1. Clinicopathological characteristics and long-term prognosis of monoclonal immunoglobulin light chain associated Fanconi syndrome
- Author
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Mingxi Li, Zhixin Chen, Jiaying Li, Wenling Ye, Wei Ye, Xiaoxiao Shi, Hang Li, Peng Xia, Limeng Chen, Ying Wang, Yan Qin, Yubing Wen, and Xuemei Li
- Subjects
0301 basic medicine ,Chemotherapy ,business.industry ,medicine.medical_treatment ,monoclonal immunoglobulin light chain ,renal function ,Fanconi syndrome ,Monoclonal immunoglobulin ,Renal function ,Hematology ,medicine.disease ,Immunoglobulin light chain ,chemotherapy ,03 medical and health sciences ,030104 developmental biology ,0302 clinical medicine ,030220 oncology & carcinogenesis ,medicine ,Cancer research ,Diseases of the blood and blood-forming organs ,RC633-647.5 ,business ,Rare disease ,Original Research - Abstract
Background and aims: Monoclonal immunoglobulin light chain associated Fanconi syndrome (LC-FS) is a rare disease that involves proximal tubules. As most of the reported cases came from western countries, we aimed to analyze the clinicopathological characteristics of Asian LC-FS and its treatment responses to chemotherapy. Methods: A total of 26 LC-FS patients in a single-center were retrospectively studied. Results: At diagnosis, the mean age of the 26 Asian LC-FS patients was 54.7 ± 14.7 years, with females accounting for 57.7%. They presented with different degrees of proximal tubular dysfunctions with normoglycemic glycosuria (88.0%), hyperphosphaturia (84.2%) and aminoaciduria (84.0%) as the most common features. The mean estimated glomerular filtration rate (eGFR) was (68.0 ± 26.4) ml/min per 1.73 m2. After chemotherapy, renal response was achieved in 58.3% cases, which was accompanied by hematological response, and tubular response was acquired in 66.7% cases. During 3 years of follow-up, the eGFR levels significantly decreased in the monoclonal gammopathy of renal significance patients, few of whom (21.4%) had received chemotherapy. Conclusion: Asian LC-FS patients had mild renal function disorder. The chemotherapy could improve both renal and tubular functions, which may be related to the hematological response.
- Published
- 2020