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Clinicopathological characteristics and long-term prognosis of monoclonal immunoglobulin light chain associated Fanconi syndrome #MMPMID33796233
Chen Z; Li J; Shi X; Wang Y; Xia P; Ye W; Ye W; Qin Y; Li H; Li M; Li X; Wen Y; Chen L
Ther Adv Hematol 2021[]; 12 (?): 2040620720983127 PMID33796233show ga
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 m(2). 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.