Terzani E, Alterini B, Doni M, Lampronti V, Lusini C, Fazzini G
Divisione di Medicina Interna IV, Policlinico di Careggi, Firenze.
Ann Ital Med Int. 1994 Apr-Jun;9(2):100-4.
We report the case of a rather rare form of K-light chain deposition disease (LCDD) in a 61-year-old man with hypertension and rapidly progressing nephropathy. Laboratory findings prompted suspicion of the diagnosis which was confirmed by light-microscopic and immunofluorescent studies of samples taken by percutaneous renal and liver biopsy. Hepatic and urinary K-light chains were present; no circulating light chains were detected. Bone marrow examination evidenced mild infiltration of lymphoid cells, all positive for K-light chain staining. Plasma cells were within normal ranges. LCDD appeared as nodular glomerulosclerosis with rare crescents and extensive tubular involvement with K-light chain deposits. There was no evidence of altered liver function, nor was amyloid found in the bone marrow, kidney or liver. After one year of continuous therapy with melphalan and prednisone, the patient's renal function has not worsened. We conclude with a review of the clinical and physiopathological features of the light chain subgroup of monoclonal immunoglobulin deposition diseases (MIDD).
我们报告了一例61岁患有高血压和快速进展性肾病的男性患者,其罹患一种相当罕见的K轻链沉积病(LCDD)。实验室检查结果引发了对该诊断的怀疑,经经皮肾活检和肝活检所取样本的光镜及免疫荧光研究得以确诊。肝和尿中存在K轻链;未检测到循环轻链。骨髓检查显示淋巴细胞轻度浸润,所有细胞K轻链染色均呈阳性。浆细胞数量在正常范围内。LCDD表现为结节性肾小球硬化,偶见新月体形成,肾小管广泛受累并伴有K轻链沉积。未发现肝功能改变的证据,骨髓、肾脏或肝脏中也未发现淀粉样物质。在接受美法仑和泼尼松持续治疗一年后,患者肾功能未恶化。我们最后对单克隆免疫球蛋白沉积病(MIDD)轻链亚组的临床和生理病理特征进行了综述。