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Monoclonal Gammopathy

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1. Assessment of monoclonal gammopathies

Assessment of monoclonal gammopathies Assessment of monoclonal gammopathies - Differential diagnosis of symptoms | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Assessment of monoclonal gammopathies Last reviewed: February 2019 Last updated: September 2018 Summary Monoclonal gammopathies represent a wide spectrum of related diseases. Rajkumar SV, Dispenzieri A, Kyle RA. Monoclonal gammopathy of undetermined significance, Waldenstrom macroglobulinemia (...) , AL amyloidosis, and related plasma cell disorders: diagnosis and treatment. Mayo Clin Proc. 2006 May;81(5):693-703. http://www.ncbi.nlm.nih.gov/pubmed/16706268?tool=bestpractice.com Kyle RA. Current concepts on monoclonal gammopathies. Aust N Z J Med. 1992 Jun;22(3):291-302. http://www.ncbi.nlm.nih.gov/pubmed/1497556?tool=bestpractice.com Kyle RA, Therneau TM, Rajkumar SV, et al. Prevalence of monoclonal gammopathy of undetermined significance. N Engl J Med. 2006 Mar 30;354(13):1362-9. https

2018 BMJ Best Practice

2. Assessment of monoclonal gammopathies

Assessment of monoclonal gammopathies Assessment of monoclonal gammopathies - Differential diagnosis of symptoms | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Assessment of monoclonal gammopathies Last reviewed: February 2019 Last updated: September 2018 Summary Monoclonal gammopathies represent a wide spectrum of related diseases. Rajkumar SV, Dispenzieri A, Kyle RA. Monoclonal gammopathy of undetermined significance, Waldenstrom macroglobulinemia (...) , AL amyloidosis, and related plasma cell disorders: diagnosis and treatment. Mayo Clin Proc. 2006 May;81(5):693-703. http://www.ncbi.nlm.nih.gov/pubmed/16706268?tool=bestpractice.com Kyle RA. Current concepts on monoclonal gammopathies. Aust N Z J Med. 1992 Jun;22(3):291-302. http://www.ncbi.nlm.nih.gov/pubmed/1497556?tool=bestpractice.com Kyle RA, Therneau TM, Rajkumar SV, et al. Prevalence of monoclonal gammopathy of undetermined significance. N Engl J Med. 2006 Mar 30;354(13):1362-9. https

2018 BMJ Best Practice

3. Monoclonal gammopathy of undetermined significance

Monoclonal gammopathy of undetermined significance Monoclonal gammopathy of undetermined significance - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Monoclonal gammopathy of undetermined significance Last reviewed: February 2019 Last updated: January 2018 Summary Asymptomatic pre-malignant disorder associated with relatively low risk (on average 0.5%-1.0% per year) of progression to multiple myeloma or related (...) plasma cell proliferative malignancies. No aetiological risk factors have been defined. Male gender, older age, family history of monoclonal gammopathy of undetermined significance, black ancestry, and exposure to radiation or pesticide are associated with a higher prevalence. There is no indication for specific treatment. Clinical- and laboratory-based follow-up is recommended. Definition Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic disorder associated

2018 BMJ Best Practice

4. The evaluation of monoclonal gammopathy of renal significance: a consensus report of the International Kidney and Monoclonal Gammopathy Research Group. (PubMed)

The evaluation of monoclonal gammopathy of renal significance: a consensus report of the International Kidney and Monoclonal Gammopathy Research Group. The term monoclonal gammopathy of renal significance (MGRS) was introduced by the International Kidney and Monoclonal Gammopathy Research Group (IKMG) in 2012. The IKMG met in April 2017 to refine the definition of MGRS and to update the diagnostic criteria for MGRS-related diseases. Accordingly, in this Expert Consensus Document, the IKMG (...) redefines MGRS as a clonal proliferative disorder that produces a nephrotoxic monoclonal immunoglobulin and does not meet previously defined haematological criteria for treatment of a specific malignancy. The diagnosis of MGRS-related disease is established by kidney biopsy and immunofluorescence studies to identify the monotypic immunoglobulin deposits (although these deposits are minimal in patients with either C3 glomerulopathy or thrombotic microangiopathy). Accordingly, the IKMG recommends a kidney

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2018 Nature reviews. Nephrology

5. Publisher Correction: The evaluation of monoclonal gammopathy of renal significance: a consensus report of the International Kidney and Monoclonal Gammopathy Research Group. (PubMed)

Publisher Correction: The evaluation of monoclonal gammopathy of renal significance: a consensus report of the International Kidney and Monoclonal Gammopathy Research Group. In the key to Figure 1 of this article, 'Amyloid microtubules' has been corrected to 'Microtubules'.

2018 Nature reviews. Nephrology

6. A risk-stratification model based on the initial concentration of the serum monoclonal protein and MYD88 mutation status identifies a subset of patients with IgM monoclonal gammopathy of undetermined significance at high risk of progression to Waldenström (PubMed)

A risk-stratification model based on the initial concentration of the serum monoclonal protein and MYD88 mutation status identifies a subset of patients with IgM monoclonal gammopathy of undetermined significance at high risk of progression to Waldenström IgM monoclonal gammopathies of undetermined significance (IgM MGUS) are associated with a risk of progression to Waldenström macroglobulinaemia (WM) or other lymphoproliferative disorders (LPD) of 1-2% per year. We analysed 176 consecutive (...) patients with IgM MGUS to evaluate risk factors for progression. With a median follow-up of 83 months (1214 person-years), 15 patients (8·5%) progressed to WM (n = 14) or marginal zone lymphoma (n = 1). The rate of progression was 1·32% per year (95% confidence interval [CI] 0·80-2·20). The serum monoclonal protein concentration and the MYD88 mutation were independent risk factors for progression (Hazard ratio [HR] 23·3, 95% CI 2·0-273·3, P = 0·012 and HR 24·4, 95% CI 2·2-275·3, P = 0·010, respectively

2019 British journal of haematology

7. Post-MGUS diagnosis serum monoclonal-protein velocity and the progression of monoclonal gammopathy of undetermined significance to multiple myeloma. (PubMed)

Post-MGUS diagnosis serum monoclonal-protein velocity and the progression of monoclonal gammopathy of undetermined significance to multiple myeloma. Multiple myeloma (MM) is a common hematologic malignancy consistently preceded by monoclonal gammopathy of undetermined significance (MGUS). Little is known about post-diagnosis clinical predictors of progression of MGUS to MM to guide MGUS management. This study aimed to investigate whether the rate of rise in serum monoclonal protein

2019 Cancer Epidemiology & Biomarkers and Prevention

8. Long-Term Follow-up of Monoclonal Gammopathy of Undetermined Significance. (PubMed)

Long-Term Follow-up of Monoclonal Gammopathy of Undetermined Significance. Monoclonal gammopathy of undetermined significance (MGUS) occurs in approximately 3% of persons 50 years of age or older.We studied 1384 patients who were residing in southeastern Minnesota and in whom MGUS was diagnosed at the Mayo Clinic in the period from 1960 through 1994; the median follow-up was 34.1 years (range, 0.0 to 43.6). The primary end point was progression to multiple myeloma or another plasma-cell (...) (ratio of kappa to lambda free light chains) and a high serum monoclonal protein (M protein) level (≥1.5 g per deciliter) - was associated with a risk of progression at 20 years of 55%, as compared with 41% among patients who had one adverse risk factor and 19% among patients who had neither risk factor. Among patients with non-IgM MGUS, the risk of progression at 20 years was 30% among those who had the two risk factors, 20% among those who had one risk factor, and 7% among those who had neither

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2018 NEJM

9. Serum Free Light Chains in Neoplastic Monoclonal Gammopathies: Relative Under-Detection of Lambda Dominant Kappa/Lambda Ratio, and Underproduction of Free Lambda Light Chains, as Compared to Kappa Light Chains, in Patients With Neoplastic Monoclonal Gammo (PubMed)

Serum Free Light Chains in Neoplastic Monoclonal Gammopathies: Relative Under-Detection of Lambda Dominant Kappa/Lambda Ratio, and Underproduction of Free Lambda Light Chains, as Compared to Kappa Light Chains, in Patients With Neoplastic Monoclonal Gammo Quantitative evaluation of serum free light chains is recommended for the work up of monoclonal gammopathies. Immunoglobulin light chains are generally produced in excess of heavy chains. In patients with monoclonal gammopathy, κ/λ ratio (...) is abnormal less frequently with lambda chain lesions. This study was undertaken to ascertain if the levels of overproduction of the two light chain types and their detection rates are different in patients with neoplastic monoclonal gammopathies.Results of serum protein electrophoresis (SPEP), serum protein immunofixation electrophoresis (SIFE), urine protein electrophoresis (UPEP), urine protein immunofixation electrophoresis (UIFE), and serum free light chain assay (SFLCA) in patients with monoclonal

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2018 Journal of clinical medicine research

10. Both Monoclonal and Polyclonal Immunoglobulin Contingents Mediate Complement Activation in Monoclonal Gammopathy Associated-C3 Glomerulopathy (PubMed)

Both Monoclonal and Polyclonal Immunoglobulin Contingents Mediate Complement Activation in Monoclonal Gammopathy Associated-C3 Glomerulopathy C3 glomerulopathy (C3G) results from acquired or genetic abnormalities in the complement alternative pathway (AP). C3G with monoclonal immunoglobulin (MIg-C3G) was recently included in the spectrum of "monoclonal gammopathy of renal significance." However, mechanisms of complement dysregulation in MIg-C3G are not described and the pathogenic effect (...) of the monoclonal immunoglobulin is not understood. The purpose of this study was to investigate the mechanisms of complement dysregulation in a cohort of 41 patients with MIg-C3G. Low C3 level and elevated sC5b-9, both biomarkers of C3 and C5 convertase activation, were present in 44 and 78% of patients, respectively. Rare pathogenic variants were identified in 2/28 (7%) tested patients suggesting that the disease is acquired in a large majority of patients. Anti-complement auto-antibodies were found in 20/41

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2018 Frontiers in immunology

11. Monoclonal light chain crystalline podocytopathy and tubulopathy associated with monoclonal gammopathy of renal significance: a case report and literature review. (PubMed)

Monoclonal light chain crystalline podocytopathy and tubulopathy associated with monoclonal gammopathy of renal significance: a case report and literature review. Monoclonal gammopathy of renal significance (MGRS) is a recently defined group of renal diseases caused by monoclonal immunoglobulin secreted by nonmalignant proliferative B cell or plasma cell. Monoclonal immunoglobulin can form different types of structures deposited in renal tissue, including fibrils, granules, microtubules (...) , crystals and casts, and has mostly been reported in multiple myeloma patients. Here we report a rare case with κ light chain crystals in both podocytes and tubular epithelial cells associated with MGRS, which adds more information to the spectrum of MGRS-related renal diseases.A 53-year old woman presented with albumin-predominant moderate proteinuria and renal failure. She had monoclonal IgGκ in the serum and monoclonal IgGκ plus free κ in the urine. Multiple myeloma and lymphoproliferative disorders

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2018 BMC Nephrology

12. Bone disease in monoclonal gammopathy of undetermined significance: results from a screened population-based study (PubMed)

Bone disease in monoclonal gammopathy of undetermined significance: results from a screened population-based study Previous studies have shown that individuals with monoclonal gammopathy of undetermined significance (MGUS) have an increased risk of fractures, although the underlying mechanisms remain unknown. Our aim was to analyze bone mineral density (BMD), bone volume, and risk of fractures among individuals with MGUS. We performed a screening using the Age, Gene/Environment Susceptibility

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2017 Blood advances

13. Changes in bone marrow innate lymphoid cell subsets in monoclonal gammopathy: target for IMiD therapy (PubMed)

Changes in bone marrow innate lymphoid cell subsets in monoclonal gammopathy: target for IMiD therapy Altered number, subset composition, and function of bone marrow innate lymphoid cells are early events in monoclonal gammopathies.Pomalidomide therapy leads to reduction in Ikzf1 and Ikzf3 and enhanced human innate lymphoid cell function in vivo.

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2017 Blood advances

14. Obesity and risk of monoclonal gammopathy of undetermined significance and progression to multiple myeloma: a population-based study (PubMed)

Obesity and risk of monoclonal gammopathy of undetermined significance and progression to multiple myeloma: a population-based study All multiple myeloma (MM) cases are preceded by the premalignant state monoclonal gammopathy of undetermined significance (MGUS). Results from previous studies show a positive association between obesity and MM; however, the association between obesity and MGUS is controversial. The aims were to determine (1) if obesity is associated with an increased risk of MGUS

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2017 Blood advances

15. Identification of comorbidities in people with Monoclonal Gammopathy of Undetermined Significance (MGUS) or Smoldering Myeloma (SMM), their incidence, prevalence and associated risk factors: an original protocol for a systematic review

Identification of comorbidities in people with Monoclonal Gammopathy of Undetermined Significance (MGUS) or Smoldering Myeloma (SMM), their incidence, prevalence and associated risk factors: an original protocol for a systematic review Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied for this submission

2019 PROSPERO

16. Dual anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody-positive crescent glomerulonephritis in a patient with monoclonal gammopathy of undetermined significance: A case report. (PubMed)

Dual anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody-positive crescent glomerulonephritis in a patient with monoclonal gammopathy of undetermined significance: A case report. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti-glomerular basement membrane (GBM) antibody disease are both rare autoimmune diseases. Monoclonal gammopathy of undetermined significance (MGUS) is one of the most common causes of plasma cell dyscrasias (...) immunofixation electrophoresis found monoclonal immunoglobulin (MIg) G κ-light chain in the serum. Renal biopsy displayed crescentic formation in glomerule by microscopy and staining for liner IgG (+), sparse C3 (+-) and light chain (κ and λ) (+-) by immunofluorescence. The bone marrow examination indicated basically normal myelogram and sporadic plasma cells positive for CD38, CD138 staining, and κ light-chain restriction.Crescentic glomerulonephritis and MGUS.The patient was treated with plasmapheresis

2019 Medicine

17. Monoclonal gammopathy of undetermined significance: A primary care guide. (PubMed)

Monoclonal gammopathy of undetermined significance: A primary care guide. Monoclonal gammopathy of undetermined significance (MGUS) is commonly diagnosed in outpatients being worked up for an array of clinical concerns. It carries a risk of progression to myeloma and other lymphoproliferative disorders that, albeit low (1% per year), warrants regular follow-up. Patients with MGUS can be risk-stratified on the basis of the amount and type of their monoclonal protein as well as whether they have

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2019 Cleveland Clinic Journal of Medicine

18. The Uncertainty of Monoclonal Gammopathies

The Uncertainty of Monoclonal Gammopathies Renal Fellow Network: The Uncertainty of Monoclonal Gammopathies | | | | | Wednesday, March 14, 2018 The Uncertainty of Monoclonal Gammopathies I spend a lot of time discussing patients with renal disease and monoclonal gammopathies with my haematology colleagues, trying to figure out what is of ‘renal significance’ and ‘undetermined significance’. One of those discussions recently was around a normally well 70 year old lady. She presented with several (...) steroids and initially plasma exchange whilst results were returned. Her renal function improved over the following week and she did not require further specific therapy given the absence of overt lymphoproliferative or autoimmune disease. It is not surprising that we pick up a lot of monoclonal gammopathies in patients with deteriorating renal function or proteinuria as MGUS affects 3% of patients over the age of 50. Recent suggests that MGUS progresses to myeloma or other plasma cell

2018 Renal Fellow Network

19. Impact of prior diagnosis of monoclonal gammopathy on outcomes in newly diagnosed multiple myeloma. (PubMed)

Impact of prior diagnosis of monoclonal gammopathy on outcomes in newly diagnosed multiple myeloma. Multiple myeloma (MM) is consistently preceded by monoclonal gammopathy of undetermined significance (MGUS), smoldering myeloma (SMM), or solitary plasmacytoma (SPC). There is a lack of data regarding impact of these pre-existing monoclonal gammopathies (MGs) on MM outcomes. Patients with prior diagnosis of MGUS, SMM, or PC from 1973 to 2015 (cases) were identified from our institution's database

2019 Leukemia

20. MALDI-TOF analysis of blood serum proteome can predict the presence of monoclonal gammopathy of undetermined significance. (PubMed)

MALDI-TOF analysis of blood serum proteome can predict the presence of monoclonal gammopathy of undetermined significance. Monoclonal gammopathy of undetermined significance (MGUS) is a plasma cell dyscrasia that can progress to malignant multiple myeloma (MM). Specific molecular biomarkers to classify the MGUS status and discriminate the initial asymptomatic phase of MM have not been identified. We examined the serum peptidome profile of MGUS patients and healthy volunteers using MALDI-TOF

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2018 PLoS ONE

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