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.

Long term outcomes in monoclonal gammopathy of renal significance

“…Similarly, although there was no effect of bortezomibbased therapy on RS detected, improved RS following bortezomib has previously been observed in other MGRS studies (Cohen et al, 2015;Sayed et al, 2015;Li et al, 2016;Chauvet et al, 2017;Ziogas et al, 2017). This may be explained by the confounding effect of CKD stage, because patients with more advanced kidney disease at diagnosis were more likely to receive clone-directed treatment at an earlier time point along their disease course.…”

“…We describe a cohort of fairly recently treated MGRS patients and their outcomes. LCDD was the most prevalent MGRS histology previously reported (Sayed et al , ; Li et al , ). However, despite higher prevalence, our data shows no statistically significant difference in RS ( P = 0·1) in LCDD compared to other MGRS pathologies.…”

“…Patients with mild tubal atrophy on renal biopsy had a RS of 77Á1%% compared to 72Á9% (P = 0Á63) in patients who had moderate to severe tubal atrophy. Tubular atrophy in MGRS has previously been identified (Li et al, 2016), but not correlated to RS. The degree of chronic damage seen on renal biopsy is used by nephrologists as a common determinant of renal prognosis and is also considered when deciding how aggressively to initiate immunosuppressive therapy in kidney disease.…”

Outcomes of patients with renal monoclonal immunoglobulin deposition disease

“…Subsequently, we performed univariate and multivariate analyses to identify risk factors for progression to RRT. On the basis of this and prior reports that have recognized these as prognostic factors, we considered age, dFLC , serum albumin < 3 (ROC defined cut‐off) , GFR < 20 mL/min/1.73 m 2 , type of therapy, hematologic and renal response as prognostic factors (Table ). On univariate analyses a hematologic CR or VGPR, renal response based on either AL or IMWG criteria were predictive of renal survival whereas a GFR < 20 mL/min/1.73 m 2 was predictive of progression to RRT.…”

“…The mainstay of treatment for MIDD is control of the underlying monoclonal plasma cell clone. Recent reports suggest that deep clonal responses to chemotherapy are associated with improved renal outcomes . However prognostic factors, optimal treatment strategy, renal and hematologic response criteria for this group of patients or the outcomes of patients that progress or relapse after first line treatment have not been described in detail.…”