Skip to main content
SpringerLink
Log in

“Idiopathic Bence-Jones proteinuria”: a new characterization of an old entity

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

Idiopathic Bence-Jones proteinuria (BJP) is a rare plasma cell dyscrasia, of which the clinical and biological characteristics are yet unclear. Historical data suggested that they are at higher risk of progression to multiple myeloma or other related neoplasms, while recent findings are contradictory. To address these open questions, we evaluated a series of both BJP and monoclonal gammopathy of undetermined significance (MGUS) with production of an intact immunoglobulin plus Bence-Jones proteinuria (MGUS+BJP) with long-term follow-up, regarding their clinical characteristics and progression to multiple myeloma, amyloidosis or other related B cell lymphoproliferative disorders. Two hundred and twenty-nine persons fulfilling the 2004 criteria of MGUS were included in the final analyses: 31 had BJP and 198 had MGUS+BJP. At the time of diagnosis, significantly more persons in the BJP group had renal impairment, anaemia and polyneuropathy. A more detailed analysis revealed discrepancies between the serum and urine light chain type in nine cases, reflecting clonal heterogeneity. The number of disease progressions was higher in MGUS+BJP (n = 30) when compared to BJP (n = 1), with a rate of 1.6 and 0.4 progressions per 100 person-years, respectively. In conclusion, BJP has distinct clinical characteristics and a lower risk of progression when compared to MGUS+BJP. Our data suggest that MGUS+BJP being closer to malignant transformation may be due to the higher portion of genetically heterogeneous, pre-malignant plasma cell subclones.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Korde N, Kristinsson SY, Landgren O (2011) Monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM): novel biological insights and development of early treatment strategies. Blood 117(21):5573–5581. doi:10.1182/blood-2011-01-270140

    Article  PubMed  CAS  Google Scholar 

  2. Dispenzieri A, Katzmann JA, Kyle RA, Larson DR, Melton LJ, Colby CL, Therneau TM, Clark R, Kumar SK, Bradwell A, Fonseca R, Jelinek DF, Rajkumar SV (2010) Prevalence and risk of progression of light-chain monoclonal gammopathy of undetermined significance: a retrospective population-based cohort study. Lancet 375(9727):1721–1728. doi:10.1016/s0140-6736(10)60482-5

    Article  PubMed  Google Scholar 

  3. Bradwell AR, Carr-Smith HD, Mead GP, Tang LX, Showell PJ, Drayson MT, Drew R (2001) Highly sensitive, automated immunoassay for immunoglobulin free light chains in serum and urine. Clin Chem 47(4):673–680

    PubMed  CAS  Google Scholar 

  4. Hopkins FG (1911) A study of Bence-Jones protein, and of the metabolism in three cases of Bence-Jones proteinuria. J Physiol 42(3):189–250

    PubMed  CAS  Google Scholar 

  5. Kyle RA, Greipp PR (1982) "Idiopathic" Bence Jones proteinuria: long-term follow-up in seven patients. N Engl J Med 306(10):564–567. doi:10.1056/NEJM198203113061002

    Article  PubMed  CAS  Google Scholar 

  6. Rajkumar SV, Kyle RA, Therneau TM, Melton LJ 3rd, Bradwell AR, Clark RJ, Larson DR, Plevak MF, Dispenzieri A, Katzmann JA (2005) Serum free light chain ratio is an independent risk factor for progression in monoclonal gammopathy of undetermined significance. Blood 106(3):812–817. doi:10.1182/blood-2005-03-1038

    Article  PubMed  CAS  Google Scholar 

  7. Fiegl M, Greil R (2002) Monoclonal gammopathy of undetermined significance. N Engl J Med 346(26):2087–2088. doi:10.1056/NEJM200206273462614, author reply 2087–2088

    Article  PubMed  Google Scholar 

  8. Eisele L, Durig J, Huttmann A, Duhrsen U, Assert R, Bokhof B, Erbel R, Mann K, Jockel KH, Moebus S, Heinz Nixdorf Recall Study Investigative G (2012) Prevalence and progression of monoclonal gammopathy of undetermined significance and light-chain MGUS in Germany. Ann Hematol 91(2):243–248. doi:10.1007/s00277-011-1293-1

    Article  Google Scholar 

  9. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group (2003). Br J Haematol 121(5):749–757

  10. Kyle RA, Therneau TM, Rajkumar SV, Offord JR, Larson DR, Plevak MF, Melton LJ 3rd (2002) A long-term study of prognosis in monoclonal gammopathy of undetermined significance. N Engl J Med 346(8):564–569. doi:10.1056/NEJMoa01133202

    Article  PubMed  Google Scholar 

  11. Jaffe E, Harris N, Stein H, Vardiman J (2001) Pathology and genetics of tumors of haematopoietic and lymphoid tissues. World Health Organization (WHO) classification of tumors. World Health Organization, Lyon

    Google Scholar 

  12. Pozzi C, D'Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, Quattrocchio G, Rollino C, Segagni S, Locatelli F (2003) Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis Off J Natl Kidney Found 42(6):1154–1163

    Article  Google Scholar 

  13. Leung N, Bridoux F, Hutchison CA, Nasr SH, Cockwell P, Fermand JP, Dispenzieri A, Song KW, Kyle RA, International K, Monoclonal Gammopathy Research G (2012) Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant. Blood 120(22):4292–4295. doi:10.1182/blood-2012-07-445304

    Article  Google Scholar 

  14. Dalakas MC, Engel WK (1981) Polyneuropathy with monoclonal gammopathy: studies of 11 patients. Ann Neurol 10(1):45–52. doi:10.1002/ana.410100109

    Article  PubMed  CAS  Google Scholar 

  15. Rajabally YA (2011) Neuropathy and paraproteins: review of a complex association. Eur J Neurol Off J Eur Fed Neurol Soc 18(11):1291–1298. doi:10.1111/j.1468-1331.2011.03380.x

    CAS  Google Scholar 

Download references

Acknowledgments

The authors thank Werner Klotz for the access to the primary data. This work was supported by a grant of the “Österreichische Krebshilfe-Krebsgesellschaft Tirol”.

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Hematology and Oncology, Medical University of Innsbruck, Anichstraße 35, 6020, Innsbruck, Austria

    Michael Mian, Irene Franz, Ines Wasle, Günther Gastl, Wolfgang Willenbacher, Eberhard Gunsilius & Michael Fiegl

  2. Division of Hematology, Azienda Ospedaliera S. Maurizio, Bolzano, Italy

    Michael Mian & Sergio Cortelazzo

  3. Department of General Internal Medicine/Rheumatology, Medical University of Innsbruck, Innsbruck, Austria

    Manfred Herold

  4. Central Institute for Medical and Chemical Laboratory Diagnosis, Medical University of Innsbruck, Innsbruck, Austria

    Andrea Griesmacher & Wolfgang Prokop

Authors
  1. Michael Mian
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Irene Franz
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ines Wasle
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Manfred Herold
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Andrea Griesmacher
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Wolfgang Prokop
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Sergio Cortelazzo
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Günther Gastl
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Wolfgang Willenbacher
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Eberhard Gunsilius
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Michael Fiegl
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Michael Mian.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mian, M., Franz, I., Wasle, I. et al. “Idiopathic Bence-Jones proteinuria”: a new characterization of an old entity. Ann Hematol 92, 1263–1270 (2013). https://doi.org/10.1007/s00277-013-1739-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-013-1739-8

Keywords

Search

Navigation