Immunoglobulin heavy/light chain assay in the diagnosis, monitoring and follow-up of renal AL amyloidosis patients at different disease stages

IF 3 3区医学 Q2 HEMATOLOGY

Annals of Hematology Pub Date : 2025-04-09 DOI:10.1007/s00277-025-06345-7

Yujie Wang, Feng Liu, Yunyun Liu, Shihui Dong, Yao lin, Xumeng Liu, Shaoshan Liang, Dandan Liang, Feng Xu, Xiaodong Zhu, Fan Yang, Lei Ma, Xinchen Yao, Xiaoyu Wang, Caihong Zeng

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引用次数: 0

Abstract

Immunoglobulin light chain (AL) amyloidosis is a rare clonal plasma cell disorder with high rate of missed diagnosis, misdiagnosis and mortality. Conventional assays, such as serum immunofixation electrophoresis (IFE) and serum free light chain (FLC) assay, are unable to accurately detect low concentrations of monoclonal protein (M protein), especially as a patient’s renal function deteriorates. The heavy/light chain (HLC) assay, a relatively new method, can quantify intact immunoglobulins in serum and has proven to be valuable in the diagnosis and monitoring of multiple myeloma (MM). However, there is limited research on its application in AL amyloidosis. In this study, we evaluate the value of HLC assay in AL amyloidosis patients at different disease stages, and compare it to the performance of IFE and FLC assay. Among 40 untreated patients, 34 (85%) were positive for IFE, 34 (85%) had an abnormal free light chain ratio (FLCr), and 31 (78%) had an abnormal heavy light chain ratio (HLCr). Among 67 serum samples obtained from 44 treated patients, 57 (85%) were positive for IFE, 9 (13%) had abnormal FLCr, and 45 (67%) had abnormal HLCr. There were 1 (14%) of 7 patients in complete response (CR), 17 (68%) of 25 patients in very good partial response (VGPR), 9 (82%) of 11 patients in partial response (PR) and 6 (75%) of 8 patients in no response (NR) showed an abnormal HLCr. Our findings identified the potential value of the HLC assay in the detection of M proteins and response and serologic residual disease monitoring.

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免疫球蛋白重/轻链试验在肾AL淀粉样变不同病程诊断、监测及随访中的应用

免疫球蛋白轻链（AL）淀粉样变性是一种罕见的克隆性浆细胞疾病，具有较高的漏诊、误诊率和死亡率。传统的检测方法，如血清免疫固定电泳（IFE）和血清游离轻链（FLC）检测，不能准确检测低浓度的单克隆蛋白（M蛋白），特别是当患者肾功能恶化时。重/轻链（HLC）分析是一种相对较新的方法，可以定量血清中完整的免疫球蛋白，并已被证明在多发性骨髓瘤（MM）的诊断和监测中有价值。然而，其在AL淀粉样变性中的应用研究有限。在本研究中，我们评估了hplc检测在AL淀粉样变性患者不同疾病阶段的价值，并将其与IFE和FLC检测的性能进行了比较。40例未经治疗的患者中，34例（85%）IFE阳性，34例（85%）自由轻链比（FLCr）异常，31例（78%）重轻链比（HLCr）异常。在44例治疗患者的67份血清样本中，57例（85%）IFE阳性，9例（13%）FLCr异常，45例（67%）HLCr异常。7例完全缓解（CR）患者中有1例（14%），25例极好部分缓解（VGPR）患者中有17例（68%），11例部分缓解（PR）患者中有9例（82%），8例无缓解（NR）患者中有6例（75%）出现HLCr异常。我们的研究结果确定了hplc测定在M蛋白检测、反应和血清学残留疾病监测方面的潜在价值。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Annals of Hematology 医学-血液学

CiteScore

5.60

自引率

2.90%

发文量

304

审稿时长

2 months

期刊介绍： Annals of Hematology covers the whole spectrum of clinical and experimental hematology, hemostaseology, blood transfusion, and related aspects of medical oncology, including diagnosis and treatment of leukemias, lymphatic neoplasias and solid tumors, and transplantation of hematopoietic stem cells. Coverage includes general aspects of oncology, molecular biology and immunology as pertinent to problems of human blood disease. The journal is associated with the German Society for Hematology and Medical Oncology, and the Austrian Society for Hematology and Oncology.