重链沉积病的临床病理特点  

Heavy chain deposition disease: A report from Chinese patients

作　　者：范芸[1,2] 徐峰[2] 陈浩[2] 梁少姗[2] 陈惠萍[2] 张明超[2] 朱小东[2] 刘志红[2] 曾彩虹[2] 

机构地区：[1]南京大学医学院 [2]南京军区南京总医院全军肾脏病研究所,南京210016

出　　处：《肾脏病与透析肾移植杂志》2013年第3期230-237,共8页Chinese Journal of Nephrology,Dialysis & Transplantation

摘　　要：目的:了解重链沉积病(HCDD)的临床病理特点。方法:回顾性分析HCDD患者临床病理资料。结果:11例患者中男性4例,女性7例,肾活检时中位年龄45岁。肾脏病病程中位值8月。8例患者存在高血压(72.7%),10例患者血浆白蛋白减低,6例(54.5%)患者血清肌酐升高,10例患者伴贫血(90.9%),4/7例(57.1%)患者血清存在λ型IgG单克隆免疫球蛋白条带,5例血κ/λ比值异常升高(>1.65),所有患者均行骨髓活检未见骨髓瘤,9例(81.8%)患者补体C3下降,7例(63.6%)补体C4下降,9例(81.8%)伴肾病范围蛋白尿,9例(81.8%)存在镜下血尿。11例患者均为γ型HCDD,光镜表现结节样病变,5例(45.5%)伴新月体形成,肾组织单纯IgG1沉积者6例,单纯IgG2沉积者1例,单纯IgG3沉积者2例,IgG1、IgG4同时沉积者1例,IgG2、IgG4同时沉积者1例。结论:HCDD多见于中年女性,临床常见蛋白尿、高血压、肾功能不全、低补体血症及血清常伴异常的单克隆条带,肾脏病理肾小球结节性病变明显,以γ型、IgG1亚型沉积最常见。To explore the clinical and pathological characteristic of heavy chain deposition disease （HCDD） from Chinese patients. Methodolgy： The clinical and pathological data from eleven patients with biopsy-proven HCDD were retrospectively analyzed from Jan, 1990 to Oct, 2012. Result： They were 4 males and 7 females with an average age of 48.2±9.9 years old （ranged from 34～65）. Nephrotic proteinuria was indentified in 9 cases （81.8%）, heamturia in 9 （81.8%）, hypertension in 8 （72.7%）, renal dysfunction in 6 （54.5%）, and anemia in 10 cases （90.9%）. None of them was myeloma by the examination of bone marrow biopsy, and serum mono-IgGλ was detected in 4 from 7 cases （57.1%） by serum protein electrophoresis （SPEP）. Serum abnormal free light chain （FLC） ratio （〈0.26 or 〉1.65） was observed in 5 cases （45.4%）. Hypocomplementemia with C3 and C4 was present in 9 and 7 cases respectively. The levels of urine β-N-Acetyl glucosaminidase （NAG） and retinol-binding protein （RBP） were 75.5？38.2U/（g.cr） and 10.5？10.7mg/L respectively. Histological examination showed glomerular nodular lesion in all patients with crescents in 5 cases （range 4-20%）. IF presented the deposition of IgG along glomerular basement membrane and tubular basement membrane in all patients, and on arteriole walls in 8 cases. Subtype of IgG staining indicated IgG1 in 6, IgG2 in one, IgG3 in 2, both IgG2 and IgG4 in one, and both IgG1 and IgG4 in one. The staining of κ、λ chain was both negative. Dense deposits were identified in glomeruli GBM and along TBM. Conclusion： HCDD characterized by nephrotic proteinuria, hypertension, renal dysfunction and nodular lesion frequently accompanied with g heavy chain and mono IgG1 deposition.

关 键 词：重链沉积病 单克隆IgG 肾脏病理 

分 类 号：R692.9[医药卫生—泌尿科学]