Benign Bence Jones Gammopathy

Paladini, G; Sala, P.; P, Santini

doi:10.1159/000207409

Cited by 12 publications

(6 citation statements)

References 7 publications

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“…A few patients have been described with idiopathic or so-called benign Bence Jones proteinuria, but these reports are limited by inadequate follow-up. 13–17 More than 30 years ago, we described a case series of seven patients with idiopathic Bence Jones proteinuria with an M-protein urinary excretion of greater than 1·0 g/24 h. 12 …”

Section: Introductionmentioning

confidence: 99%

Clinical course of light-chain smouldering multiple myeloma (idiopathic Bence Jones proteinuria): a retrospective cohort study

Kyle

Larson

Therneau

et al. 2014

The Lancet Haematology

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Summary Background Bence Jones proteinuria is a disorder that is defined by the excretion of monoclonal light-chain protein. About 15–20% of patients with multiple myeloma secrete monoclonal light chains only, without expression of the normal immunoglobulin heavy chain, which constitutes light-chain multiple myeloma. The definition, prevalence, and progression of these premalignant phases of light-chain multiple myeloma have not been fully characterised. We aimed to identify a subset of patients with idiopathic Bence Jones proteinuria who had a high risk of progression to light-chain multiple myeloma analogous to that seen in patients with smouldering multiple myeloma. Methods In this retrospective cohort study, we studied all patients seen at the Mayo Clinic (Rochester, MN, USA) within 30 days of diagnosis of idiopathic Bence Jones proteinuria between Jan 1, 1960, and June 30, 2004. Inclusion criteria were monoclonal light chain in the urine (≥0·2 g/24 h), absence of intact monoclonal immunoglobulin (M protein) in the serum, and no evidence of multiple myeloma, light-chain amyloidosis, or other related plasma-cell proliferative disorders. The primary endpoint was progression to symptomatic multiple myeloma or light-chain amyloidosis. We examined the cumulative probability of progression and the association of potential risk factors on progression rates to identify patients with a high risk of progression to multiple myeloma or light-chain amyloidosis. Findings We identified 101 patients with idiopathic Bence Jones proteinuria. During 901 total person-years of follow-up, 27 (27%) patients developed multiple myeloma and seven (7%) developed light-chain amyloidosis. The major risk factors for progression were amount of urinary excretion of M protein per 24 h, proportion of bone marrow plasma cells, presence of a markedly abnormal free-light-chain ratio (<0·01 or >100), and reduction of all three uninvolved immunoglobulins. Based on the risk of progression, monoclonal light-chain excretion of 0·5 g/24 h or greater or at least 10% bone marrow plasma cells, or both, in the absence of end-organ damage was used to define light-chain smouldering multiple myeloma. The cumulative probability of progression to active multiple myeloma or light-chain amyloidosis in patients with light-chain smouldering multiple myeloma was 27·8% (95% CI 14·2–39·2) at 5 years, 44·6% (27·9–57·4) at 10 years, and 56·5% (36·3–70·2) at 15 years. Interpretation Light-chain smouldering multiple myeloma as defined in this study is associated with a high risk of progression to symptomatic light-chain multiple myeloma, and this subset of patients needs careful observation and could benefit from clinical trials of early intervention. Funding Jabbs Foundation (Birmingham, UK), US National Cancer Institute, and Henry J Predolin Foundation (Madison, WI, USA).

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Section: Introductionmentioning

confidence: 99%

Clinical course of light-chain smouldering multiple myeloma (idiopathic Bence Jones proteinuria): a retrospective cohort study

Kyle

Larson

Therneau

et al. 2014

The Lancet Haematology

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“…These cases are certainly worth monitoring, because, even if rarely, and perhaps after many years, these processes may transform into malignant ones (1,4). If such a change occurs, the excreted amount of BJ proteins, nonvarying for years, may rocket, and this may be the first sign of the malignant transformation of the disease.…”

Section: Discussionmentioning

confidence: 99%

“…In addition to accumulated monoclonal immunoglobulins in the serum (component M), BJ proteins excreted in the urine is an important indicator of multiple myeloma. However, in varying concentrations, they can also be detected in other B cell tumors, lymphoproliferative diseases, and even in some cases of benign monoclonal gammopathy (1)(2)(3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

Monoclonal free light chains in urine and their significance in clinical diagnostics: Are they really tumor markers?

Végh

Ottó

Eckhardt

1990

Clinical Laboratory Analysis

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Bence Jones proteins (monoclonal free light chains of immunoglobulins) are the earliest known biological markers of malignant cell dyscrasia; Bence Jones proteinuria is also present in many types of B cell-related neoplasms. Sometimes, it may also occur in Hodgkin's disease. In some cases, benign monoclonal gammapathy was found to be associated nontumorous diseases as well. The type of monoclonal light chain, the degree of polymerization, and the isoelectric point of the molecule may affect the course of the disease. Urine samples from 637 patients with true or suspected lymphoproliferative diseases were investigated over a 2-yr period by different immunochemical methods. Bence Jones proteinuria was identified in 71 cases by isoelectric focusing combined with immunofixation, while the pathological protein was detected only in 63 cases by conventional methods. Bence Jones proteins can be detected by this new method at a level below the sensitivity of conventional procedures. Bence Jones proteins in the urine may signal a malignant tumor or malignant transformation of an earlier disease. The early detection of monoclonal immunoglobulin light chains in the urine may be important in clinical diagnosis, therapy, and follow-up.

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“…Qualitative and quantitative studies of serum im munoglobulins and urinary light chains were per formed using methods described previously [9].…”

Section: Methodsmentioning

confidence: 99%

Indomethacin-Induced Hyperkalemia and Renal Failure in Multiple Myeloma

Paladini¹,

Tonazzi²

1982

Acta Haematol

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3 patients with multiple myeloma developed hyperkalemic hypoaldosteronism and renal insufficiency while receiving indomethacin for bone pain. Claims are made that indomethacin has been responsible for these complications, possibly by inhibiting prostaglandin biosynthesis. Careful observation of myeloma patients during administration of this agent and perhaps other prostaglandin inhibitors appears warranted.

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Benign Bence Jones Gammopathy

Cited by 12 publications

References 7 publications

Clinical course of light-chain smouldering multiple myeloma (idiopathic Bence Jones proteinuria): a retrospective cohort study

Clinical course of light-chain smouldering multiple myeloma (idiopathic Bence Jones proteinuria): a retrospective cohort study

Monoclonal free light chains in urine and their significance in clinical diagnostics: Are they really tumor markers?

Indomethacin-Induced Hyperkalemia and Renal Failure in Multiple Myeloma

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