Audard V, Larousserie F, Grimbert P, Abtahi M, Sotto J-J, Delmer A, Boue F, Nochy D, Brousse N, Delarue R, Remy P, Ronco P, Sahali D, Lang P, Hermine O
Department of Nephrology, Henri Mondor Hospital, Paris 12 University, Creteil, Paris, France.
Kidney Int. 2006 Jun;69(12):2251-60. doi: 10.1038/sj.ki.5000341. Epub 2006 May 3.
Minimal change nephrotic syndrome (MCNS) is described as a paraneoplastic manifestation of classical Hodgkin's lymphoma (cHL). We reassessed the pathophysiological and clinical significance of this association. A retrospective study was performed to evaluate a cohort of adult patients who developed MCNS and cHL. Twenty-one patients recruited in 15 French centers were analyzed. cHL was associated with inflammatory and general symptoms in most cases. The morphological subtype was predominantly nodular sclerosis (71.4%). MCNS appeared before the diagnosis of lymphoma in eight patients (38.1%) and in this case, it was characterized by a nephrotic syndrome (NS) frequently resistant (50%) or dependent (12.5%) to steroid treatment. Interestingly, diagnosis (3-120 months after MCNS) and effective treatment of the hemopathy were associated with the disappearance of the MCNS. cHL was diagnosed before MCNS in nine patients (42.9%), and in this case, glomerulopathy was associated with cHL relapse in 55.5% of cases. In four patients (19%), the two diseases occurred simultaneously. Extensive immunohistochemical study of lymph nodes was performed in eight patients and did not reveal particular features. In conclusion, MCNS associated with cHL is frequently dependent or resistant to steroid regimen, but remission of NS is obtained with the cure of lymphoma.
微小病变肾病综合征（MCNS）被描述为经典型霍奇金淋巴瘤（cHL）的一种副肿瘤表现。我们重新评估了这种关联的病理生理和临床意义。进行了一项回顾性研究，以评估一组发生MCNS和cHL的成年患者。对在15个法国中心招募的21例患者进行了分析。在大多数情况下，cHL与炎症和全身症状相关。形态学亚型主要为结节硬化型（71.4%）。8例患者（38.1%）的MCNS出现在淋巴瘤诊断之前，在这种情况下，其特征为肾病综合征（NS），常对类固醇治疗耐药（50%）或依赖（12.5%）。有趣的是，血液病的诊断（MCNS后3 - 120个月）和有效治疗与MCNS的消失相关。9例患者（42.9%）的cHL在MCNS之前被诊断，在这种情况下，55.5%的病例中肾小球病与cHL复发相关。4例患者（19%）两种疾病同时发生。对8例患者的淋巴结进行了广泛的免疫组织化学研究，未发现特殊特征。总之，与cHL相关的MCNS常对类固醇治疗方案依赖或耐药，但淋巴瘤治愈后NS可缓解。
