改进单克隆免疫球蛋白沉积病的肾反应评估:挑战、限制和需要达成共识

IF 13.4 1区 医学 Q1 HEMATOLOGY
Sabine Karam, Matthew J. Pianko, Hani Hassoun
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引用次数: 0

摘要

单克隆免疫球蛋白沉积病(MIDD)是一种罕见的浆细胞或b细胞疾病,其特征是单克隆免疫球蛋白分子以完整形式或片段形式沉积,最常见于肾脏,但有时也见于其他器官。MIDD的类型包括轻链沉积病、重链沉积病或轻链和重链沉积病[1]。肾MIDD与其他肾脏病变一起被归类为肾意义单克隆γ病(MGRS)。MIDD最常见于浆细胞疾病,但也可发生在淋巴样或淋巴浆细胞性肿瘤。如果不及时治疗,大多数患者将发展为终末期肾病(ESKD),导致显著的发病率和死亡率。本报告所涉及的肾性MIDD的临床表现、治疗和结局的数据主要来自回顾性研究,目前只有一项小型前瞻性研究的数据[1]。尽管如此,肾脏MIDD患者的临床表现已经有了很好的描述[3,4,5,6,7,8]。MIDD影响肾小球和小管,蛋白尿和肾小球滤过率(eGFR)的改变是关键的临床表现。在诊断时,由eGFR反映的肾脏疾病程度和蛋白尿程度均由受影响的肾间室(肾小球、血管、肾小管)、病理/结构损伤程度以及间质纤维化和肾小管萎缩(IFTA)的百分比[4]决定。肾小球范围蛋白尿是一种常见的疾病,约有20-60%的病例表现为肾病综合征[3,5,6]。然而,很少或没有蛋白尿的血管形式也被描述为[6]。2004年以来的研究已经证实,针对MIDD的克隆定向治疗既安全又有效[9,10,11,12,13,14,15],最近的研究表明,对治疗的深度血液学反应与肾脏反应和生存率的改善有关[3,5,6,16]。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Refining renal response assessment in monoclonal immunoglobulin deposition disease: Challenges, limitations and need for consensus

Refining renal response assessment in monoclonal immunoglobulin deposition disease: Challenges, limitations and need for consensus

Refining renal response assessment in monoclonal immunoglobulin deposition disease: Challenges, limitations and need for consensus
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来源期刊
Leukemia
Leukemia 医学-血液学
CiteScore
18.10
自引率
3.50%
发文量
270
审稿时长
3-6 weeks
期刊介绍: Title: Leukemia Journal Overview: Publishes high-quality, peer-reviewed research Covers all aspects of research and treatment of leukemia and allied diseases Includes studies of normal hemopoiesis due to comparative relevance Topics of Interest: Oncogenes Growth factors Stem cells Leukemia genomics Cell cycle Signal transduction Molecular targets for therapy And more Content Types: Original research articles Reviews Letters Correspondence Comments elaborating on significant advances and covering topical issues
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