论文部分内容阅读
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。
Objective: To understand the clinicopathological features of heavy chain deposition disease (HCDD). Methods: The clinical and pathological data of HCDD patients were retrospectively analyzed. Results: Of the 11 patients, 4 were males and 7 were females. The median age at renal biopsy was 45 years. The median duration of kidney disease in August. Eight patients had hypertension (72.7%), 10 patients had decreased plasma albumin, 6 (54.5%) had elevated serum creatinine, 10 patients had anemia (90.9%), and 4/7 patients (57.1%) There was a band of λ-IgG monoclonal immunoglobulin in serum, and the ratio of kappa to λ in 5 patients was abnormally elevated (> 1.65). No bone marrow was found in all patients undergoing bone marrow biopsy. The complement C3 was decreased in 9 patients (81.8% Seven patients (63.6%) had decreased C4, while 9 (81.8%) had proteinuria in the area of nephropathy and 9 (81.8%) had microscopic hematuria. All of the 11 patients had γ-type HCDD. Nodules-like lesions were observed in 5 cases (45.5%) with crescent formation, 6 cases with pure IgG1 in renal tissue, 1 case with IgG2 alone, 2 cases with IgG3 alone Cases, IgG1, IgG4 simultaneous deposition in 1 case, IgG2, IgG4 deposition in 1 case. Conclusion: HCDD is more common in middle-aged women, clinically common proteinuria, hypertension, renal insufficiency, hypo-hyperlipidemia and serum often with abnormal monoclonal bands, renal pathological glomerular nodular lesions were obvious, γ , IgG1 subtype deposition the most common.