| Title |
“Idiopathic Bence-Jones proteinuria”: a new characterization of an old entity
|
|---|---|
| Published in |
Annals of Hematology, April 2013
|
| DOI | 10.1007/s00277-013-1739-8 |
| Pubmed ID | |
| Authors |
Michael Mian, Irene Franz, Ines Wasle, Manfred Herold, Andrea Griesmacher, Wolfgang Prokop, Sergio Cortelazzo, Günther Gastl, Wolfgang Willenbacher, Eberhard Gunsilius, Michael Fiegl |
| 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. |
X Demographics
The data shown below were collected from the profiles of 3 X users who shared this research output. Click here to find out more about how the information was compiled.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United States | 2 | 67% |
| Unknown | 1 | 33% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 2 | 67% |
| Scientists | 1 | 33% |
Mendeley readers
The data shown below were compiled from readership statistics for 24 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United Kingdom | 1 | 4% |
| Unknown | 23 | 96% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 8 | 33% |
| Other | 2 | 8% |
| Student > Doctoral Student | 2 | 8% |
| Lecturer > Senior Lecturer | 2 | 8% |
| Student > Ph. D. Student | 2 | 8% |
| Other | 4 | 17% |
| Unknown | 4 | 17% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 12 | 50% |
| Biochemistry, Genetics and Molecular Biology | 2 | 8% |
| Agricultural and Biological Sciences | 2 | 8% |
| Nursing and Health Professions | 1 | 4% |
| Social Sciences | 1 | 4% |
| Other | 1 | 4% |
| Unknown | 5 | 21% |
Attention Score in Context
This research output has an Altmetric Attention Score of 2. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 19 July 2013.
All research outputs
#14,168,358
of 22,707,247 outputs
Outputs from Annals of Hematology
#985
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Outputs of similar age
#113,198
of 198,792 outputs
Outputs of similar age from Annals of Hematology
#14
of 21 outputs
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