关键词: Clinicopathological characteristics Monoclonal gammopathy Prognosis Renal damage

Mesh : Humans Retrospective Studies Kidney Diseases / diagnosis etiology pathology Paraproteinemias / complications diagnosis Monoclonal Gammopathy of Undetermined Significance / complications diagnosis Prognosis Hematologic Neoplasms / complications

来  源:   DOI:10.1038/s41598-024-58467-z   PDF(Pubmed)

Abstract:
Renal involvement is common in monoclonal gammopathy (MG); however, the same patient may have both MG and non-paraprotein-associated renal damage. Accordingly, distinguishing the cause of renal damage is necessary because of the different clinical characteristics and associated treatments. In this multicenter retrospective cohort study, we described the clinicopathological characteristics and prognosis of 703 patients with MG and renal damage in central China. Patients were classified as having MG of renal significance (MGRS), MG of undetermined significance (MGUS), or hematological malignancy. 260 (36.98%), 259 (36.84%), and 184 (26.17%) had MGRS, MGUS, and hematological malignancies, respectively. Amyloidosis was the leading pattern of MGRS (74.23%), followed by thrombotic microangiopathy (8.85%) and monoclonal immunoglobulin deposition disease (8.46%). Membranous nephropathy was the leading diagnosis of MGUS (39.38%). Renal pathological findings of patients with hematological malignancies included paraprotein-associated lesions (84.78%) and non-paraprotein-associated lesions (15.22%). The presence of nephrotic syndrome and an abnormal free light chain (FLC) ratio were independently associated with MGRS. The overall survival was better in patients with MGUS than in those with MGRS or hematological malignancies.
摘要:
肾受累在单克隆丙种球蛋白病(MG)中很常见;然而,同一患者可能同时存在MG和非副蛋白相关性肾损害.因此,由于不同的临床特征和相关治疗方法,区分肾损害的原因是必要的。在这项多中心回顾性队列研究中,我们描述了中国中部地区703例MG合并肾损害患者的临床病理特征和预后。患者被分类为具有肾脏意义的MG(MGRS),未确定显著性MG(MGUS),或者恶性血液病.260(36.98%),259(36.84%),184人(26.17%)患有MGRS,MGUS,和血液恶性肿瘤,分别。淀粉样变是MGRS的主要类型(74.23%),其次是血栓性微血管病(8.85%)和单克隆免疫球蛋白沉积病(8.46%)。膜性肾病是MGUS的主要诊断(39.38%)。血液系统恶性肿瘤患者的肾脏病理结果包括副蛋白相关病变(84.78%)和非副蛋白相关病变(15.22%)。肾病综合征的存在和游离轻链(FLC)比率异常与MGRS独立相关。MGUS患者的总生存期优于MGRS或恶性血液病患者。
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