Moulin B, Ronco P M, Mougenot B, Francois A, Fillastre J P, Mignon F
Department of Nephrology, CHU Rouen, Paris, France.
Kidney Int. 1992 Jul;42(1):127-35. doi: 10.1038/ki.1992.270.
We retrospectively analyzed clinical presentation, immunopathological data and renal outcome in 13 patients with glomerulonephritis (GN) and chronic lymphocytic leukemia (CLL) or related diffuse well-differentiated lymphocytic lymphoma (WDLL) of B-cell lineage. B-cell proliferation and glomerulopathy were simultaneously diagnosed in seven of the 13 patients. Nephrotic syndrome was observed in nine patients. Serum creatinine was elevated (greater than 120 mumol/liter) in 10 patients and exceeded 400 mumol/liter in three patients. A clear cut relationship between GN and hematologic disease could be established in nine cases: five patients had MPGN caused by type I or type II cryoglobulinemia; two had MPGN or mesangial hypertrophy with circulating and deposited noncryoprecipitating monoclonal IgG K and IgM K, respectively; in the two remaining patients, monotypic IgG K glomerular deposits exhibiting fibrillary organization were observed in association with MGN or MPGN, despite the absence of circulating M-component by immunofixation. The pathophysiologic link between glomerular lesions and B-cell proliferation was further evidenced by effectiveness of specific treatment of the malignancy by chlorambucil. This drug, used in the absence of steroids, induced complete remission of nephrotic syndrome in the five patients to whom it was given. Moreover, in the five patients with creatininemia greater than 200 mumol/liter who received chemotherapy, substantial improvement in renal function was observed. These overall data demonstrate that the occurrence of GN in B-CLL and related lymphoma is not fortuitous, and testify to the paraneoplastic nature of glomerular involvement mediated by deposition and possibly processing of cryoprecipitating or noncryoprecipitating M-components.
我们回顾性分析了13例患有肾小球肾炎(GN)和慢性淋巴细胞白血病(CLL)或相关B细胞系弥漫性高分化淋巴细胞淋巴瘤(WDLL)患者的临床表现、免疫病理数据及肾脏转归。13例患者中有7例同时诊断出B细胞增殖和肾小球病变。9例患者出现肾病综合征。10例患者血清肌酐升高(大于120μmol/L),3例患者超过400μmol/L。9例患者可明确建立GN与血液系统疾病之间的关系:5例患者患有由I型或II型冷球蛋白血症引起的MPGN;2例分别患有MPGN或系膜增生,伴有循环及沉积的非冷沉淀性单克隆IgG κ和IgM κ;在其余2例患者中,尽管免疫固定法检测未发现循环M成分,但观察到与MGN或MPGN相关的单型IgG κ肾小球沉积物呈纤维状结构。苯丁酸氮芥对恶性肿瘤的特异性治疗效果进一步证明了肾小球病变与B细胞增殖之间的病理生理联系。在未使用类固醇的情况下,该药物使接受治疗的5例患者的肾病综合征完全缓解。此外,在5例肌酐血症大于200μmol/L且接受化疗的患者中,观察到肾功能有显著改善。这些总体数据表明,B-CLL及相关淋巴瘤中GN的发生并非偶然,并证明了由冷沉淀或非冷沉淀M成分的沉积及可能的处理介导的肾小球受累的副肿瘤性质。