Myeloma kidneyC 90.0
Synonym(s)
DefinitionThis section has been translated automatically.
The myeloma kidney is a paraproteinemic kidney disease. It is caused by deposits of intact immunoglobulins or immunoglobulin fragments in the renal parenchyma.
Occurrence/EpidemiologyThis section has been translated automatically.
30% of all patients with multiple myeloma develop a myeloma kidney (= cast nephropathy)
EtiopathogenesisThis section has been translated automatically.
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)
Clinical featuresThis section has been translated automatically.
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
DiagnosisThis section has been translated automatically.
Clinic, lab, ultrasound,
Diagnostic confirmation of multiple myeloma
Kidney biopsy to secure the kidney
Complication(s)This section has been translated automatically.
A myeloma kidney can lead to chronic renal failure requiring dialysis (Favà A et al. 2018).
TherapyThis section has been translated automatically.
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).
AftercareThis section has been translated automatically.
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
LiteratureThis section has been translated automatically.
- 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.