Prager Alisa J, Habib Larissa A, Gambogi Talita, Busam Klaus J, Marr Brian P
Department of Ophthalmology, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
Ocul Oncol Pathol. 2018 Sep;4(5):313-317. doi: 10.1159/000485918. Epub 2018 Feb 13.
Conjunctival amyloidosis is a rare cause of ocular inflammation, mass, and hemorrhage that can be difficult to diagnose and treat. In this case series, we describe 4 patients with a histopathological diagnosis of conjunctival amyloidosis treated at a single institution. All patients underwent surgical excision and biopsy. On histopathological examination, 3 patients had local deposition of either kappa or lambda monoclonal immunoglobulin light chains, favoring localized amyloid light-chain amyloidosis. Systemic workup to exclude rheumatologic disorders (e.g., anti-neutrophil cytoplasmic antibody and rheumatoid factors) and hematological disorders (e.g., imaging, biopsies, and serum protein electrophoresis/urine protein electrophoresis) was negative except for a positive abdominal fat biopsy in 1 patient. Patients were followed for an average of 6.1 years (range 4 months to 15 years) with stable ocular disease.
结膜淀粉样变性是眼部炎症、肿物及出血的罕见病因,可能难以诊断和治疗。在本病例系列中,我们描述了在单一机构接受治疗的4例经组织病理学诊断为结膜淀粉样变性的患者。所有患者均接受了手术切除及活检。经组织病理学检查,3例患者存在κ或λ单克隆免疫球蛋白轻链的局部沉积,支持局限性淀粉样轻链淀粉样变性。除1例患者腹部脂肪活检呈阳性外,排除风湿性疾病(如抗中性粒细胞胞浆抗体和类风湿因子)及血液系统疾病(如影像学检查、活检及血清蛋白电泳/尿蛋白电泳)的全身检查均为阴性。患者平均随访6.1年(范围4个月至15年),眼部疾病稳定。