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Treatment of multiple myeloma with manifestations of light chain deposition disease; complete remission with minimal residual disease negativity following treatment with daratumumab, dexamethasone, and lenalidomide – a case report and a review of the literature
Authors: Z. Adam 1; J. Svojanovský 2; I. Svobodová 3; A. Tkadlec 4; M. Borský 5; M. Krejčí 1; M. Štork 1; I. Boichuk 1; Z. Král 1; L. Pour 1
Authors‘ workplace: Interní hematologická a onkologická klinika (IHOK) LF MU a FN Brno 1; I. ústav patologie LF MU a FN u sv. Anny v Brně 2; II. interní klinika LF MU a FN U sv. Anny v Brně 3; Baťova nemocnice, Zlín 4; Centrum molekulární biologie a genetiky, Interní hematologická a onkologická klinika (IHOK) LF MU a FN Brno 5
Published in: Transfuze Hematol. dnes,32, 2026, No. 1, p. 37-47.
Category: Review/Educational Papers
doi: https://doi.org/10.48095/cctahd202609Overview
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 haematuria 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 31month 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 aetiology 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.
Keywords:
light chain deposition disease – free light chain
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PODÍL AUTORŮ NA PŘÍPRAVĚ RUKOPISU
A. T. včas diagnostikoval nefrotický syndrom nejasné etiologie a poslal pacienta na biopsii do FN u svaté Anny panu doktorovi Svojanovskému k provedení biopsie. Připomínkoval text z pohledu nefrologa.
J. S. prováděl biopsii ledviny a podílel se na textu z úhlu pohledu nefrologa a psal text o biopsiích ledvin, které rutinně provádí.
I. S. hodnotila bioptický vzorek z biopsie ledviny a podílela se na textu z úhlu pohledu patologa specializovaného na nefropatologii.
M. B. hodnotil kostní dřen metodou průtokové cytometrie.
Z. A., L. P., M. K., M. Š., I. B. a Z. K. se podíleli na léčbě nemoci tohoto pacienta i na textu z úhlu pohledu hematologů.
Článek vznikl složením pohledů všech spolupracujících lékařů do jednotného textu.
ČESTNÉ PROHLÁŠENÍ
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Doručeno do redakce dne: 31. 1. 2025.
Přijato po recenzi dne: 20. 2. 2025.
prof. MUDr. Zdeněk Adam, CSc.
Interní hematologická a onkologická klinika
LF MU a FN Brno
Jihlavská 20
625 00 Brno Bohunice
e-mail: adam.zdenek@fnbrno.cz
Labels
Haematology Internal medicine Clinical oncology
Article was published inTransfusion and Haematology Today
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