Lafargue Marie-Camille, Cohen Camille
Université Paris Cité, Assistance Publique-hôpitaux de Paris, hôpital Necker, service de néphrologie, 149, rue de Sèvres, 75015 Paris, France.
Inserm U1151 « mechanisms and therapeutic strategies of chronic kidney diseases », hôpital Necker, université Paris Cité, service de néphrologie et transplantation rénale, hôpital Necker, Assistance Publique-hôpitaux de Paris, Paris, France.
Bull Cancer. 2024 Jul-Aug;111(7-8):741-747. doi: 10.1016/j.bulcan.2022.12.014. Epub 2023 Feb 15.
Various hematologic malignancies can lead to renal complications. The most common of these hemopathies to affect the kidney is multiple myeloma, however an increasing number of kidney diseases are associated with other monoclonal gammopathies. It is recognized that clones in small abundance can be responsible for severe organ damage, thus the concept of monoclonal gammopathy of renal significance (MGRS) has emerged. Although the hemopathy in these patients is more consistent with monoclonal gammopathy of undetermined significance (MGUS) than with multiple myeloma, the diagnosis of a renal complication changes the therapeutic management. Preservation and restoration of renal function is possible with treatment targeting the responsible clone. In this article, we take as an example immunotactoid and fibrillary glomerulopathies, two distinct entities with different etiologies and consequently different management. Immunotactoid glomerulopathy is most often associated with monoclonal gammopathy or chronic lymphocytic leukemia, the deposits on renal biopsy are monotypic, and treatment is therefore based on clone targeting. Fibrillary glomerulonephritis, on the other hand, is caused by autoimmune diseases or solid cancers. Deposits on renal biopsy are in the vast majority polyclonal. There is a specific immunohistochemical marker, DNAJB9, and treatment is less well established.
多种血液系统恶性肿瘤可导致肾脏并发症。这些血液病中最常影响肾脏的是多发性骨髓瘤，然而，越来越多的肾脏疾病与其他单克隆丙种球蛋白病相关。人们认识到，少量的克隆也可能导致严重的器官损害，因此出现了具有肾意义的单克隆丙种球蛋白病（MGRS）这一概念。尽管这些患者的血液病与意义未明的单克隆丙种球蛋白病（MGUS）比与多发性骨髓瘤更为一致，但肾脏并发症的诊断改变了治疗管理方式。针对相关克隆进行治疗有可能保留和恢复肾功能。在本文中，我们以免疫触须样和纤维样肾小球病为例，这是两种病因不同、治疗方法也不同的不同疾病实体。免疫触须样肾小球病最常与单克隆丙种球蛋白病或慢性淋巴细胞白血病相关，肾活检沉积物为单型，因此治疗基于针对克隆。另一方面，纤维样肾小球肾炎由自身免疫性疾病或实体癌引起。肾活检沉积物绝大多数为多克隆。有一种特定的免疫组化标志物DNAJB9，其治疗方法尚不明确。
