Léčba mnohočetného myelomu s projevy choroby z ukládání lehkých řetězců (Light Chain Deposition Disease – LCDD). Kompletní remise s negativitou minimální reziduální nemoci po léčbě daratumumabem, dexametazonem a lenalidomidem. Popis případu a přehled lite

Abstract

Summary

Treatment of multiple myeloma with manifestations of Light Chain Deposition Disease (LCDD). Complete remission with minimal residual disease negativity following treatment with daratumumab, dexamethasone, and lenalidomide. A case report and a review of the literature.

Light chain deposition disease (LCDD) is a term used for kidney damage by clonal kappa light chain deposits in an amorphous unorganized form. In the kidneys, it causes a decrease in filtration, nephrotic syndrome with microscopic hematuria and proteinuria. Changes induced by FLC kappa binding to mesangium structures cause irreversible kidney damage. Similar deposits can damage the heart, lungs and liver. LCDD is more commonly diagnosed in patients with non-malignant monoclonal gammopathy who, had it not been for kidney damage, would have met the MGUS diagnosis, than in people who met the criteria for multiple myeloma.

The first symptom of the disease in the described case was nephrotic syndrome. Kidney biopsy showed evidence of LCDD-type kidney damage and subsequent haematological examination showed multiple myeloma, although no lytic changes were present. The first treatment line based on bortezomib, completed with high-dose chemotherapy, achieved a 31-month complete remission of the disease. Then, due to increasing FLC kappa concentrations and increasing FLC kappa/lambda ratio, it was necessary to initiate a second line of treatment based on antiCD38 antibody.

After 24 months of treatment with daratumumab, lenalidomide and dexamethasone, complete haematological remission was achieved with minimal residual disease negativity. Thanks to the early initiation of second-line treatment, kidney function remained without further deterioration.

The fate of the kidneys in patients with LCDD, which causes irreversible kidney damage, depends on early diagnosis of light chain monoclonal gammopathy, i.e. FLC examination in people with oedema of unclear etiology or with increasing creatinine concentration, early histological diagnosis and effective treatment leading to suppression of nephrotoxic light chain formation, i.e. to achieve complete remission with minimal residual disease negativity. It is essential not to forget to perform examinations in patients with fluid retention and oedema, which can prove nephrotic syndrome. And if ti is proven, ask specialists for a differential diagnosis of nephrotic syndrome, which can lead to evidence of kidney damage by monoclonal gammopathy.

Key words: Light chain deposition disease, free light chain.