Dialysis Unit, Institute of Nephrology and Hypertension, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel.
Laboratory of FMF and Amyloidosis, Heller Institute of Medical Research, Sheba Medical Center, Tel Hashomer, 52621, Ramat Gan, Israel.
Clin Exp Nephrol. 2019 Jun;23(6):763-772. doi: 10.1007/s10157-019-01699-5. Epub 2019 Feb 8.
Plasma cell dyscrasias (PCD) comprise a wide spectrum of disorders, which may adversely affect the kidney. However, in some PCD cases associated with kidney disease, the routine laboratory tests may be incapable to determine precisely the form of PCD, i.e., benign or malignant. Moreover, the kidney biopsy needed for precise diagnosis may be risky or declined. To overcome these limitations, we have developed and reported a new non-invasive technique based on serum free light chains (FLC) monomer (M) and dimer (D) pattern analysis (FLC MDPA), which allowed differentiation between malignant and benign PCD forms. The objective of our retrospective study was to demonstrate the utility of FLC MDPA in solving ten puzzling PCD cases complicated with kidney disease (patients 1-10).
Ten patients with uncertain form of PCD or with a questionable response to treatment were studied. In addition to routine laboratory tests and clinical evaluation of the PCD patients, our previously developed FLC MDPA in sera and biochemical amyloid typing in biopsy tissues were applied.
The FLC MDPA aided the diagnosis of the PCD underlying or accompanying the kidney disease in patients 1-5, and helped to interpret properly the response to treatment in patients 1, 6-10. The FLC MDPA findings were confirmed by a biochemical analysis of tissue amyloid deposits and subsequently by the outcome of these patients.
FLC MDPA is a non-invasive diagnostic test useful in the management of ambiguous cases of PCD associated with kidney disease.
浆细胞异常增生症（PCD）包括广泛的疾病谱，可能对肾脏造成不良影响。然而，在一些与肾脏疾病相关的 PCD 病例中，常规实验室检查可能无法准确确定 PCD 的形式，即良性或恶性。此外，为了进行准确诊断而需要的肾脏活检可能存在风险或被拒绝。为了克服这些限制，我们开发并报告了一种基于血清游离轻链（FLC）单体（M）和二聚体（D）模式分析（FLC MDPA）的新的非侵入性技术，该技术允许区分恶性和良性 PCD 形式。我们回顾性研究的目的是证明 FLC MDPA 在解决十个伴有肾脏疾病的浆细胞异常增生症（患者 1-10）的疑难病例中的实用性。
研究了 10 名 PCD 形式不确定或对治疗反应可疑的患者。除了对 PCD 患者进行常规实验室检查和临床评估外，我们还应用了先前开发的血清 FLC MDPA 和活检组织的生化淀粉样蛋白分型。
FLC MDPA 辅助了患者 1-5 中肾脏疾病相关或伴发的 PCD 的诊断，并有助于正确解释患者 1、6-10 对治疗的反应。FLC MDPA 的发现通过组织淀粉样蛋白沉积物的生化分析得到证实，随后通过这些患者的结局得到证实。
FLC MDPA 是一种非侵入性诊断测试，可用于管理伴有肾脏疾病的浆细胞异常增生症的疑难病例。
