Clinical Characteristics and Prognostic Analysis of Non-Amyloid Monoclonal Gammopathy of Renal Significance (MGRS) in China

作者：Yuhang Song, Yang Liu, Junru Liu, Juan Li, Jin Lu, Junling Zhuang · 发表于：Blood · 年份：2024 · DOI：10.1182/blood-2024-206458 · 研究领域：Amyloidosis: Diagnosis, Treatment, Outcomes、Pharmacological Effects and Toxicity Studies

Objective: To analyze the treatment efficacy and prognostic factors of patients with non-amyloid monoclonal gammopathy of renal significance (MGRS) across multiple centers, aiming to inform clinical management and improve patient outcomes. Methods: A retrospective study was conducted on 136 non-amyloid MGRS patients from four major hospitals in China. Patients were diagnosed through kidney biopsy, and clinical data were collected, including demographics, laboratory results, pathology types, genetic testing results, and treatment regimens. Median follow-up period was 33.5 months. The endpoints included renal response, progression, and hematologic efficacy according to the International Society of Amyloidosis (ISA) criteria. Results: The study included 136 patients, with a variety of MGRS pathologies , among which MIDD was the most frequent type (64 patients), followed by 20 PGNMID, 19 LCPT and 19 cryoglobulinemic nephropathy There were 104 patients receiving anti-plasma cell therapies, among which 71 were treated with bortezomib-based regimens. Baseline serum free light chain level >400mg/L were found to be an independent risk factor for poor hematologic response to first-line therapy (P=0.042, HR=5.98). Deep hematologic remission was a significant protective factor (p=0.006, HR=20) for renal response. The presence of t (11; 14) translocation was identified as an independent risk factor (p=0.029, HR=0.138) for poor renal response . Patients achieving deep hematologic re...