以肾小球内“假血栓”形成为表现的三种肾小球病的临床和病理特征的比较——3例报道及文献复习

Comparison of clinical and pathologic characteristics of three types of glomerulopathy presenting 'pseudothrombi' within glomeruli-Three case reports and a literature review

目的通过对3例以肾小球内"假血栓"形成为表现的不同类型肾小球病的临床和病理特征的比较,提髙对其病理形态特点的认识和诊断水平。方法用纤维蛋白染色(Weigert法)鉴别肾小球毛细血管腔内微血栓形成的狼疮性肾炎和"假血栓"形成为表现的脂蛋白性肾病(LPN)、冷球蛋白血症性肾小球肾炎(CrGN)和华氏巨球蛋白血症性肾小球肾炎(WMG),并对其临床特征及肾活检组织的光镜、免疫荧光和电镜观察结果进行详细比较。结果LPN、CrGN和WMG3种肾病均可以肾病综合征为表现,但其血清学检查结果显示:LPN伴发高脂蛋白血症;CrGN伴发于尿本周蛋白呈阳性的淋巴细胞增生症;WMG病例则有血清IgM单株峰升高的特点。这3种肾小球病尽管在病理形态上均以肾小球毛细血管腔内"假血栓"形成为表现,但其纤维蛋白染色结果均为阴性,显然与狼疮性肾炎病变肾小球内形成的"微血栓"不同,又结合其"假血栓"的光镜特点、免疫荧光检查和电镜观察结果可将其加以区别。结论对以肾小球内"假血栓"形成为表现的肾小球病的确诊,必须做到临床和病理学检查的密切结合。

Purpose To enhance an insight of pathological features and differential diagnosis in some nephropathies presenting ‘ pseudothrombi＇, we reported the clinical and pathologic characteristics of three types of cases with glomerulopathy. Methods Weigert method for fibrin staining was applied to distinguish microthrombi appearing in lupus nephritis（LN） and ‘ pseudothrombi＇ presenting in lipoprotein nephropathy（LPN）, cryoglobulinemic glomerulonephritis（CrGN） and Waldenstrom macroglob- ulinemic glomerulonephritis （WMG）. Clinical data and pathologic features in light microscopy, immunofluorescent staining and electron microscopy were carefully compared in three types of cases. Results LPN,CrGN and WMG all presented nephrotic syndrome clinically, but LPN associated with hyperlipoproteinemia,CrGN with Bence Jones protein in lymphocytic proliferative disease, WMG with a peak of monoclonal IgM in serum were distinctly identified in their serologic tests. Three types of glomerulopathy presented ‘pseudothrombi＇ formation in glomerular capillary lumens pathologically, however, the staining results of fibrin in the glomeruli were obviously different from the microthrombi appearing in diseased glomeruli of LN,and the differentiating diagnosis of 3 types of glomerulopathy was depended on light microscopy, immunoflurrescent staining and electron microscopy examination.Conclusions The patholgical diagnosis of some types of glomerulopathy with pseudothrombus formation in diseased glomeruli should cautiously refer to their clinical manifestation.