Clinicopathological characteristics and outcomes of light chain deposition disease: an analysis of 48 patients in a single Chinese center.

ABSTRACT

To explore the clinicopathological characteristics and outcomes of light chain deposition disease (LCDD) in a Chinese population, we retrospectively studied the clinicopathological data, treatment, and outcomes of 48 patients with biopsy-proven LCDD from a single center. Among the patients, there were 29 males and 19 females, with an average age of 51 years. The patients presented with hypertension (79.2 %), edema (60.4 %), renal insufficiency (95.8 %), anemia (93.8 %), nephrotic proteinuria (≥3.0 g/24 h) (44.4 %), and hematuria (75.0 %). Moreover, 33.3 % had hypocomplementemia of C3, and 25 % were diagnosed with multiple myeloma. Serum immunofixation electrophoresis and a serum free light chain assay showed that 26.7 and 85.4 % of patients presented with monoclonal immunoglobulin, respectively. Nodular mesangial sclerosis was identified in 83.3 % of our cases and vascular involvement was observed in 77.1 % by light microscopy. Over an average of 22 months of follow-up, the mean renal survival was 32.5 months. Of the patients, 34.1 % had stable or improved renal dysfunction, 2.3 % had worsening renal function, and 63.6 % progressed to end-stage renal disease. Of the 33 patients receiving chemotherapy, 15 patients had stable or improved renal function and the renal survival was higher in patients with hematological and renal responses than in those without. The independent predictors of ESRD by multivariate analysis were serum creatinine (p = 0.008) and urinary retinol binding protein (RBP) (p = 0.045). In conclusion, LCDD was characterized in Chinese patients by renal dysfunction, hypertension, anemia, proteinuria, abnormal free light chain ratios, and less overt hematologic malignancies. Serum creatinine and RBP were independent prognostic factors of LCDD. As better hematologic and renal responses to chemotherapy were associated with improved renal survival, there is an urgent need for multicenter and prospective studies to establish the standardized therapy for LCDD.