Department of Pathology and Laboratory Medicine, Harbor-UCLA Medical Center, Torrance, CA 90502, USA.
Department of Ophthalmology, Ronald Reagan UCLA Medical Center, 757 Westwood Plaza, Los Angeles, CA 90095, USA.
Exp Mol Pathol. 2018 Apr;104(2):151-154. doi: 10.1016/j.yexmp.2018.03.003. Epub 2018 Mar 15.
Amyloidosis is a disorder characterized by the deposition of insoluble abnormal proteins in the extracellular space. It may occur as a localized lesion or as a systemic disease involving multiple organs and systems. Localized conjunctival amyloidosis is rare and is less frequently associated with systemic involvement. Although amyloidosis itself is a benign lesion involvement of multiple organs and systems is associated with poor prognosis. Diagnosis of amyloidosis is made on biopsy specimens with Congo red staining for the appearance of apple-green birefringence under polarized light microscopy. Liquid chromatography tandem-mass spectrometry (LC-MS/MS) is much more sensitive in diagnosing amyloidosis and can determine the type of amyloid deposit. Here we reported a case of conjunctival amyloidosis in a 52 year-old male patient who was presented with left lower eyelid swelling to our medical center. He has a complicated past medical history of anti-phospholipid antibody syndrome, Buerger's disease (thromboangitis obliterans), and small cell lymphoma (SLL) of the right orbit/eyelid. The patient received radiation to the right orbit to treat SLL with therapy completed one and a half years prior to presentation. Physical examination revealed a firm, raised yellowish colored lesion in the left lower conjunctiva. The conjunctival lesion was biopsied, and tissue sections were examined with Congo red stains and LC-MS/MS analysis. The biopsy showed amyloid deposits without evidence of malignancy, and the type of proteins in the deposit was immunoglobulin light chain (AL) of kappa type. A complete work up was taken for possible systemic involvement of amyloidosis and results were all negative. To our knowledge, this is the first case of localized conjunctival amyloidosis with a history of contralateral orbit/eyelid SLL.
淀粉样变性是一种以细胞外不可溶性异常蛋白沉积为特征的疾病。它可以表现为局限性病变或系统性疾病,累及多个器官和系统。局限性结膜淀粉样变性罕见,且较少与系统性受累相关。尽管淀粉样变性本身是良性病变,但累及多个器官和系统与预后不良相关。淀粉样变性的诊断基于活检标本,刚果红染色后在偏振光显微镜下观察到苹果绿双折射。液相色谱串联质谱(LC-MS/MS)在诊断淀粉样变性方面更为敏感,可确定淀粉样沉积物的类型。本文报道了 1 例 52 岁男性患者的结膜淀粉样变性病例,该患者因左眼下眼睑肿胀至我院就诊。他既往有复杂的病史,包括抗磷脂抗体综合征、伯格氏病(血栓闭塞性脉管炎)和右侧眼眶/眼睑小细胞淋巴瘤(SLL)。患者曾接受右侧眼眶放疗以治疗 SLL,治疗完成于就诊前 1 年半。体格检查显示左眼下结膜有一个坚实、隆起的黄色病变。对结膜病变进行了活检,并用刚果红染色和 LC-MS/MS 分析进行了组织学检查。活检显示有淀粉样沉积物,但无恶性证据,沉积物中的蛋白类型为κ型免疫球蛋白轻链(AL)。对可能存在的系统性淀粉样变性进行了全面检查,结果均为阴性。据我们所知,这是首例有对侧眼眶/眼睑 SLL 病史的局限性结膜淀粉样变性病例。