Myeloma kidney C 90.0

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

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

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

• Multiple myeloma (aggressive B-cell NHL: per day production of about 85g monoclonal light chains = paraproteins; healthy people produce about 0.9g/day).
• Macroglobulinemia Waldenström (rare occurrence: Ashraf M et al. 2019)

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

Clinic, lab, ultrasound,

Diagnostic confirmation of multiple myeloma

Kidney biopsy to secure the kidney

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

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).

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

• 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.