Institute of Pathology, Faculty of Medicine, University of Ljubljana, Korytkova 2, 1000, Ljubljana, Slovenia.
Pathol Oncol Res. 2010 Jun;16(2):249-52. doi: 10.1007/s12253-009-9218-1. Epub 2009 Dec 5.
Light chain deposition disease (LCDD) is a rare disorder associated with a clonal proliferation of plasma cells, which synthesize abnormal monoclonal immunoglobulin light chains. It is characterized by systemic deposition of light chains in various organs, with the kidneys being most commonly affected. There have been few reports of isolated LCDD, i.e. in the brain, lungs and cervical lymph nodes. We here report on another patient with an isolated form of LCDD, which was limited to the pharyngeal mucosa and was associated with an extraosseus plasmacytoma of the pharynx, expanding the spectrum that has been recognized for LCDD. The patient was treated by local radiotherapy, with an excellent response. A less aggressive clinical course can probably be expected than in the usual form of LCDD, but a long-term follow-up is necessary to establish the clinical significance of this variant of LCDD.
轻链沉积病（LCDD）是一种罕见的疾病，与浆细胞的克隆性增殖有关，这些浆细胞合成异常的单克隆免疫球蛋白轻链。其特征是在各种器官中沉积异常的轻链，其中肾脏最常受累。孤立性 LCDD（即脑、肺和颈部淋巴结）的报道较少。我们在此报告另一位孤立性 LCDD 患者，其仅局限于咽黏膜，伴有咽腔外浆细胞瘤，扩大了对 LCDD 的认识范围。该患者接受了局部放疗，反应良好。与常见的 LCDD 形式相比，这种变异型可能具有较轻的侵袭性病程，但需要长期随访以确定这种 LCDD 变异型的临床意义。
