中心接受活检证实的局限性眼周轻链淀粉样变性外照射放疗患者的回顾性研究。主要结局指标是放疗后一年的临床疾病稳定性,通过裂隙灯检查和外部摄影证明。受影响区域放疗前后的MRI成像也用作监测疾病进展的手段。
4例有症状的局限性眼周AL淀粉样变性患者接受了20 - 30 Gy的外照射放疗,分10 - 20次进行。4例患者中有3例曾接受过手术减瘤,伴或不伴有上睑下垂修复。一年时,所有患者的淀粉样蛋白沉积均未进展。对2例患者的进一步随访显示,放疗后两年淀粉样蛋白出现进展。
局限性眼周AL淀粉样变性的外照射放疗在一年时显示出阻止疾病进展的疗效;然而,长期疗效尚不清楚。与MRI相比,通过裂隙灯检查和外部摄影能更好地监测眼周淀粉样变性。