[Light-chain deposition disease is a hematologic problem]
I G Rekhtina1, L P Mendeleeva1, L S Biryukova1
1National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia.
Insights
Treatment for light-chain deposition disease (LCDD) effectively reduced monoclonal light chains but rarely improved kidney function, likely due to late diagnosis and intervention in these patients with chronic kidney disease.
Area of Science:
- Nephrology
- Hematology
- Oncology
Background:
- Light-chain deposition disease (LCDD) is a rare condition characterized by the deposition of monoclonal immunoglobulin light chains in organs, primarily the kidneys.
- LCDD often presents with significant kidney injury, including chronic kidney disease and end-stage renal disease requiring dialysis.
Purpose of the Study:
- To evaluate clinical data, laboratory findings, and treatment outcomes in patients diagnosed with light-chain deposition disease (LCDD).
- To assess the efficacy of bortezomib, cyclophosphamide, and dexamethasone (VCD) therapy in patients with LCDD and kidney injury.
Main Methods:
- Retrospective analysis of nine patients with LCDD and kidney injury.
- Diagnosis confirmed by renal biopsy using light and immunofluorescence microscopy.
- All patients received VCD induction therapy, with some receiving second-line lenalidomide or high-dose melphalan chemotherapy with autologous stem cell transplantation.
Main Results:
- Six patients had multiple myeloma, and three had LCDD associated with monoclonal gammopathy primarily affecting the kidneys.
- All patients presented with chronic kidney disease (Stages III-IV) or dialysis-related renal failure.
- Hematologic response to VCD therapy was achieved in 7 of 9 patients, with 5 achieving complete remission and 3 very good partial remission.
- Renal function improvement was observed in only 2 (22%) patients.
Conclusions:
- Therapy targeting monoclonal light chains is highly effective in achieving hematologic response in LCDD patients.
- Limited renal function recovery suggests that the initiation of treatment is often delayed, highlighting the importance of early diagnosis and intervention.
- Further research is needed to optimize treatment strategies for improving renal outcomes in LCDD.
Aim:
To analyze clinical and laboratory data and treatment results in patients with light-chain deposition disease (LCDD).
Subjects And Methods:
Nine patients with LCDD and kidney injury were examined. The diagnosis was based on the results of light and immunofluorescence microscopy of renal biopsy specimens. All the patients received bortezomib, cyclophosphamide, and dexamethasone (VCD) induction therapy.
Results:
Six patients were diagnosed with multiple myeloma; in 3 patients LCDD was considered within monoclonal gammopathy manly involving the kidney. By the initiation of therapy, all the patients were diagnosed as having chronic kidney disease (Stage III (n=2), Stage IV (n=2), and dialysis-related renal failure (n=5)). After the VCD treatment, 7 of 9 patients achieved a hematologic response. Second-line therapy with lenalidomide proved to be effective in the other 2 cases. Five patients achieved complete remission; 3 had a very good partial remission. Thereafter, 2 patients received high-dose melphalan chemotherapy and autologous hematopoietic stem cell transplantation. Better renal function was noted in only 2 cases.
Conclusion:
Despite the high efficiency of therapy aimed to reduce monoclonal light chains; improved renal function was observed in only 2 (22%) patients. Such low rates of a renal response were due to the late initiation of therapy.
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