3,712 results on '"Bence Jones protein"'
Search Results
2. Inaccurate definition of Bence Jones proteinuria in the EFLM Urinalysis Guideline 2023.
- Author
-
Mussap, Michele, Dolci, Alberto, and Graziani, Maria Stella
- Subjects
- *
IMMUNOGLOBULIN light chains , *GTPASE-activating protein , *IMMUNOGLOBULINS , *ALBUMINURIA , *URINALYSIS - Abstract
The letter addresses concerns regarding the inaccurate definition of Bence Jones proteinuria (BJP) in the EFLM European Urinalysis Guideline 2023. The authors argue that the guideline's terminology does not accurately reflect the characteristics of BJP, which is defined as monoclonal free light chains of immunoglobulins. They caution against using total proteinuria as a screening method for BJP, as it may not be sensitive enough to detect clinically significant concentrations. The authors emphasize the importance of using strict and accurate definitions in guidelines and recommend urine immunofixation as the gold standard test for detecting BJP. [Extracted from the article]
- Published
- 2024
- Full Text
- View/download PDF
3. A new challenge for urinary free light chains: assessment of the upper reference limit in healthy subjects.
- Author
-
NATALI, P., DEBBIA, D., CUCINELLI, M. R., NASILLO, V., RIVA, G., CIGLIANA, G., CARNAZZO, V., TRENTI, T., MARINO, M., and BASILE, U.
- Abstract
OBJECTIVE: Free light chains (FLCs) can be measured in both urine (uFLC) and serum (sFLC) in immunochemistry. We aim to compare FLC levels in serum and urine assessed among healthy volunteers and measured upper reference limits (URLs) of urinary FLC to creatinine ratio (uFLC/uCr) in mg/g to compare with the manufacturer's recommended URLs. PATIENTS AND METHODS: Eligibility criteria: normal serum and urine FLC measure and negative serum/urinary immunofixation. Immunoturbidimetry was used to assess both κ and λ FLCs. The URLs were calculated with the 97.5th percentile of uFLC concentrations according to the Clinical and Laboratory Standards Institute recommendations. RESULTS: 126 healthy subjects (median age 46 years, 62% females) met the inclusion criteria. Median concentrations of κ and λ sFLCs were similar both for males and females without significant differences. κ and λ uFLCs were significantly higher in males than in females (p < 0.001 and p = 0.004, respectively). Slower clearance for λ FLC compared to λ FLC was observed with an increased λ/λ uFLC ratio in both males and females. URLs for male and female subjects: λ uFLC mg/g uCr = 34.35 vs. 23.18, and λ uFLC mg/g uCr = 3.59 vs. 1.96, respectively compared well with manufacturer's URLs. CONCLUSIONS: FLC catabolism is gender-dependent and occurs less rapidly in λ FLC than in λ FLC. The determination of the URL of uFLC, as uFLC/uCr, in healthy subjects in morning urine, proved to be consistent with the manufacturer's recommendations, but with a significant difference according to gender. [ABSTRACT FROM AUTHOR]
- Published
- 2023
4. The dark side of current analytic methods for Bence Jones Proteinuria.
- Author
-
NATALI, P., CIGLIANA, G., NAPODANO, C., BASILE, V., DEBBIA, D., POCINO, K., SAVOIA, M., MARINO, M., GULLI, F., and BASILE, U.
- Abstract
OBJECTIVE: Bence Jones proteinuria (BJP) refers to monoclonal free immunoglobulin light chains detected in urine, deriving from the clonal expansion of plasma cells in the bone marrow in patients with plasma cell dyscrasias, associated with monoclonal gammopathies of uncertain origin. This review summarizes routinely diagnostic procedures to assess BJP highlighting critical steps of pre-analytical, analytical, and post-analytical phases. QUALITATIVE AND QUANTITATIVE METHODS: The best option for BJP detection is the first morning void urine sample and immunofixation electrophoresis detection technique (IFE) the recommended method, with the employment of specific polyvalent antisera. Other qualitative tests for a quick evaluation of BJP are currently available. Densitometric analysis performed on the 24-hour urine is the recommended method to quantify BJP. To overcome the 24-hour collection, it is possible to use morning urine sample and correlate the assessed value of BJP to creatininuria. In addition to the traditional ones, we here reviewed screening methods currently used to avoid false negatives and reduce the time around test (TAT), together with immunochemical quantification methods for increased sensitivity, after checking BJP by IFE. Mass spectrometry emerges as a new challenge in the determination of BJP. CONCLUSIONS: The employment of different based-assays methods may be useful for diagnostic purposes to improve the accuracy of BJP monitoring in monoclonal gammopathies. [ABSTRACT FROM AUTHOR]
- Published
- 2022
5. (F)utility of urine Bence Jones proteins for "routine" screening for plasma cell dyscrasia.
- Author
-
Parmar, Malvinder S.
- Subjects
- *
PLASMA cells , *MULTIPLE myeloma , *PLASMA cell diseases , *URINE , *PROTEINS - Abstract
Testing urine for Bence Jones Protein (BJP) had been a time old procedure used for screening and monitoring of monoclonal disorders since its description. However, has poor sensitivity and despite advances in diagnostic methods of monoclonal disorders it is being continued to be requested in individuals for evaluation of myeloma or plasma cell disorders. Effective utilization and minimizing untimely or unnecessary investigations is important in the evaluation and management of any medical condition. Though, we are hard-wired during our education with some "trigger" or "peculiar" words that make us jump to actions too quickly, without comprehending the actual problem. Supporting evidence is presented to avoid reflexive use of multiple tests and utilize tests that improve utilization, reduce waste, and uphold the Choosing Wisely principles in providing optimal care to the patients. [ABSTRACT FROM AUTHOR]
- Published
- 2021
- Full Text
- View/download PDF
6. Comparison of 24-hour versus random urine samples for determination and quantification of Bence Jones protein in a South African population
- Author
-
Ashandree Reddy, Nadine Rapiti, and Verena Gounden
- Subjects
multiple myeloma ,bence jones protein ,random urine ,estimated 24-h bence jones protein ,Public aspects of medicine ,RA1-1270 ,Medicine (General) ,R5-920 - Abstract
Background: The International Myeloma Working Group and College of American Pathologists recommend a 24-h urine collection to determine the Bence Jones protein (BJP) excretion level for monitoring treatment response in patients with multiple myeloma (MM). There are several issues related to sample collection and the method is prone to inaccuracy. Objective: This study compared measured 24-h to random urine collections for the quantitation of BJP in a South African population. Methods: Sixty-six patients with MM submitted random urine samples with their routine 24-h urine collection from April 2016 – March 2018. Measured 24-h urine BJP was compared to two estimated 24-h BJP excretions calculated as follows: Estimation 1 (E1): Estimated 24-h BJP (mg/24 h) = Urine BJP/Creatinine ratio (mg/mmol) × 10. Estimation 2 (E2): Estimated 24-h BJP (mg/24 h) = Urine BJP/Creatinine ratio (mg/mmol) × 15 mg/kg for women or × 20 mg/kg for men. Results: Correlation of estimation equations E1 and E2 to the measured 24-h urine BJP was 0.893. Patients showed no difference in classification of treatment response using either the E1 or E2 estimation equations when compared to the measured 24-h urine BJP results. Conclusion: This study demonstrates that the estimated 24-h BJP shows a high degree of correlation with the measured 24-h BJP and can likely be used to monitor treatment response in South African patients with MM.
- Published
- 2021
- Full Text
- View/download PDF
7. Elements Involved in the Physical Evaluation of Urine
- Author
-
Ridley, John W. and Ridley, John W.
- Published
- 2018
- Full Text
- View/download PDF
8. Comparison of 24-hour versus random urine samples for determination and quantification of Bence Jones protein in a South African population.
- Author
-
Reddy, Ashandree, Rapiti, Nadine, and Gounden, Verena
- Subjects
- *
SOUTH Africans , *STATISTICAL sampling , *MULTIPLE myeloma , *RANDOM measures , *URINE - Abstract
Background: The International Myeloma Working Group and College of American Pathologists recommend a 24-h urine collection to determine the Bence Jones protein (BJP) excretion level for monitoring treatment response in patients with multiple myeloma (MM). There are several issues related to sample collection and the method is prone to inaccuracy. Objective: This study compared measured 24-h to random urine collections for the quantitation of BJP in a South African population. Methods: Sixty-six patients with MM submitted random urine samples with their routine 24-h urine collection from April 2016 – March 2018. Measured 24-h urine BJP was compared to two estimated 24-h BJP excretions calculated as follows: Estimation 1 (E1): Estimated 24-h BJP (mg/24 h) = Urine BJP/Creatinine ratio (mg/mmol) × 10. Estimation 2 (E2): Estimated 24-h BJP (mg/24 h) = Urine BJP/Creatinine ratio (mg/mmol) × 15 mg/kg for women or × 20 mg/kg for men. Results: Correlation of estimation equations E1 and E2 to the measured 24-h urine BJP was 0.893. Patients showed no difference in classification of treatment response using either the E1 or E2 estimation equations when compared to the measured 24-h urine BJP results. Conclusion: This study demonstrates that the estimated 24-h BJP shows a high degree of correlation with the measured 24-h BJP and can likely be used to monitor treatment response in South African patients with MM. [ABSTRACT FROM AUTHOR]
- Published
- 2021
- Full Text
- View/download PDF
9. Glycosylated Bence Jones Protein with Poor Thermal Reactivity in Heat Coagulation Tests.
- Author
-
Mayumi Imoto, Katsunori Watanabe, Koji Yoshida, Ken-ichi Nakae, Toshinori Kamisako, and Toshiyuki Yamada
- Subjects
BLOOD coagulation ,HEAT ,MULTIPLE myeloma ,URINALYSIS ,PROTEINS - Abstract
Background: We experienced a patient with multiple myeloma whose urine contained a considerable amount of Bence Jones protein (BJP), which demonstrated poor thermal reactivity in heat coagulation test. The mechanism for this phenomenon was assessed. Methods: Immunoelectrophoretic analyses reveal that a band corresponding to BJP in the urine had 2,600 Dalton by reduction after glycosidase treatment, but not after sialidase treatment. In addition, the glycosidase-treated urine tested positive in heat coagulation test. Conclusions: Glycosylation of the immunoglobulin light chain, which has rarely been seen, is the cause of the unexpected behavior of this patent's BJP in heat coagulation tests. [ABSTRACT FROM AUTHOR]
- Published
- 2020
- Full Text
- View/download PDF
10. Bence Jones KWR protein structures determined by X‐ray crystallography
- Author
-
Makino, Debora L, Henschen‐Edman, Agnes H, Larson, Steven B, and McPherson, Alexander
- Subjects
Inorganic Chemistry ,Biochemistry and Cell Biology ,Chemical Sciences ,Biological Sciences ,Amino Acid Sequence ,Amyloid ,Bence Jones Protein ,Crystallization ,Crystallography ,X-Ray ,Humans ,Hydrogen Bonding ,Hydrogen-Ion Concentration ,Models ,Molecular ,Protein Conformation ,Physical Sciences ,Biophysics ,Biological sciences ,Chemical sciences ,Physical sciences - Abstract
A Bence Jones protein isolated in the early 1960s from a patient (initials KWR) suffering from plasma-cell dyscrasia was crystallized and its structure was analyzed in four different unit cells by X-ray diffraction. The final models of the molecule in all crystal forms were virtually the same, although the elbow angles relating the constant and variable domains of the Bence Jones dimers varied over a range of 10 degrees. The tetragonal form had an R factor of 22.6% and an R(free) of 28.3% at 2.2 A resolution. Phosphate or sulfate ions (depending on the crystallization conditions) were found in the antigen-combining sites in all crystals, as well as an unidentified ligand tightly bound in the hydrophobic 'deep pocket' beneath the antigen-binding site. The ligand was treated as a phenol molecule. Two trigonal crystal forms were among those solved. One was grown at pH 4.0 and the other was only obtained after sitting for more than eight months at room temperature. The latter crystal was composed of molecules that were degraded in their constant domains. Both low pH and proteolytic degradation of constant domains are known to promote the polymerization of some Bence Jones proteins into amyloid fibrils. Indeed, in both trigonal crystal forms the molecules are organized with pseudo-hexagonal symmetry about the unique crystallographic axes in a manner suggestive of such fibrils. The arrangement of Bence Jones dimers is also consistent with other observations regarding Bence Jones amyloid-fibril structure and current models.
- Published
- 2007
11. Four crystal forms of a Bence‐Jones protein
- Author
-
Makino, Debora L, Henschen‐Edman, Agnes H, and McPherson, Alexander
- Subjects
Adult ,Bence Jones Protein ,Crystallization ,Freeze Drying ,Humans ,Middle Aged ,Multiple Myeloma ,Protein Conformation ,X-Ray Diffraction ,Chemical Sciences ,Biological Sciences ,Biophysics - Abstract
Four crystal forms have been grown and characterized by X-ray diffraction of a Bence-Jones protein collected from the urine of a multiple myeloma patient more than 40 years ago. Closely related tetragonal and orthorhombic forms belonging to space groups P4(3)2(1)2 and P2(1)2(1)2(1), with unit-cell parameters a = b = 68.7, c = 182.1 and a = 67.7, b = 69.4, c = 87.3 A, diffract to 1.5 and 1.9 A, respectively. Two closely related trigonal forms, both belonging to space group P3(1)21 with unit-cell parameters a = b = 154.3 A but differing by a doubling of the c axis, one 46.9 A and the other 94.0 A, diffract to 2.9 and 2.6 A resolution, respectively. The trigonal crystal of short c-axis length shows a positive indication of twinning. The trigonal crystal of longer c axis, which appeared only after eight months of incubation at room temperature, is likely to be composed of proteolytically degraded molecules and unlike the other crystal forms contains two entire Bence-Jones dimers in the asymmetric unit. This latter crystal form may shed some light on the formation of fibrils common to certain storage diseases.
- Published
- 2005
12. Multiple Myeloma Presenting With Bronchopneumonia: A Case Report.
- Author
-
Karnan A, Ghewade B, Alone VD, and Ledwani A
- Abstract
Multiple myeloma is a disease of the plasma cells of the bone marrow, resulting in the proliferation and release of the monoclonal protein, which further causes end-organ damage. We report an unusual presentation of multiple myeloma, thereby insisting on the need for the treating physician to be aware of the various presentations that can be encountered in regular practice. It is often difficult to diagnose, and the diagnosis is usually made at a late stage of the disease. Even though uncurable, with recent advances, a proper regimen, newer chemotherapeutic agents, and stem cell transplantation, the disease can be brought into remission., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Karnan et al.)
- Published
- 2024
- Full Text
- View/download PDF
13. Myeloma and the Kidney
- Author
-
Findlay, Mark, Isles, Christopher, Findlay, Mark, and Isles, Christopher
- Published
- 2015
- Full Text
- View/download PDF
14. Light-chain plasma cell myeloma caused by 14q32/IGH translocation and loss of the other allele.
- Author
-
Nishio, Yuji, Sakai, Hirotaka, Saiki, Yusuke, Uchida, Akiko, Uemura, Yu, Matsunawa, Manabu, Isobe, Yasushi, Kato, Masayuki, Tomita, Naoto, and Miura, Ikuo
- Abstract
Light-chain plasma cell myeloma (LC-PCM) is a PCM subtype in which only immunoglobulin light-chain is secreted. However, the absence of immunoglobulin heavy-chain (IGH) production in this condition has not been fully elucidated. To address this issue, we retrospectively analyzed patients at our center with LC-PCM and found a group who had only split signals of IGH gene derived from 14q32/IGH translocations by fluorescence in situ hybridization (FISH). Six patients were identified with only split signals of the IGH gene derived from 14q32/IGH translocations. Five of these patients were newly diagnosed, while one had IgG-λ PCM at presentation, which transformed to λ LC-PCM after treatment. The translocation partners were identified in four patients: two cases of (11;14)(q13;q32) and two cases of (4;14)(p16;q32). The development of LC-PCM appears to be explained by the application of allelic exclusion in these patients, such that 14q32/IGH translocation in one allele contributes to the pathogenesis of PCM and the subsequent loss of the other allele is responsible for the loss of IGH production. These findings suggest that a FISH pattern of IGH with "split and loss" may constitute a unique subgroup of LC-PCM. [ABSTRACT FROM AUTHOR]
- Published
- 2019
- Full Text
- View/download PDF
15. BENS DŽONSOV PROTEIN - PRVI TUMORSKI MARKER U ISTORIJI MEDICINE.
- Author
-
Govedarović, Nenad
- Subjects
- *
PHARMACEUTICAL chemistry , *MYELOMA proteins , *MULTIPLE myeloma , *TUMOR markers , *HISTORY of medicine - Abstract
Bence Jones protein is generally accepted term for protein described in urine of myeloma patients. Today, qualitative, as well as quantitative determination of Bence Jones protein serve as routine analyses for diagnosis and screening of myeloma and skeletal affections. Although Bence Jones is not the first to recognize the characteristics of the urine of the diseased, his merit is that he recognized the importance of this protein in patients with myeloma. He is considered as the pioneer in medical chemistry and is one of the first doctors who emphasized the importance of chemical analysis for the diagnosis of the disease. His discovery of proteinuria in multiple myeloma has long been the only biochemical test for cancer until the seventies of the twentieth century, with the discovery of carcinoembrion antigen (CEA) and alpha-fetoprotein (alpha-FP) It can rightly be said that a protein named after him is the first tumor marker in the history of medicine. [ABSTRACT FROM AUTHOR]
- Published
- 2019
- Full Text
- View/download PDF
16. Emergence of proteinase 3-antineutrophil cytoplasmic antibody-associated glomerulonephritis with mesangial immune deposition during the clinical course of IgG λ monoclonal gammopathy of uncertain significance
- Author
-
Masaki Ueno, Sawako Kobayashi, Shinichiro Asakawa, Shigeyuki Arai, Michito Nagura, Osamu Yamazaki, Yoshifuru Tamura, Ryuji Ohashi, Shigeru Shibata, and Yoshihide Fujigaki
- Subjects
Male ,Glomerulonephritis ,Myeloblastin ,Immunoglobulin G ,Paraproteinemias ,Humans ,Antibodies, Monoclonal ,Case Report ,General Medicine ,Monoclonal Gammopathy of Undetermined Significance ,Aged ,Antibodies, Antineutrophil Cytoplasmic ,Bence Jones Protein - Abstract
Patients with monoclonal gammopathy of uncertain significance (MGUS) is sometimes associated with renal diseases, usually due to the deposition of secreted monoclonal immunoglobulin or a fragment thereof, a condition which is defined as monoclonal gammopathy of renal significance. Patients with MGUS appear to be at increased risk for various autoimmune conditions. We report the case of a 68-year-old man developed nephritic syndrome and mild renal insufficiency during the course of IgG λ MGUS. Laboratory findings showed hypocomplementemia, cryoglobulinemia, proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) positivity and monoclonal proteins (λ light chain and λ-Bence-Jones protein) in the urine. A kidney biopsy revealed crescentic glomerulonephritis with mesangial immune deposits without paraproteins. Treatment with prednisolone for ANCA-associated glomerulonephritis, normalized urinalysis and decreased PR3-ANCA but MGUS persisted. This is a rare case of PR3-ANCA-associated glomerulonephritis with comorbid IgG λ MGUS with various pathological paraproteins. We highlight it as a clinical example with diagnostic and therapeutic implications.
- Published
- 2022
- Full Text
- View/download PDF
17. Myeloma Cast Nephropathy
- Author
-
Leung, Nelson, Gertz, Morie A., editor, and Rajkumar, S. Vincent, editor
- Published
- 2014
- Full Text
- View/download PDF
18. Is this a true lambda free light chain?
- Author
-
Volovik, Yael, Nimri-Ammouri, Suheir, Adler, Tanya, Barak, Mira, and Henig, Clara
- Subjects
- *
IMMUNOGLOBULIN light chains , *IMMUNOGLOBULIN M , *MONOCLONAL antibodies , *BLOOD protein electrophoresis , *PROTEOMICS , *IMMUNE serums - Abstract
Nevertheless, in the remaining four samples (summarized in Table 1) the -FLC band was still present shifted to a new migration point (indicated by a black arrow) that was different from the intact IgM- immunoglobulin band location, on the sIF gel (Figure 1, lanes 7-12). In order to further evaluate this phenomenon we performed additional specific sIF tests on previously typed as IgM- positive routine samples (with total IgM 1.46-49.33 g/L, M. protein<30.8 g/L, -FLC 9.25-1145 mg/L), using a FLC antisera. The quantitative -FLC values determined were far below the minimal detection limit of the immunofixation gel (120 mg/L for the Electrophoresis protein - (ELP) lane and 50 mg/L for the -free lane) [[3]], thus the -FLC band was supposed not to be visible on the sIF. [Extracted from the article]
- Published
- 2023
- Full Text
- View/download PDF
19. History of Multiple Myeloma
- Author
-
Steensma, David P., Kyle, Robert A., Wiernik, Peter H., editor, Goldman, John M., editor, Dutcher, Janice P., editor, and Kyle, Robert A., editor
- Published
- 2013
- Full Text
- View/download PDF
20. Biochemical profile of multiple myeloma about 50 cases
- Author
-
ELGHOUAT Ghita, NAKHLI Raja, RAISSI Abderrahim, CHELLAK Saliha, and BOUKHIRA Abderrahim
- Subjects
Multiple myeloma ,electrophoresis ,monoclonal peak ,Ig G ,light chains ,Bence Jones protein - Abstract
Multiple myeloma (MM) is a clonal proliferation of plasma cells invading the bone marrow and secreting monoclonal immunoglobulin. In order to study the epidemiological and biological and biochemical characteristics of MM, we carried out a retrospective work on a cohort of 50 cases collected at the Avicenna Military Hospital in Marrakesh, during a period of 5 years (from January 2013 to December 2017). Our study included 32 men (64%) and 18 women (36%), with an average age of 60.6 years, with extremes at 44 and 87 years. The circumstances of discovery were dominated by bone pain and alteration in general condition, which are revealing in more than 65% of cases. Biologically: the sedimentation rate was accelerated in 86% of cases, a monoclonal peak appearance was revealed on serum proteins electrophoresis in 88%of cases, most often located in the γ zone (64%), a predominance of the Ig G isotype (64%), and kappa light chains in 60% of cases, Bence Jones protein (BJP) was found in 7 patients, i.e. 14% of cases, and plasmacytosis over 10% was found on the myelograms in 90 % of cases.
- Published
- 2021
- Full Text
- View/download PDF
21. History of Multiple Myeloma
- Author
-
Kyle, Robert A., Steensma, David P., Moehler, Thomas, editor, and Goldschmidt, Hartmut, editor
- Published
- 2011
- Full Text
- View/download PDF
22. Biochemical profile of Bence-Jones type multiple myeloma
- Author
-
Nina Vasilyevna Lyubimova, Yu. S. Timofeev, V. M. Abaev, O. M. Votyakova, E. A. Osmanov, and N. E. Kushlinskii
- Subjects
Medical Laboratory Technology ,C-Reactive Protein ,Immunoglobulin lambda-Chains ,Immunoglobulin M ,Immunoglobulin G ,Biochemistry (medical) ,Paraproteinemias ,Humans ,Antibodies, Monoclonal ,General Medicine ,Multiple Myeloma ,Bence Jones Protein ,Immunoglobulin A - Abstract
Multiple myeloma (MM) is a malignant tumor occurring from plasma cells that produce an abnormal monoclonal immunoglobulin - a paraprotein. A distinctive feature of Bence-Jones myeloma is the excretion of monoclonal free light chains of immunoglobulins with 24h urine, and the absence of monoclonal intact immunoglobulins secretion. Comprehensive analysis of biochemical parameters in blood serum and 24h urine in patients with Bence-Jones multiple myeloma using electrophoretic and immunoturbidimetric methods to assess their sensitivity as biomarkers. 50 patients with a morphologically confirmed diagnosis of MM of the Bence-Jones immunochemical type were examined. 28 people without oncological diseases were examinedas a control. Detection of monoclonal secretion in blood serum and daily urine was performed by immunofixation electrophoresis on the Hydrasys 2 electrophoretic system (Sebia). The determination of free light chains of immunoglobulins (FLC) was performed by the immunoturbidimetric method (Binding Site) on an Advia 1800 analyzer (Siemens). Analysis of IgG, IgA, IgM, β2-microglobulin and C-reactive protein was performed on Cobas 6000 analyzer (Roche). The median excretion of Bence-Jones protein in 24h urine of MM patients was 0.49 g/24h (0.06-2.45 g/24h). In the blood serum, in 86% of cases, the presence of paraproteinemia, represented by κ and λ type light chains of immunogloublins was detected. At the same time, the frequency of detection of monoclonal secretion in blood serum in Bence-Jones type λ myeloma was 95.7%, which was statistically significantly higher than the frequency of detection of monoclonal secretion of type κ - 77.8%. In patients with identified paraproteinemia, Bence-Jones protein excretion in daily urine (median 0.82 g/day) was statistically significantly higher than in patients without a monoclonal component detected in blood serum (median 0.04 g/24h). The levels of FLC in blood serum obtained by immunoturbidimetry in Bence-Jones myeloma of the corresponding type were higher than the reference levels in 100% of cases. The median level of κ-FLC reached 4358 mg/l, λ-FLC - 2225 mg/l, which was statistically significantly higher than the control levels. The median concentrations of IgG, IgA and IgM in patients with Bence-Jones myeloma were statistically significantly lower than in the control group, while the medians of β2-microglobulin and C-reactive protein were significantly higher than in the control. Our investigation showed high diagnostic efficiency of electrophoretic and immunoturbidimetric analysis of monoclonal secretion in patients with Bence-Jones MM, while FLC analysis demonstrated maximum sensitivity. Bence-Jones MM revealed biochemical signs of secondary immunodeficiency and general inflammatory syndrome.
- Published
- 2022
23. The dark side of current analytic methods for Bence Jones Proteinuria
- Author
-
Natali, P, Cigliana, G, Napodano, Cecilia, Basile, V, Debbia, D, Pocino, Krizia, Savoia, M, Marino, Mariapaola, Gulli, F, Basile, Umberto, Napodano, C (ORCID:0000-0002-8720-6284), Pocino, K (ORCID:0000-0003-2456-5308), Marino, M (ORCID:0000-0001-9155-6378), Basile, U, Natali, P, Cigliana, G, Napodano, Cecilia, Basile, V, Debbia, D, Pocino, Krizia, Savoia, M, Marino, Mariapaola, Gulli, F, Basile, Umberto, Napodano, C (ORCID:0000-0002-8720-6284), Pocino, K (ORCID:0000-0003-2456-5308), Marino, M (ORCID:0000-0001-9155-6378), and Basile, U
- Abstract
OBJECTIVE: Bence Jones proteinuria (BJP) refers to monoclonal free immunoglobulin light chains detected in urine, deriving from the clonal expansion of plasma cells in the bone marrow in patients with plasma cell dyscrasias, associated with monoclonal gammopathies of uncertain origin. This review summarizes routinely diagnostic procedures to assess BJP highlighting critical steps of preanalytical, analytical, and post-analytical phases.QUALITATIVE AND QUANTITATIVE METH-ODS: The best option for BJP detection is the first morning void urine sample and immunofixation electrophoresis detection technique (IFE) the recommended method, with the employment of specific polyvalent antisera. Other qualitative tests for a quick evaluation of BJP are currently available. Densitometric analysis per-formed on the 24-hour urine is the recommended method to quantify BJP. To overcome the 24-hour collection, it is possible to use morning urine sample and correlate the assessed value of BJP to creatininuria. In addition to the traditional ones, we here reviewed screening methods currently used to avoid false negatives and reduce the time around test (TAT), together with immunochemical quantification methods for in-creased sensitivity, after checking BJP by IFE. Mass spectrometry emerges as a new challenge in the determination of BJP.CONCLUSIONS: The employment of different based-assays methods may be useful for diagnostic purposes to improve the accuracy of BJP monitoring in monoclonal gammopathies.
- Published
- 2022
24. AL amyloidosis presenting as inflammatory polyarthritis: a case report
- Author
-
Nahiduzzamane Shazzad, Mohammad Mamun Khan, Johannes J. Rasker, Syed Atiqul Haq, Mohammed Kamal, Muhammad Shoaib Momen Majumder, Sohrab Alam, Shamim Ahmed, and Psychology, Health & Technology
- Subjects
030203 arthritis & rheumatology ,musculoskeletal diseases ,Pathology ,medicine.medical_specialty ,Amyloid ,medicine.diagnostic_test ,business.industry ,Amyloidosis ,UT-Hybrid-D ,medicine.disease ,Bence Jones protein ,03 medical and health sciences ,0302 clinical medicine ,Biopsy ,medicine ,Macroglossia ,AL amyloidosis ,Polyarthritis ,030212 general & internal medicine ,medicine.symptom ,business ,Subclinical infection - Abstract
Amyloidosis is a condition characterised by extracellular tissue deposition of fibrils causing a wide range of clinical manifestations. This protein deposition can occur in any tissue, most commonly in the kidney, heart, skin, peripheral nervous system, and gastrointestinal tract. However, the deposition of amyloid fibrils in the synovium is seldom reported. Musculoskeletal manifestations are subtle, subclinical and rarely the patient presents with symptoms that resemble rheumatic diseases. Here, we describe a 55-year-old man with AL (amyloid light chain) amyloidosis who presented with inflammatory polyarthritis with significant morning stiffness, inflammatory low back pain, and painful thickened tongue. The patient had anaemia, macroglossia with lateral scalloping of the tongue, papules, and plaques in the periocular, perioral and perinasal area, bilateral carpal tunnel syndrome, localised soft tissue swelling over the joints, restricted movement in different joints with flexion contractures in some joints. Rheumatoid factor and ACPA were negative and the X-ray of the sacroiliac joints was normal. We confirmed amyloidosis by biopsy of an affected skin lesion. In the urine, no Bence Jones protein was found and bone marrow study and x-ray of the skull were normal. Plasma immuno-electrophoresis and serum free light chain (FLC) assay confirmed lambda light chain type monoclonal gammopathy. Take home message: Although AL amyloidosis is a rare condition, it should be considered while evaluating atypical symptoms in patients presenting with rheumatic complaints. A high index of suspicion is necessary for proper diagnosis as delay in diagnosis will yield a poorer treatment outcome.
- Published
- 2021
- Full Text
- View/download PDF
25. Light chain myeloma and detection of free light chains in serum and urine of dogs and cats
- Author
-
Dillon Donaghy, Robert Adam Harris, A Russell Moore, Todd Shockey, Laura V. Ashton, Catherine Langston, and Matthew C. Miller
- Subjects
Immunofixation ,Colorado ,Urine ,Standard Article ,Cat Diseases ,Dogs ,Gammopathy ,hemic and lymphatic diseases ,Medicine ,Animals ,Dog Diseases ,Retrospective Studies ,Proteinuria ,CATS ,Chromatography ,lcsh:Veterinary medicine ,General Veterinary ,biology ,Bence‐Jones proteins ,business.industry ,Gel electrophoresis of proteins ,Bence Jones protein ,Standard Articles ,Oncology ,electrophoresis ,Agarose gel electrophoresis ,biology.protein ,Cats ,gammopathy ,lcsh:SF600-1100 ,SMALL ANIMAL ,medicine.symptom ,proteinuria ,business ,Multiple Myeloma ,immunoglobulin - Abstract
Background Detection of free light chains (fLC) in animals relies on protein electrophoresis or the Bence-Jones protein test on urine. Objective To describe the detection of both serum fLC (sfLC) and urine fLC (ufLC) in 8 dogs and 2 cats using a commercially available human immunofixation (IF) kit. Animals Archived serum or urine samples from 27 dogs and 2 cats submitted to the Colorado State University Veterinary Diagnostic Laboratory for routine diagnostics. Methods Retrospective study evaluating the presence of fLC in dogs and cats using agarose gel electrophoresis and routine and fLC IF performed on serum and urine. The performance of the fLC IF reagents was evaluated using samples characterized by routine IF, tandem mass spectrometry, and a combination of fLC IF and western blotting. Free light chains were documented by paired electrophoresis and fLC IF. Results The fLC only myeloma case developed end-stage renal failure 5 months post initial diagnosis. All electrophoresis-defined urinary Bence-Jones proteins were labeled by the anti-free λ light chain (anti-fλ) reagent; none were labeled by the anti-free κ light chain (anti-fκ); 2 of these were identified as fκ by mass spectrometry. An electrophoretically identical protein restriction that was labeled by the anti-fλ reagent was present in the paired serum from 5/8 of cases, documenting sfLC. Conclusions and clinical importance Commercially available human IF reagents identified sfLC and ufLC in both dogs and cats. Free light chains may be nephrotoxic in dogs.
- Published
- 2021
26. Monoclonal gammopathy of renal significance (MGRS)-related AL amyloidosis complicated by amyloid myopathy: a case report
- Author
-
Naoya Kondo, Natsuko Koita, Shuichiro Endo, Erina Ono, Takeshi Matsubara, Shunsuke Takayanagi, Ryosuke Takahashi, Katsuya Tanigaki, Ichizo Nishino, Takashi Ayaki, Motoko Yanagita, Hideki Yokoi, Kaoru Sakai, Yoshiaki Higashi, Akira Ishii, and Sachiko Minamiguchi
- Subjects
Male ,medicine.medical_specialty ,Pathology ,Amyloid ,Case Report ,Monoclonal gammopathy of undetermined significance ,lcsh:RC870-923 ,03 medical and health sciences ,0302 clinical medicine ,Muscular Diseases ,Internal medicine ,medicine ,AL amyloidosis ,Humans ,Serum amyloid A ,Aged, 80 and over ,Muscle biopsy ,Amyloid myopathy ,medicine.diagnostic_test ,business.industry ,Amyloidosis ,medicine.disease ,lcsh:Diseases of the genitourinary system. Urology ,Bence Jones protein ,Nephrology ,030220 oncology & carcinogenesis ,Monoclonal gammopathy of renal significance ,Sporadic late-onset nemaline myopathy ,Kidney Diseases ,Renal biopsy ,business ,030217 neurology & neurosurgery - Abstract
Background Lately, monoclonal gammopathy of renal significance (MGRS) has been defined as a group of renal disorders that are strongly associated with monoclonal protein, including amyloid immunoglobulin light chain (AL) amyloidosis. Amyloid myopathy is rare (1.5% of all patients with amyloidosis) and the prognosis is poor. Furthermore, only approximately 20% of patients with amyloid myopathy are reported to have renal involvement, indicating a lack of data in the literature. Case presentation Here, we report a rare case of MGRS-related AL amyloidosis complicated by amyloid myopathy that presented with muscle weakness in the upper and lower limbs, neck and fingers, and nephrotic syndrome. Blood, urine, and bone marrow examination revealed monoclonal gammopathy of undetermined significance (MGUS) (Bence Jones protein-lambda). Muscle biopsy of the vastus lateralis muscle demonstrated amyloid proteins in the sarcolemma and in the blood vessel walls on Congo red staining, suggesting amyloid myopathy, and tiny inclusions in fibers on modified Gomori trichrome stain. Although we thought they were reminiscent of nemaline bodies, we could not confirm the nature of this structure. Renal biopsy demonstrated amyloid proteins in the mesangial region, part of the capillary walls, and the blood vessel walls on direct fast scarlet staining. As these amyloid proteins were positive for p-component staining and negative for amyloid A staining, β2-microglobulin, and pre-albumin, and as lambda light chains were positive in the mesangial region, we diagnosed the patient with MGRS-related AL amyloidosis. Although he was treated with melphalan and dexamethasone, his symptoms did not improve. Conclusions AL amyloidosis involving the kidneys and muscles has a poor prognosis, and a delayed diagnosis of amyloid myopathy is common because of its rarity and frequent misdiagnosis, which increases organ function deterioration. Therefore, early detection, therapeutic intervention, and careful follow-up are crucial.
- Published
- 2021
27. Antibodies Binding Antigens
- Author
-
Wu, Tai Te
- Published
- 2001
- Full Text
- View/download PDF
28. A simple scheme for large scale purification of urine - Derived Bence Jones Kappa protein
- Author
-
Shamkant B. Badgujar, Arti M. Rane, Aditi A. Palav, Saurabh Kumar, Anil P. Dabholkar, Satish A. Sawant, Babasaheb U. Tandale, Siddharth B. Daftary, Narendra P. Sawant, and Sanjeev Lala
- Subjects
Endotoxins ,Waste Products ,Clinical Biochemistry ,Humans ,Cell Biology ,General Medicine ,Peptides ,Biochemistry ,Biomarkers ,Analytical Chemistry ,Bence Jones Protein - Abstract
We have investigated and optimized purification process, suitable for industrial scale, to obtain high purity grade Bence Jones Kappa Protein ('BJK-Protein'), while preserving its physiological properties and functions. BJK-Protein was obtained from a biological waste product i.e. human urine of renal failure patients. Isolated 'BJK-Protein' was analyzed by electrophoresis, western blotting, double immunodiffusion, SEC-HPLC assay and Mass Spectrometry (MS). The relative molecular mass of 'BJK-Protein' is 23054.2 Da. Moreover, dimer forms of 'BJK-Protein' were also detected in SDS-PAGE and mass spectrum corresponding to 46054.4 Da. The results of western blotting, immunoelectrophoresis, SEC-HPLC assay, and mass spectrometry analysis indicate a high purity (99 %) of 'BJK-Protein'. Peptide mass fingerprint analysis of 'BJK-Protein' yielded peptides that partially matches the known database sequences of kappa variable region (KV139_HUMAN) of immunoglobulin. This protein was found to be stable up to 20 months at 2-8 °C temperature and also found negative for major undesirable viral markers as well as bacterial endotoxin. With this purification approach, the cost of purified 'BJK-Protein' is significantly reduced as compared to the current market price of Kappa light chain available in international market.
- Published
- 2022
29. Multiple myeloma of the jaw: A case report
- Author
-
Shubhasini A Raghavan, Praveen Birur Nagaraj, Bhanushree Ramaswamy, and Darshana S Nayak
- Subjects
Anemia ,Bence Jones protein ,mandible ,multiple myeloma ,Dentistry ,RK1-715 ,Medical physics. Medical radiology. Nuclear medicine ,R895-920 - Abstract
Multiple myeloma is a systemic B-cell lymphoproliferative disease that causes osteolytic lesions in the vertebra, ribs, pelvic bone, skull and jaw. Rarely jaw lesions are seen as the first sign in multiple myeloma. This is a case report with follow up of a 57-year-old female patient, previously treated for osteoporosis, who presented with a swelling of the jaw. On radiographic examination, she was found to have osteolytic lesions in the mandible and skull bones. These conventional aids led to the diagnosis of multiple myeloma thereby proving that the osteoporotic lesions were a part of the spectrum of multiple myeloma. The patient underwent chemotherapy and is currently on follow-up. This case report emphasizes the importance of early diagnosis of multiple myeloma in the jaw using readily available technologies and illustrates the contribution that oral assessment can provide.
- Published
- 2014
- Full Text
- View/download PDF
30. THE ROLE OF PROGNOSTIC FACTORS IN OVERALL SURVIVAL IN PATIENTS WITH BENCE-JONES MULTIPLE MYELOMA - OUR EXPERIENCE.
- Author
-
IVIĆ, Anđelina ŽIVANOVIĆ, ATANASKOVIĆ, Lavinika, ELEZ, Marija, TASIĆ, Olga RADIĆ, BALINT, Bela, and STAMATOVIĆ, Dragana
- Subjects
- *
MULTIPLE myeloma , *PLASMA cells , *IMMUNOGLOBULINS , *CANCER chemotherapy , *MEDICAL statistics , *PROGNOSIS - Abstract
Introduction. Bence-Jones myeloma multiplex is a progressive disease characterized by excessive numbers of abnormal plasma cells in the bone marrow and overproduction of incomplete immunoglobulins, containing only the light chain portion of the immunoglobulins. This type of myeloma occurs 15-20%. The median overall survival is approximately 4 years. Aim of this study was to define prognostic factors affecting overall survival in Bence-Jones multiple myeloma patients. Material and Methods. From 1995 to 2015, we treated 75 patients (49 men and 26 female), average age 55.9 years (range 31-85). Results. Conventional chemotherapy introductory clinical response was achieved in 54 patients (72%), while in 21 patients (28%) the established disease was resistant. Transplantation was done in 45 patients (60%), while 30 patients (40%) were treated with conventional chemotherapy. In the group of patients with transplantation done, tandem was carried out in 11 patients and secondary stem cell transplantation was done in 5 relapsed patients. With 1 patient with tandem stem cell transplantation allogenic (singen) stem cell transplantation was done. Transplant related mortality is 1.5%. The transplanted patients had significantly longer PFS (mediana 13 months vs 7 months, p<0,05) and longer overal survival (mediana 55 months vs 26 months, p<0,001). Univariate log regression analysis showed that non-transplant patients are 5,1 times more likely to terminate lethal compared to transplant patients (RR 5,1; 95% C.I.43,47-2,52), p<0,001). Conclusion. Our study showed autologous stem cell transplantation is a more effective method of treatment of patients with Bence-Jones myeloma compared to the conventional chemotherapy. [ABSTRACT FROM AUTHOR]
- Published
- 2017
- Full Text
- View/download PDF
31. A new suggested approach in screening for Bence Jones protein and potential kidney damage.
- Author
-
Natali, Patrizia, De Santis, Elena, Patelli, Giovanna, Cucinelli, Maria Rosaria, Varani, Manuela, and Trenti, Tommaso
- Subjects
- *
IMMUNOGLOBULINS , *SERUM albumin - Published
- 2019
- Full Text
- View/download PDF
32. Autoantibody Catalysis
- Author
-
Paul, Sudhir and Paul, Sudhir, editor
- Published
- 1999
- Full Text
- View/download PDF
33. Immunoglobulin D-kappa multiple myeloma in a patient with rheumatoid arthritis: a case report and review of the literature
- Author
-
Hiroo Hashimoto, Yoko Kosaka, Sho Mokuda, Tomohiro Sugimoto, Tomoyo Kumagai, Eiji Sugiyama, Shintaro Hirata, Kiichi Komoto, Hideho Wada, Risa Shimizu, Yusuke Yoshida, and Tadahiro Tokunaga
- Subjects
Immunofixation ,medicine.medical_specialty ,Gastroenterology ,Immunoglobulin D ,Arthritis, Rheumatoid ,Immunoglobulin kappa-Chains ,Bone Marrow ,Internal medicine ,medicine ,Humans ,Multiple myeloma ,Aged ,Hematology ,medicine.diagnostic_test ,biology ,business.industry ,medicine.disease ,Bone marrow examination ,Myeloma Proteins ,Serum protein electrophoresis ,Rheumatoid arthritis ,biology.protein ,Female ,Differential diagnosis ,Multiple Myeloma ,business ,Bence Jones Protein - Abstract
A 77-year-old Japanese woman with a 21-year history of seropositive, erosive rheumatoid arthritis (RA) and a 10-year history of methotrexate (MTX) therapy was admitted with malaise and mild consciousness disturbance. Laboratory data showed hypercalcemia, acute kidney injury, normocytic anaemia, and thrombocytopenia. As we first assumed drug-induced toxicity by MTX and eldecalcitol, both were discontinued and leucovorin rescue therapy and calcitonin were administered. However, her condition continued to worsen. Serum protein electrophoresis showed only a small M-peak, immunoelectrophoresis of both the serum and urine demonstrated Bence-Jones kappa (κ) type monoclonal protein without immunoglobulin heavy chain, and bone marrow examination revealed proliferation of plasma cells. We diagnosed her with Bence-Jones κ type multiple myeloma (MM) and transferred her to the department of haematology of a higher order medical institution. Conclusively, the diagnosis of immunoglobulin (Ig) D-κ type MM, a rare variant of this disorder, was determined in accordance with serum immunofixation. Several previous studies have suggested that pre-existing RA is a risk factor for MM. Although IgD MM is characterised by its clinical severity and poor prognosis compared to other subtypes, it is often misdiagnosed or mistaken as light chain type MM, as in the present case, because of the low level of IgD M-protein, resulting in delayed diagnosis. Physicians must take MM into consideration as a differential diagnosis when inactive RA patients present with inexplicable elevated calcium, renal failure, anaemia, and bone lesion symptoms and should be aware of IgD MM to establish the correct diagnosis promptly.
- Published
- 2020
- Full Text
- View/download PDF
34. Historical perspectives in clinical pathology: Bence Jones protein—early urine chemistry and the impact on modern day diagnostics
- Author
-
Tahir S Pillay and Sheromna Sewpersad
- Subjects
medicine.medical_specialty ,Pathology ,030232 urology & nephrology ,Chemical pathologist ,Urinalysis ,Immunoglobulin light chain ,History, 21st Century ,Pathology and Forensic Medicine ,03 medical and health sciences ,0302 clinical medicine ,Predictive Value of Tests ,Serum free ,Biomarkers, Tumor ,medicine ,Humans ,Neoplasms, Plasma Cell ,Multiple myeloma ,Urine chemistry ,Pathology, Clinical ,Clinical pathology ,business.industry ,History, 19th Century ,General Medicine ,History, 20th Century ,medicine.disease ,Bence Jones protein ,030220 oncology & carcinogenesis ,Diffusion of Innovation ,business ,Bence Jones Protein - Abstract
This is the third in the series of historical articles dealing with developments in clinical pathology. Bence Jones proteins are immunoglobulin light chains found in excessive quantities in urine in multiple myeloma and are believed to be one of the first tumour markers ever discovered . Dr Henry Bence Jones is credited with the discovery of this protein in 1847 that bears his name and he can also be regarded as the first chemical pathologist/clinical chemist. Since then, numerous advances and refinements have been made in the measurement and detection of urine light chain proteins which have resulted in the current sensitive serum free light chain assays used today.
- Published
- 2020
- Full Text
- View/download PDF
35. Monoclonal gammopathy of renal significance: A novel combination of C3 glomerulopathy and light-chain proximal tubulopathy
- Author
-
Elena Zakharova, Olga A Vorobyeva, Ekaterina S Stolyarevich, Irina G. Rekhtina, and Tatyana A Makarova
- Subjects
0301 basic medicine ,Pathology ,medicine.medical_specialty ,Kidney ,business.industry ,030232 urology & nephrology ,Monoclonal immunoglobulin ,Immunoglobulin light chain ,medicine.disease ,Bence Jones protein ,03 medical and health sciences ,Monoclonal gammopathy ,030104 developmental biology ,0302 clinical medicine ,medicine.anatomical_structure ,Proximal Tubulopathy ,Glomerulopathy ,Monoclonal ,medicine ,medicine.symptom ,business - Abstract
Introduction: According to the recent International Kidney and Monoclonal Gammopathy Research Group Consensus report, the spectrum of monoclonal gammopathies of renal significance merges more than 12 conditions; and combinations of various patterns of monoclonal gammopathies of renal significance–associated kidney damage have already been described. We present a case of a previously unreported combination of C3 glomerulopathy and light-chain proximal tubulopathy. Case description: A 53-year-old Caucasian man presented with general weakness. Three years prior, a check-up revealed proteinuria and microhematuria, later accompanied by arterial hypertension, elevated serum creatinine, and a low serum C3 levels. On admission, his creatinine was 1.5 mg/dL; his protein excretion was 1900 mg/24 h with microhematuria at 150 hpf. His serum C3 was 79 mg/dL with a normal C4 level. Serum protein electrophoresis revealed an M-spike, immunochemistry confirmed monoclonal immunoglobulin G kappa secretion of 920 mg/dL, and traces of Bence–Jones protein kappa in his urine. A kidney biopsy showed mesangial hypercellularity with diffuse fine granular C3 mesangial expression and tubular atrophy with diffuse coarse granular kappa light-chain tubular epithelial cytoplasmic expression. Computed tomography did not reveal any destructive lesions. A bone marrow smear showed 5% plasma cells, and bone marrow cell flow cytometry demonstrated 87.4% of plasma cells with an aberrant phenotype. The patient was diagnosed with an monoclonal gammopathy of renal significance and administered bortezomib–cyclophosphamide–dexamethasone. After four courses, his monoclonal immunoglobulin G kappa secretion halved, but his serum creatinine and proteinuria remained almost unchanged. Chemotherapy was switched to lenalidomide–dexamethasone, and after five courses his immunoglobulin G kappa secretion decreased to traces, Bence–Jones protein was not observed and his urinalysis, serum creatinine, and C3 levels were detected as normal. Conclusion: The combination of kidney damage patterns, one of which was directly and the other indirectly associated with monoclonal gammopathy, complements the monoclonal gammopathies of renal significance spectrum. Chemotherapy led to a very good partial response and kidney function recovery.
- Published
- 2020
- Full Text
- View/download PDF
36. The dark side of current analytic methods for Bence Jones Proteinuria
- Author
-
P, Natali, G, Cigliana, C, Napodano, V, Basile, D, Debbia, K, Pocino, M, Savoia, M, Marino, F, Gulli, U, Basile, Natali, P, Cigliana, G, Napodano, C, Basile, V, Debbia, D, Pocino, K, Savoia, M, Marino, M, Gulli, F, and Basile, U
- Subjects
Electrophoresis ,Proteinuria ,Immunofixation ,Immunofixation, Electrophoresis, Free light chains, Bence Jones protein, Monoclonal component ,Immune Sera ,Neoplasms ,Monoclonal component ,Bence Jones protein ,Paraproteinemias ,Humans ,Immunoglobulin Light Chains ,Free light chains ,Settore MED/05 - PATOLOGIA CLINICA - Abstract
Bence Jones proteinuria (BJP) refers to monoclonal free immunoglobulin light chains detected in urine, deriving from the clonal expansion of plasma cells in the bone marrow in patients with plasma cell dyscrasias, associated with monoclonal gammopathies of uncertain origin. This review summarizes routinely diagnostic procedures to assess BJP highlighting critical steps of pre-analytical, analytical, and post-analytical phases.The best option for BJP detection is the first morning void urine sample and immunofixation electrophoresis detection technique (IFE) the recommended method, with the employment of specific polyvalent antisera. Other qualitative tests for a quick evaluation of BJP are currently available. Densitometric analysis performed on the 24-hour urine is the recommended method to quantify BJP. To overcome the 24-hour collection, it is possible to use morning urine sample and correlate the assessed value of BJP to creatininuria. In addition to the traditional ones, we here reviewed screening methods currently used to avoid false negatives and reduce the time around test (TAT), together with immunochemical quantification methods for increased sensitivity, after checking BJP by IFE. Mass spectrometry emerges as a new challenge in the determination of BJP.The employment of different based-assays methods may be useful for diagnostic purposes to improve the accuracy of BJP monitoring in monoclonal gammopathies.
- Published
- 2022
37. INSIGHTS INTO DIAGNOSIS AND MANAGEMENT OF ADVANCED MULTIPLE MYELOMA
- Author
-
Vasile Musteata, Doina Ranga, Larisa Musteata, Cristina Dudnic, and Nina Sghibneva-Bobeico
- Subjects
medicine.medical_specialty ,Chemotherapy ,Hematology ,business.industry ,medicine.medical_treatment ,medicine.disease ,Gastroenterology ,Bence Jones protein ,medicine.anatomical_structure ,Internal medicine ,Cohort ,Immunology and Allergy ,Medicine ,Diseases of the blood and blood-forming organs ,Bone marrow ,Stage (cooking) ,RC633-647.5 ,business ,Complication ,Multiple myeloma - Abstract
Objective The advanced stages of multiple myeloma (MM) commonly manifest a recurrent evolution, unfavorable prognosis and negative socio-economic impact. The increased rates of morbidity and DALYs, frequent complications and relapses, unfavorable socio-economic impact characterize MM as an actual issue of hematology and public health. The objective of the study was the identification of diagnostic patterns and the evaluation of short- and long-term results of treatment of the advanced stages of MM. Methodology The study is a cross-sectional descriptive analysis of a cohort of 50 newly diagnosed patients with advanced stages of MM, who have been treated and followed-up at the Hematology Dept. of the Oncology Institute from Moldova during 2016-2020. The diagnosis was assessed by cytological, immunohistochemical examinations of the bone tissue and bone marrow, and ELISA immunological test of the peripheral blood. The stage was asserted in each case according to the Revised International Staging System. Results The patients age ranged between 28-75 years (median - 57.7 years). MM developed mainly in persons aged 60-69 (52%) years and in rare cases under 39 years (6%). Females were 29 (58%), and males - 21 (42%). 31 (62%) patients were diagnosed in stage III, 14 (28%) - in stage II and 5 (10%) - in stage I. Immunoglobulin (Ig) G isotype was detected in 28 (56%) cases, IgA - in 12 (24%), light chains (Bence Jones MM) - in 10 (20%). Very good partial responses were achieved in 25 (50%) of patients. Conclusion MM was diagnosed mostly in patients of 60-69 years, females and stage III disease. Bone marrow myeloma cells ranged between 30-67% (median - 46%). Concerning the Ig isotype distribution in MM, IgG accounted the majority of cases. Refractory chronic renal failure was the most common complication (50% of cases) in advanced MM. Targeted chemotherapy proved to be efficient in the advanced stages of MM regardless of the gender, age and disease span. Very good partial responses lasted 12-24 months.
- Published
- 2021
38. [Atypical presentation of Bence Jones myeloma in a geriatric patient: A case report].
- Author
-
Burgos Bencosme N, Herrera Parra JE, López Álvarez EM, and Gutiérrez Rodríguez J
- Subjects
- Humans, Aged, Bence Jones Protein, Multiple Myeloma diagnosis
- Published
- 2023
- Full Text
- View/download PDF
39. Diagnostic reference range of κ/λ free light chain ratio to screen for Bence Jones proteinuria is not significantly influenced by GFR.
- Author
-
Schmidt‐Hieltjes, Yvonne, Elshof, Clemens, Roovers, Lian, and Ruinemans‐Koerts, Janneke
- Subjects
- *
MEDICAL screening , *PROTEINURIA , *GLOMERULAR filtration rate , *ELECTROPHORESIS , *URINE - Abstract
Objectives The aim of our study was to analyse whether the κ/λ free light chain ratio reference range for screening for Bence Jones proteinuria should be dependent on the estimated glomerular filtration rate ( eGFR). Methods The serum κ/λ free light chain ratio, eGFR, serum M-protein and Bence Jones protein were measured in 544 patients for whom Bence Jones protein analysis was ordered. Results In the population of patients without Bence Jones proteinuria or a M-protein ( n = 402), there is no gradual increase in κ/λ free light chain ratio with diminishing eGFR. The κ/λ free light chain ratio in this group was 0.56-1.86 (95% interval). With this diagnostic reference range of the κ/λ ratio, 105 of the 110 patients with Bence Jones protein could be identified correctly. Only five patients with Bence Jones proteinuria (<0.17 g/L) were missed, without diagnostic or therapeutic consequences. In 36 patients (6.6%), an abnormal κ/λ free light chain ratio was measured without the presence of Bence Jones proteinuria. Conclusions A κ/λ free light chain ratio in serum can be used safely and efficiently to select urine samples which should be analysed for Bence Jones proteinuria with an electrophoresis/immunofixation technique. Using this diagnostic reference range, the number of urine samples which should be analysed by electrophoresis/immunofixation could be reduced by 74%. The diagnostic reference interval can be determined best in a group of patients for whom Bence Jones analysis is indicated. For calculation of this reference range, the eGFR value does not need to be taken into account. [ABSTRACT FROM AUTHOR]
- Published
- 2016
- Full Text
- View/download PDF
40. Testicular extramedullary plasmacytoma
- Author
-
M Samet, B Nemat-Gorgani, and M Abbasi
- Subjects
Plasmacell tumor ,Extramedullary plasmacytoma ,Multiple Myeloma ,Bence jones protein ,Testis ,Medicine (General) ,R5-920 - Abstract
Extramedullary plasmacytomas are plasma cell tumors that arise outside the bone marrow. They are most often located in the head and neck. Extramedullary plasmacytomas account for approximately 3 percent of plasma cell malignancies. The diagnosis of an extramedullary plasmacytoma depends upon the demonstration of a monoclonal plasma cell tumor at an extramedullary site with no evidence of multiple myeloma based upon bone marrow examination and roentgenographic studies, absence of an M-protein in serum and/ or urine, and no related organ or tissue impairment. These tumors are highly responsive to local radiation therapy and rarely progress or recur. A 77 year old man referred for gradual enlargement of scrotum in April, 2004. He had no fever, weight loss or loss of appetite during the period. No tenderness, warmth or erythem of scrotum was observed on physical examination. No lymphadenopathy was detected. Later he had temporary relief because of antibiotic therapy . 7 months later, he referred again following the aggravation of his symptom. Ultrasonography of his scrotum was done and enlargement in the left testis was reported. Lab data including CBC,diff,ESR,AFP were normal but βHCG titer was reported higher than normal . He underwent radical orchiectomy and the specimen was sent for pathological study. Spermatocytic seminoma and plasmacytoma were suspected, but plasmacytoma was reported after the IHC . In order to rule out multiple myeloma, whole body bone scanning with sestamibi and urine Bence Jones protein were examined, all of which were negative. In bone marrow aspiration, the cellularity was normal and no pathologic lesion was reported. Considering clinical and paraclinical evidences, the diagnosis was extramedullary plasmacytoma.
- Published
- 2006
41. Structural Studies of Two Carbohydrate-Containing AL Chains (Λll) AL NØV And AL MC.
- Author
-
Gullaksen, N., Idsø, H., Nilsen, R., Sletten, K., Husby, G., Cornwell, G. G., Natvig, Jacob B., editor, Førre, Øystein, editor, Husby, Gunnar, editor, Husebekk, Anne, editor, Skogen, Bjørn, editor, Sletten, Knut, editor, and Westermark, Per, editor
- Published
- 1991
- Full Text
- View/download PDF
42. Multiple myeloma presenting as acute tubulointerstitial nephritis
- Author
-
Ayman Layka, Abed Alhalim Aljamal, Guillermo A. Herrera, Niharika Yedla, Ying Long, Hisham F. Bahmad, and Michael Schwartz
- Subjects
Pathology ,medicine.medical_specialty ,Case Reports ,Electron ,Pathology and Forensic Medicine ,medicine ,Clinical Case Report ,Bone pain ,Acute tubulointerstitial nephritis ,Internal medicine ,Multiple myeloma ,Microscopy ,Proteinuria ,Nephritis ,medicine.diagnostic_test ,business.industry ,Acute kidney injury ,medicine.disease ,RC31-1245 ,Bence Jones protein ,Microscopy, Electron ,Nephritis, Interstitial ,Medicine ,Renal biopsy ,medicine.symptom ,business ,Multiple Myeloma ,Interstitial - Abstract
Background Acute tubulointerstitial nephritis (ATIN) is a very rare paraneoplastic manifestation in patients with multiple myeloma (MM). It is an uncommon pattern of renal disease in such patients. Case presentation We report a case of an 82-year-old male who was admitted with acute kidney injury. Renal biopsy showed typical findings of light chain-associated ATIN with scattered inflammatory cells in the interstitium and associated active tubulitis. No other common manifestations of MM were present at the time of presentation, including hypercalcemia, hyperuricemia, proteinuria, bone pain or lytic bone lesions. Subsequent immunoassays revealed significant serum lambda light chain burden and Bence Jones protein in urine. Immunofluorescence demonstrated linear tubular basement membranes with positive staining for lambda light chain (3+). Electron microscopy (EM) further showed interstitial edema and inflammation. All the aforementioned findings are consistent with ATIN and supported the diagnosis of MM. Conclusions In conclusion, light chain-associated ATIN should be considered in the differential diagnosis of acute interstitial nephritis. Henceforth, serum free light chains as well as serum and urine protein electrophoresis should be included in the workup of such patients.
- Published
- 2021
43. Comparison of 24-hour versus random urine samples for determination and quantification of Bence Jones protein in a South African population
- Author
-
Nadine Rapiti, Verena Gounden, and Ashandree Reddy
- Subjects
medicine.medical_specialty ,Creatinine ,Treatment response ,business.industry ,random urine ,Clinical Biochemistry ,Public Health, Environmental and Occupational Health ,Urology ,Urine ,bence jones protein ,Bence Jones protein ,Urine collection device ,Excretion ,multiple myeloma ,Medical Laboratory Technology ,chemistry.chemical_compound ,estimated 24-h bence jones protein ,African population ,chemistry ,medicine ,Sample collection ,Public aspects of medicine ,RA1-1270 ,business ,Original Research - Abstract
Background The International Myeloma Working Group and College of American Pathologists recommend a 24-h urine collection to determine the Bence Jones protein (BJP) excretion level for monitoring treatment response in patients with multiple myeloma (MM). There are several issues related to sample collection and the method is prone to inaccuracy. Objective This study compared measured 24-h to random urine collections for the quantitation of BJP in a South African population. Methods Sixty-six patients with MM submitted random urine samples with their routine 24-h urine collection from April 2016 - March 2018. Measured 24-h urine BJP was compared to two estimated 24-h BJP excretions calculated as follows: Estimation 1 (E1): Estimated 24-h BJP (mg/24 h) = Urine BJP/Creatinine ratio (mg/mmol) × 10. Estimation 2 (E2): Estimated 24-h BJP (mg/24 h) = Urine BJP/Creatinine ratio (mg/mmol) × 15 mg/kg for women or × 20 mg/kg for men. Results Correlation of estimation equations E1 and E2 to the measured 24-h urine BJP was 0.893. Patients showed no difference in classification of treatment response using either the E1 or E2 estimation equations when compared to the measured 24-h urine BJP results. Conclusion This study demonstrates that the estimated 24-h BJP shows a high degree of correlation with the measured 24-h BJP and can likely be used to monitor treatment response in South African patients with MM.
- Published
- 2021
44. Nephelometric assay of urine free light chains: an alternative and early clinical test for Bence-Jones protein quantification.
- Author
-
Pieri, Massimo, Pignalosa, Stefano, Franceschini, Luca, Rizzo, Manuela, Cantonetti, Maria, Zenobi, Rossella, Bernardini, Sergio, and Dessi, Mariarita
- Subjects
- *
MULTIPLE myeloma , *NEPHELOMETRY , *B cells , *GEL electrophoresis - Published
- 2018
- Full Text
- View/download PDF
45. Clinico-hematological profile of multiple myeloma in a teaching hospital - A 2 year study
- Author
-
Naseeruddin Sheik, Sandhya Krupal Variganji, Venkata Renuka Inuganti, Pravallika Uppala, and Phani Meghana Bolla
- Subjects
medicine.medical_specialty ,Anemia ,business.industry ,Urinary system ,Incidence (epidemiology) ,medicine.disease ,Bence Jones protein ,Internal medicine ,medicine ,Histopathology ,Differential diagnosis ,business ,Pathological ,Multiple myeloma - Abstract
Introduction: Multiple myeloma accounts for 1% of all cancers and 10-15% of all hematologic malignancies. It is characterized by bone marrow infiltration with clonal plasma cells, production of monoclonal immunoglobulin, and associated end-organ damage. The study aims to find out the incidence of multiple myeloma and to study the clinico-hematological profile along with radiological features. Materials and Methods: The present study was done both retrospectively and prospectively in 26 patients of multiple myeloma diagnosed over a period of 2 years from June 2017 to June 2019 in our teaching hospital. Data from hematological, biochemical, and radiological investigations were collected. For evaluation of each case, revised International Myeloma Working Group criteria were applied. Results: 26 patients were diagnosed during the study period, with the majority of them in the 6th decade with age range was 41-74 years. The male to female ratio was 1.3:1. Most common clinical feature was fever (50%) followed by bone pains (42%) and generalized weakness (42 %). Anemia was the most common hematological manifestation. All the patients had ‘M band’ on serum electrophoresis, and 27% of patients had urinary Bence Jones proteins. Among the skeletal system, the spine (63%) is the most common site of involvement. Conclusion: Among the 26 patients, various clinical presentations observed were pathological fracture, infections, renal impairment, generalized weakness in addition to anemia, and bone pains. Multiple myeloma should be considered as a differential diagnosis in old age patients presenting with such complaints. Keywords: Myeloma, Anemia, M band lytic lesions, Bence Jones protein.
- Published
- 2020
- Full Text
- View/download PDF
46. Plasma Cell Leukemia in a 7-month-old Infant
- Author
-
Zegadlo, M., Matysiak, M., Ochochka, M., Hiddemann, W., editor, Büchner, T., editor, Wörmann, B., editor, Ritter, J., editor, Creutzig, U., editor, Keating, M., editor, and Plunkett, W., editor
- Published
- 1998
- Full Text
- View/download PDF
47. Chest pain without a clue-ultrasound to rescue occult multiple myeloma: A case report
- Author
-
Virender Kumar Meena, Gopal Chawla, Naveen Dutt, and Kunal Deokar
- Subjects
medicine.medical_specialty ,Sternum ,business.industry ,Anemia ,Ultrasound ,Chest pain ,medicine.disease ,Occult ,Bence Jones protein ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,Clinching ,0302 clinical medicine ,Multiple myeloma ,Case report ,Medicine ,Radiology ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
Background Chest pain is one of the most common symptoms with which a patient presents to a doctor. Differentials include, but are not limited to, cardiac pulmonary, gastrointestinal, psychosomatic and musculoskeletal causes. In our case, ultrasound of the chest wall paved the way for the diagnosis of multiple myeloma, which occultly presented with chronic chest pain. Case summary Here we report a case of 50-year-old man with chronic chest pain without anemia or renal failure who was diagnosed with multiple myeloma, despite negative bence jones protein and M band electrophoresis. An ultrasound of the chest wall showed cortical irregularities along with a hypoechoic mass in the sternum and left 5th rib, which helped us in clinching the diagnosis. Conclusion Ultrasound of bone can often aid in reaching a diagnosis indirectly if not directly.
- Published
- 2019
48. Nanoporous silica coupled MALDI-TOF MS detection of Bence-Jones proteins in human urine for diagnosis of multiple myeloma
- Author
-
Yan Wang, Qin Qin, Baohong Liu, Yuning Wang, Yang Yi, Anmei Deng, Shuping Long, and Liang Qiao
- Subjects
Surface Properties ,02 engineering and technology ,Urine ,Mass spectrometry ,01 natural sciences ,Analytical Chemistry ,medicine ,Humans ,Particle Size ,Multiple myeloma ,Chromatography ,Chemistry ,Nanoporous ,010401 analytical chemistry ,Silicon Dioxide ,021001 nanoscience & nanotechnology ,medicine.disease ,Bence Jones protein ,0104 chemical sciences ,Matrix-assisted laser desorption/ionization ,Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization ,Clinical diagnosis ,Nanoparticles ,Biomarker (medicine) ,Multiple Myeloma ,0210 nano-technology ,Porosity ,Bence Jones Protein - Abstract
Bence-Jones protein is a biomarker in urine for multiple myeloma. Traditional methods for urine Bence-Jones protein detection are either less-sensitive or laborious. Herein, we describe a new method for the detection of urine Bence-Jones protein using nanoporous materials and matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). Macroporous ordered silica foams (MOSF) were used to enrich proteins in urine, and then the materials-proteins composites were analyzed by MALDI-TOF MS. Based on the presence of specific mass spectrometric signals, Bence-Jones protein can be detected for the diagnosis of multiple myeloma. Twenty-one clinical positive and twenty-seven clinical negative urine samples were analyzed by the method. High sensitivity (95.24%, 20/21) and specificity (100%, 27/27) for the diagnosis of multiple myeloma were achieved. Compared to other methods for multiple myeloma diagnosis, e.g. immunofixation electrophoresis and immunonephelometry, our approach is more rapid, economical and convenient, which can be a new choice for the clinical diagnosis of Bence-Jones protein related diseases.
- Published
- 2019
- Full Text
- View/download PDF
49. Difficulties evaluating hematological response in patients with multiple myeloma and dialysis - dependent renal impairment
- Author
-
I G Rekhtina, Larisa P. Mendeleeva, and N P Soboleva
- Subjects
History ,medicine.medical_specialty ,Endocrinology, Diabetes and Metabolism ,medicine.medical_treatment ,Urology ,lcsh:Medicine ,Renal function ,Diuresis ,Urine ,dialysis - dependent renal impairment ,Renal Dialysis ,Oliguria ,medicine ,Humans ,Renal Insufficiency ,Multiple myeloma ,Dialysis ,business.industry ,lcsh:R ,General Medicine ,medicine.disease ,Bence Jones protein ,hematological response ,serum free light chain level ,Monoclonal ,Immunoglobulin Light Chains ,medicine.symptom ,Multiple Myeloma ,Family Practice ,business ,Biomarkers ,Bence Jones Protein - Abstract
Aim: to determine serum free light chains (FLC) level by patients with multiple myeloma (MM) and dialysis - dependent renal impairment in which the amount Bence Jones (BJ) protein in the urine met the criteria of hematological response. Patients and methods: This study included 13 MM with dialysis - dependent renal impairment patients (estimated glomerular filtration rate < 10 ml/min), whose urine BJ protein content was less than 200 mg/day after antimyeloma therapy (including 11 patients whose urine BJ protein content was less than 100 mg/day). Results. The median serum concentration of monoclonal FLC was 608.7 (298-8380) mg/l. Thus, with trace amounts BJ protein in the urine serum content monoclonal FLC varied 28 times with the same degree of severity of renal failure. In patients with oliguria serum SLC content was significantly higher than in normal diuresis (1109 and 307 mg/L; p
- Published
- 2019
- Full Text
- View/download PDF
50. Clinical profile of multiple myeloma in a tertiary medical college northern Kerala, India
- Author
-
Kanniyan Binub, Ramani, and Sreeraj
- Subjects
Diplopia ,medicine.medical_specialty ,business.industry ,Anemia ,Incidence (epidemiology) ,medicine.disease ,Work-up ,Bence Jones protein ,Sickle cell anemia ,Internal medicine ,Medicine ,medicine.symptom ,business ,Paraplegia ,Multiple myeloma - Abstract
Introduction: Multiple myeloma is a clonal plasma cell disorder which varies its clinical course ranging from relatively indolent form to frankly aggressive neoplasia. Objectives: To study the clinical profile of Multiple myeloma. Materials and Methods: Descriptive study was conducted at a tertiary medical College in Kerala. 70 patients of Multiple myeloma was found with help of laboratory work up including urine Bence Jones with heat coagulation method. Serum albumin, calcium, uric acid was also done using standard biochemical kits. X-ray of Thoracic, Lumbar spine, Humerus, Hip region was evaluated. The frequency of variables was expressed as proportions and analysed. Results: The mean age of males was 62 and females 59 appropriately. The male/female ratio of Multiple myeloma was found to be 1.6:1. The associated neurological symptoms were root pain for 14.3% of patients. Almost 8.6% had paraparesis and 2.9% were suffering from paraplegia and 1.4% had quadriplegia, paraesthesia, buldging right eye and diplopia. Anemia was found in 87.1% and ESR was raised above 30 in 92.8% of patients. The total count more than Eleven thousand was found for 10% and less than four thousand for 1.4% of patients. Easnophilia was seen among 7.1%, 1.4% of patients had Peripheral smear >10% and Electrophoresis M band was seen in 78.6% of patients. Lytic lesions on X- Ray Skull neck were found in 62.9% and X Ray Spine were found in 1.4% of patients. Conclusion: 70 cases detected at a tertiary Medical College at Northern Kerala in a year indicate significant incidence of the disease. Professional Medical Associations should give importance to this topic and case studies of Multiple Myeloma should be included in medical conferences to raise the competence of doctors regarding the disease. Keywords: Multiple Myeloma, Northern Kerala.
- Published
- 2019
- Full Text
- View/download PDF
Catalog
Discovery Service for Jio Institute Digital Library
For full access to our library's resources, please sign in.