Bence Jones proteinuria
| Classification according to ICD-10 | |
|---|---|
| R80 | Isolated proteinuria Bence-Jones proteinuria |
| ICD-10 online (WHO version 2019) | |
As Bence Jones proteinuria is defined as the presence of low molecular weight nephrotoxic Para proteins , called Bence Jones proteins in the urine , the monoclonal synthesized free light chain . It is not an independent disease, but rather a symptom of monoclonal gammopathy , such as multiple myeloma or monoclonal gammopathy of unclear significance . In the kidney , the Bence Jones proteins cause damage. This can lead to kidney failure .
causes
Diseases that may cause Bence Jones proteinuria:
- Monoclonal gammopathy of unclear significance (MGUS) with formation of light chains (so-called light chain MGUS).
- Multiple myeloma. If only free light chains are produced, this is called also Leichtkettenplasmozytom , Bence Jones myeloma , or light chain disease . However, Bence Jones proteinuria can also occur in IgG or IgA producing multiple myeloma.
- Waldenström's disease
- Lymphoma
- Amyloidosis
- transient monoclonal gammopathy in infections
diagnosis
Bence Jones proteinuria is usually diagnosed by the detection of monoclonal free light chains of the kappa or lambda type in the urine , with tests of the serum (including the determination of free light chains ) providing important information about the underlying disease. A combination of immunofixation electrophoresis from serum and / or urine , serum protein electrophoresis or urine protein electrophoresis, as well as the quantitative determination of the free light chains has proven to be particularly sensitive.
therapy
Depending on the cause of the Bence Jones proteinuria, therapy may not be necessary. Otherwise the focus is on the therapy of the underlying monoclonal gammopathy. In the context of dialysis in the case of acute kidney failure, a special filter, the so-called "high cut off membrane", can temporarily reduce the concentration of lambda and kappa chains from the blood by filtering them out. However, this therapy is purely palliative and must be supplemented by curative therapy .
history
The name of this proteinuria goes back to the English doctor Henry Bence Jones , who for the first time was able to establish a connection between proteinuria and multiple myeloma.
literature
- JE Scherberich: Laboratory tests in nephrology. In: Nephrology specialist. Elsevier, 2008, pp. 4-135.
- wikilite.com: A brief history of diagnostic tests for myeloma: Bence Jones protein and beyond English language site with information about the discovery of the Bence Jones protein and about monoclonal gammopathies in general.
Individual evidence
- ↑ B. Neumeister, I. Bensenthal, H. Liebich: Clinic guidelines for laboratory diagnostics. 3. Edition. Urban & Fischer, ISBN 3-437-22231-7 .
- ↑ Gerd Herold: Internal Medicine 2009 . Self-published, Cologne 2009.
- ↑ B. Neumeister, I. Bensenthal, H. Liebich: Clinic guidelines for laboratory diagnostics. 3. Edition. Urban & Fischer, ISBN 3-437-22231-7 .
- ^ SV Rajkumar et al .: International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma . In: Lancet Oncology . November 15, 2014, pp. E538 – e548. doi : 10.1038 / leu.2010.60 . PMID 20410922 .
- ↑ A Dispenzieri et al .: International Myeloma Working Group guidelines for serum-free light chain analysis in multiple myeloma and related disorders . In: Leukemia . 23, No. 2, November 20, 2008, pp. 215-224. doi : 10.1038 / leu.2008.307 . PMID 19020545 .
- ^ Henry Bence Jones: On a new substance occurring in the urine of a patient with "mollities ossium." In: Phil Trans Royal Soc London. 1848, pp. 55-62. ( Full text ).
