Abstract: SA-PO0832
Light and Heavy Chain Deposition Disease: Systematic Review of Case Reports
Session Information
- Glomerular Management: Real-World Lessons and Emerging Therapies
November 08, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Imai, Asataro, Japan Community Healthcare Organization Sendai Hospital, Sendai, Miyagi, Japan
- Sanada, Satoru, Japan Community Healthcare Organization Sendai Hospital, Sendai, Miyagi, Japan
- Sato, Mitsuhiro, Japan Community Healthcare Organization Sendai Hospital, Sendai, Miyagi, Japan
Background
Light and heavy chain deposition disease (LHCDD) is a rare subtype of monoclonal immunoglobulin deposition disease (MIDD). Due to its rarity, comprehensive data on its clinicopathological features and outcomes are limited. We conducted a systematic review to summarize reported cases of renal LHCDD and highlight clinical characteristics, pathological findings, treatment approaches, and outcomes.
Methods
A systematic search of PubMed and Scopus databases was performed to identify case reports and case series involving renal LHCDD, published up to October 2024. Extracted data included demographics, clinical presentation, diagnostic findings, treatment strategies, and outcomes. Descriptive statistics were used to summarize the data.
Results
A total of 80 patients from 25 studies were included. The mean age was 56.6 ± 6.1 years with 54.5% male. Mean serum creatinine level was 2.8 ± 1.4 mg/dL and urine protein was 3.6 ± 1.9 g/day. Hematuria, hypertension, nephrotic syndrome, and myeloma were observed in 78.0%, 72.2%, 37.1%, and 23.8% of cases, respectively. Monoclonal immunoglobulin was detected in the serum of 74.1% and in the urine of 71.4% of patients. An abnormal serum free light chain ratio was found in 91.7% of tested patients. Renal biopsy showed nodular mesangial sclerosis in 90.5%. Dominant immunofluorescence stainings were IgG heavy chain and monoclonal κ light chain in 76.6% and 58.4% of patients, respectively. Among 49 patients with available treatment data, 81.6% received chemotherapy and 16.3% underwent stem cell transplantation. Clone-directed therapy was applied to 32.7% of total cases. Only two patients were treated conservatively. During a median follow-up of 43 months, renal function improved or remained stable in 32.6% of cases, while 43.5% cases progressed to end-stage renal disease. The mortality rate was 26.0%.
Conclusion
This systematic review demonstrates that LHCDD is associated with poor renal outcomes. However, recent progress in the treatment using clone-directed therapies may improve the prognosis.