Monoclonal gammopathy of renal significance

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Classification according to ICD-10
D47.2 Monoclonal gammopathy
ICD-10 online (WHO version 2019)
Various forms of monoclonal free light chain deposits

The Monoclonal gammopathy renal significance ( MGRS ) is symptomatic Monoclonal gammopathy is a kidney disorder in which the criteria for (by definition) malignant disease such as multiple myeloma are not met. MGRS is therefore a preliminary stage of the disease, in which hematological-oncological therapy may also be necessary in certain cases. In a broader sense, malignant diseases such as AL amyloidosis can also be categorized as MGRS.

Emergence

In monoclonal gammopathy of unclear significance (MGUS), malignant B lymphocytes produce monoclonal immunoglobulins and / or immunoglobulin components (e.g. free light chains ), which are also known as monoclonal protein (M protein / gradient). One speaks of a B-cell clone , since these are identical B-lymphocytes. Deposits of the monoclonal protein in the kidneys can lead to renal insufficiency, which is why this disease is known as MGRS. Most of the time, the deposited M protein is a free light chain with a high affinity for tissue. A high concentration of these monoclonal free light chains favors the deposition and thus the development of the disease. In almost a quarter of all those affected, impairment of kidney function is observed in the course of MGUS disease. Regular checks (6 months after diagnosis and annually thereafter) are therefore advisable. An early diagnosis of MGRS usually enables better management of the patient and the timely initiation or adjustment of therapy, which is ultimately reflected in a higher life expectancy.

Diagnostic examinations

A basic examination, comparable to that in the case of a suspected monoclonal gammopathy of unclear significance (MGUS), should be part of the diagnosis of exclusion. In addition, histological and imaging methods are part of a more differentiated diagnosis for the exact determination of possible lesions of the kidney . This allows the B-cell clone and the deposited monoclonal protein such. B. free light chains, immunoglobulins or their fragments can be identified and characterized.

Laboratory diagnostics

histology

Imaging methods

Clinical picture

Monoclonal tumor products can be deposited in different areas of the nephron and in different ways. The resulting clinical pictures can be combined or isolated. In general, the impairment of kidney function is associated with a poor overall condition of the affected person. The average life expectancy is shorter than that of patients with monoclonal gammopathy without impaired kidney function.

Diseases with deposits of monoclonal proteins in the kidney
Type of deposit pathology
Fibrillar AL amyloidosis; non-amyloid fibrillary glomerulitis
Microtubular Type 1 and Type 2 cryoglobulinemic glomerulonephritis
Amorphous Light-Chain-Deposition-Disease (LCDD), Light / Heavy-Chain-Deposition Disease (LHCDD), Heavy-Chain Deposition Disease (HCDD)
Other Proliferative glomerulonephritis with monoclonal immunoglobulin deposits, Waldenström's disease

therapy

So far there is no prophylaxis against the deposition of monoclonal immunoglobulins. Although the underlying B-cell clone is not yet a malignant form by definition, chemotherapy is the only possible option to counteract progressive damage to the kidneys and other organs. In addition, palliative dialysis is indicated in certain cases to compensate for the loss of kidney function. A removal of free light chains in particular from the serum is possible when using a large-pored dialysis membrane. At the moment, however, the data are not sufficient to conclusively assess the benefits of this method. In any case, it must be taken into account that the cause of the loss of kidney function is not eliminated by this method.

Individual evidence

  1. ↑ a b N Leung et al .: Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant . In: Blood . 120, No. 22, November 22, 2012, pp. 4292-4295. doi : 10.1182 / blood-2012-07-445304 . PMID 23224402 .
  2. ↑ A Dispenzieri et al .: Prevalence and risk of progression of light-chain monoclonal gammopathy of undetermined significance: a retrospective population-based cohort study . In: Lancet . 375, No. 9727, May 15, 2010, pp. 1721-1728. doi : 10.1016 / S0140-6736 (10) 60482-5 . PMID 20472173 .
  3. ↑ a b N van de Donk et al .: The clinical relevance and management of monoclonal gammopathy of undetermined significance and related disorders: recommendations from the European Myeloma Network . In: Haematologica . 99, No. 6, March 21, 2014, pp. 984-96. doi : 10.3324 / haematol.2013.100552 . PMID 23224402 . PMC 4040895 (free full text).
  4. ↑ a b c JP Fermand et al .: How I treat monoclonal gammopathy of renal significance (MGRS) . In: Blood . 122, No. 22, October 9, 2013, pp. 3583-3590. doi : 10.1182 / blood-2013-05-495929 . PMID 25607108 .
  5. ^ S Sawhney et al .: Long-term prognosis after acute kidney injury (AKI): what is the role of baseline kidney function and recovery? A systematic review . In: BMJ open . 4, 2015. doi : 10.1136 / bmjopen-2014-006497 .
  6. ↑ C Hutchison et al .: Immunoglobulin free light chain levels and recovery from myeloma kidney on treatment with chemotherapy and high cut-off haemodialysis . In: Nephrol Dial Transplant . 27, No. 10, October 27, 2012, pp. 3823-3828. doi : 10.1093 / ndt / gfr773 . PMID 22273664 .
  7. ↑ 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 .
  8. ↑ a b F Bridoux et al .: Diagnosis of monoclonal gammopathy of renal significance . In: Kidney Int . 87, No. 4, February 28, 2014, pp. 698-711. doi : 10.1038 / ki.2014.408 . PMID 25607108 .
  9. ^ C Hutchison et al .: Efficient Removal of Immunoglobulin Free Light Chains by Hemodialysis for Multiple Myeloma: In Vitro and In Vivo Studies . In: J am Soc Nephrol . 18, No. 3, January 17, 2007, pp. 886-895. doi : 10.1681 / ASN.2006080821 . PMID 17229909 .
  10. ↑ Mark Cook et al .: High Cut-Off Haemodialysis (HCO-HD) Does Not Improve Outcomes In Myeloma Cast Nephropathy: Results Of European Trial Of Free Light Chain Removal By Extended Haemodialysis In Cast Nephropathy (EULITE) . Poster presentation at the EHA Congress 2016.