Болезнь депозитов легких цепей — гематологическая проблема

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.