Abstract: SA-PO1091
Clinical and Pathological Analysis of Renal Light Chain Deposition Disease With or Without Cast Nephropathy
Session Information
- Pathology and Lab Medicine: Clinical
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pathology and Lab Medicine
- 1502 Pathology and Lab Medicine: Clinical
Authors
- Li, Dan Yang, Peking university first hospital, Beijing, China
- Ying, Gao Xin, Xining First People's Hospital, Xining, China
- Wang, Su Xia, Laboratory of Electron Microscopy, Peking University First Hospital, Bejing, China
Background
To investigate the clinicopathological features of renal light chain deposition disease (LCDD) with or without cast nephropathy (LCDD & LCN).
Methods
The clinical features of the 55 cases of LCDD diagnosed by renal biopsy from January 2000 to March 2018 were retrospectively analyzed. The pathological sections were re-read and divided into two groups: isolated LCDD group (I-LCDD, 36 cases) and LCDD coexisted with cast nephropathy group (LCDD & LCN, 19 cases). The semi-quantitative evaluation of the main features of renal pathology include the degree of glomerular mesangial expansion, acute tubular injury, tubulointerstitial inflammatory cell infiltration, tubular atrophy with interstitial fibrosis, and renal arteriosclerosis.
Results
all LCDD patients (55 cases) had a mean age of 53.6 years with a male to female ratio of 2.7:1, and the average duration of the disease was 10.3 months. The multiple myeloma (MM) accounted for 31.58% in LCDD patients. The type of light chain is predominantly κ (κ: λ, 2.6:1). The incidence of hypertension, anemia, renal dysfunction, urine protein (≥3.5 g/24h) and microscopic hematuria in patients with LCDD were 43.6%, 80.4%, 82.1%, 54.5%, and 76.4%, respectively. The percentage of LCDD & LCN was 34.6% (19/55 cases). Compared with the I-LCDD group, the LCDD&LCN group had an acute onset (P=0.04), higher serum creatinine (P=0.000), lower hemoglobulin level (p=0.027). The composition of urine protein was mainly small-molecule protein in LCDD&LCN and albumin dominant urinary protien in I-LCDD respectively (P = 0.007, P = 0.021). The type of light chain was λ dominant (λ:κ=7:3) in LCDD&LCN (vs I-LCDD, λ:κ=3:11, p=0.035). The glomerulopathy varied from mild mesangial expansion to typical mesangial nodular sclerosis, and crescents were existed in 27.3% of LCDD patients. The frequency of typical mesangial nodular sclerosis was significant lower in LCDD&LCN patients (15.8% vs 58.2% in I-LCDD, p=0.01).There was no significant difference in tubulointerstitial lesions between the two groups.
Conclusion
One third of LCDD coexisted with cast nephropathy. The dominant λ light chain and less mesangial nodular sclerosis was the prominent features of LCDD&LCN compared with isolated LCDD, which suggested LCDD&LCN has a distinct pathogenesis from that of I-LCDD.