Myeloma kidney C 90.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

CAST nephropathy; MCN; Myeloma cast nephropathy

Definition
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The myeloma kidney is a paraproteinemic kidney disease. It is caused by deposits of intact immunoglobulins or immunoglobulin fragments in the renal parenchyma.

Occurrence/Epidemiology
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30% of all patients with multiple myeloma develop a myeloma kidney (= cast nephropathy)

Etiopathogenesis
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Caused by the myeloma kidney toxic effects of the light chains on the renal tubules through accumulation of the light chains Atrophy of the tubule cells

Clinical features
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The myeloma kidney may exhibit one or more of the following symptoms:

  • Nephrotic syndrome with proteinuria <3g/24
  • Renal insufficiency with normal sized kidneys and bland urine sediment
  • Night sweat of Bence-Jones proteins and low levels of albumin

Diagnosis
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Clinic, lab, ultrasound,

Diagnostic confirmation of multiple myeloma

Kidney biopsy to secure the kidney

Complication(s)
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A myeloma kidney can lead to chronic renal failure requiring dialysis (Favà A et al. 2018).

Therapy
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Conventional chemotherapy with melphalan (Alkeran®, 15 mg/m² i.v. as bolus day 1) + prednisolone (60 mg/m² p.o. day 1-4), repetition day 29

Polychemotherapy according to VAD protocol (vincristine, adriamycin, dexamethasone) before planned high-dose chemotherapy or in case of insufficient response to melphalan + prednisolone or in renal insufficiency

High-dose chemotherapy (with melphalan) followed by autologous stem cell transplantation

Questionable: High cutoff hemodialysis (HCO-HD). The procedure is suitable for removing large amounts of paraproteins from the blood. The long-term outcome of patients treated in this way is rather disappointing (Hutchison CA et al. 2019).

Aftercare
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Medical history, physical examination, immunoglobulins quantitatively and Bence-Jones proteins in urine, blood count, calcium, creatinine, bone marrow puncture, X-ray control of osteolytic foci

Literature
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  • Agrawal P et al (2019) Monoclonal Gammopathy of Renal Significance Triggered by Viral E Hepatitis. Indian J Nephrol 29:50-52.
  • Ashraf M et al (2019) Waldenström Macroglobulinemia: Unusual Presentation With Cast Nephropathy/Light Chain Tubulopathy. Clin Med Insights Case Rep 12:1179547619828704. https://www.ncbi.nlm.nih.gov/pubmed/30799965
  • Favà A et al (2018) Treatment of multiple myeloma with renal involvement: the nephrologist's view. Clin Kidney J 11:777-785. https://www.ncbi.nlm.nih.gov/pubmed/30524711
  • Hutchison CA et al (2019) High cutoff versus high-flux haemodialysis for myeloma cast nephropathy inpatients receiving bortezomib-based chemotherapy (EuLITE): a phase 2 randomised controlled trial.lancet Haematol 6:e217-e228. https://www.ncbi.nlm.nih.gov/pubmed/30872075
  • Kanzaki G et al (2019) Monoclonal Immunoglobulin Deposition Disease and Related Diseases. J Nippon Med Sch 86:2-9.
  • Mukherjee T et al (2019) Cholemic nephrosis (bile cast nephropathy) with severe liver dysfunction. Med J Armed Forces India 75:216-218.
  • Leung N et al (2018) Dysproteinemias and Glomerular Disease. Clin J Am Soc Nephrol 13:128-139.
  • Luan H et al (2019) Cast nephropathy associated with monoclonal immunoglobulin M-secreting mucosa-associated lymphoid tissue B-cell lymphoma.Clin Nephrol doi: 10.5414/CN109682.
  • Sallée M et al (2019) Myeloma cast nephropathy: the dusk of high cutoff haemodialysis. Lancet Haematol 6:e174-e176.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020