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European Journal of Medical Research Jul 2021This study aimed to analyze the clinicopathological characteristics of patients with paraproteinemia and renal damage.
BACKGROUND
This study aimed to analyze the clinicopathological characteristics of patients with paraproteinemia and renal damage.
METHODS
Ninety-six patients from 2014 to 2018 with paraproteinemia and renal damage were enrolled and the clinical data, renal pathology, treatment and prognosis data were collected.
RESULTS
A total of 96 patients (54 male and 42 female), accounting for 2.7% of all renal biopsies, were enrolled in this study. Among them, 42 were monoclonal gammopathy of renal significance (MGRS), 21 were renal monotypic immunoglobulin alone (renal monoIg), and 19 were monoclonal gammopathy of undetermined significance (MGUS). Individuals with multiple myeloma (MM) accounted for the fewest number of patients (n = 14). In the MGRS group, the main diseases were amyloidosis (n = 25) and cryoglobulinemic glomerulonephritis (n = 7), while in the MM group, the main diseases were cast nephropathy (n = 9) and light chain deposit disease (n = 3). In the MGUS group, it was mainly IgA nephropathy (IgAN, n = 10) and idiopathic membranous nephropathy (n = 5); while in the renal monoIg group, most of the cases were IgAN (n = 19). Chemotherapy was mainly administered to patients in the MM group, while immunosuppression therapy was mostly administered to patients in the renal monoIg group. Most patients with renal monoIg exhibited a major response, followed by the patients with MGUS and MGRS, while most of the patients with MM had a partial response but none had a major response. Approximately more than half (57.1%) of the patients with MM progressed to end-stage renal disease (ESRD), followed by MGRS (33.3%); however, the mortality rate was low in both the MGRS and MM groups. The survival analysis reviewed that serum creatinine, hemoglobin levels, and the serum κ/λ ratio were independent risk factors for ESRD in patients with MGRS.
CONCLUSIONS
The clinicopathological changes in patients with MGRS were between those in patients with MM and MGUS. The treatment for MGRS and MM was more intensive, and the overall mortality rate was low. Both MGUS and renal monoIg alone exhibited slighter clinicopathological features than MGRS and MM, and the treatment was focused mostly on primary renal diseases.
Topics: Adult; Biopsy; China; Female; Humans; Incidence; Kidney; Kidney Diseases; Male; Middle Aged; Paraproteinemias; Prognosis
PubMed: 34217367
DOI: 10.1186/s40001-021-00538-2 -
Frontiers in Immunology 2020
Topics: Animals; Antibodies; Cellular Microenvironment; Humans; Hypersensitivity; Paraproteinemias; Parasitic Diseases; Phenotype; Plasma Cells; Time Factors
PubMed: 33193457
DOI: 10.3389/fimmu.2020.606737 -
Ugeskrift For Laeger Mar 2020This review summarises the work-up of patients with monoclonal gammopathy of undetermined significance (MGUS). In persons above 70 years of age, around 5% have MGUS, a... (Review)
Review
This review summarises the work-up of patients with monoclonal gammopathy of undetermined significance (MGUS). In persons above 70 years of age, around 5% have MGUS, a premalignant state with a monoclonal plasma immunoglobulin or light chain (M protein) in blood and/or urine. Continuous follow-up is recommended due to a risk of malignant progression of around 1% per year. Immunoglobulin M MGUS primarily progresses to Waldenström's macroglobulinaemia, whereas non-immunoglobulin M MGUS typically progresses to multiple myeloma or amyloid light-chain amyloidosis. Treatment is unnecessary unless in rare cases of severe non-malignant complications. Screening is not advised.
Topics: Humans; Immunoglobulin Light-chain Amyloidosis; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Waldenstrom Macroglobulinemia
PubMed: 32138824
DOI: No ID Found -
Nephrology, Dialysis, Transplantation :... Oct 2023Light chain proximal tubulopathy (LCPT) is a rare form of paraprotein-related disease, occurring in two main histopathological forms: crystalline and non-crystalline....
BACKGROUND
Light chain proximal tubulopathy (LCPT) is a rare form of paraprotein-related disease, occurring in two main histopathological forms: crystalline and non-crystalline. The clinicopathological features, treatment strategies and outcomes, especially of the non-crystalline form, are not well described.
METHODS
We conducted a single-centre retrospective case series of 12 LCPT patients, 5 crystalline and 7 non-crystalline, between 2005 and 2021.
RESULTS
The median age was 69.5 years (range 47-80). Ten patients presented with CKD and significant proteinuria (median estimated glomerular filtration rate of 43.5 ml/min/1.73 m2; urine protein:creatinine ratio 328 mg/mmol). Only six patients had known haematological disease at the time of renal biopsy. Multiple myeloma (MM) was diagnosed in seven patients cases and monoclonal gammopathy of renal significance (MGRS) in five patients. A clone was detected in all cases combining serum/urine electrophoresis and free light chain (LC) assays. Crystalline and non-crystalline variants had similar clinical presentations. For the non-crystalline variant, a diagnosis was reached based on a combination of CKD without another cause, haematological workup, LC restriction on immunofluorescence and abnormalities on electron microscopy (EM). Nine of 12 patients received clone-directed treatment. Patients who achieved haematological response (including all non-crystalline LCPT) had improved renal outcomes over a median follow-up of 79 months.
CONCLUSIONS
The non-crystalline variant may go unrecognised because of its subtle histopathological features and requires EM to distinguish it from 'excessive LC resorption without tubular injury'. Clone-directed treatment with good haematological response improves renal outcomes in both variants but limited data exist in MGRS. Multicentre prospective studies are needed to better define the clinicopathological characteristics associated with poor outcomes and optimize treatment strategies in patients with MGRS.
Topics: Humans; Middle Aged; Aged; Aged, 80 and over; Retrospective Studies; Kidney Diseases; Kidney; Multiple Myeloma; Immunoglobulin Light Chains; Renal Insufficiency, Chronic; Paraproteinemias
PubMed: 37120733
DOI: 10.1093/ndt/gfad085 -
Acta Haematologica 2019
Topics: B-Lymphocytes; Hematopoietic Stem Cell Transplantation; Homeostasis; Humans; Paraproteinemias; Prognosis; Stem Cell Transplantation
PubMed: 30439698
DOI: 10.1159/000494426 -
Blood Cancer Journal Dec 2023Monoclonal gammopathy of undetermined significance (MGUS) is the earliest discernible stage of multiple myeloma (MM) and Waldenström's macroglobulinemia (WM). Early...
Immunophenotypic assessment of clonal plasma cells and B-cells in bone marrow and blood in the diagnostic classification of early stage monoclonal gammopathies: an iSTOPMM study.
Monoclonal gammopathy of undetermined significance (MGUS) is the earliest discernible stage of multiple myeloma (MM) and Waldenström's macroglobulinemia (WM). Early diagnosis of MG may be compromised by the low-level infiltration, undetectable to low-sensitive methodologies. Here, we investigated the prevalence and immunophenotypic profile of clonal (c) plasma cells (PC) and/or cB-lymphocytes in bone marrow (BM) and blood of subjects with a serum M-component from the iSTOPMM program, using high-sensitive next-generation flow cytometry (NGF), and its utility in the diagnostic classification of early-stage MG. We studied 164 paired BM and blood samples from 82 subjects, focusing the analysis on: 55 MGUS, 12 smoldering MM (SMM) and 8 smoldering WM (SWM). cPC were detected in 84% of the BM samples and cB-lymphocytes in 45%, coexisting in 39% of cases. In 29% of patients, the phenotypic features of cPC and/or cB-lymphocytes allowed a more accurate disease classification, including: 19/55 (35%) MGUS, 1/12 (8%) SMM and 2/8 (25%) SWM. Blood samples were informative in 49% of the BM-positive cases. We demonstrated the utility of NGF for a more accurate diagnostic classification of early-stage MG.
Topics: Humans; Plasma Cells; Bone Marrow; Paraproteinemias; B-Lymphocytes; Multiple Myeloma; Monoclonal Gammopathy of Undetermined Significance; Waldenstrom Macroglobulinemia; Smoldering Multiple Myeloma
PubMed: 38072838
DOI: 10.1038/s41408-023-00944-1 -
Cancer Control : Journal of the Moffitt... Jan 2015Disease complications associated with certain malignancies may be mediated by cells or soluble molecules that traffic in the bloodstream. Because of this, therapeutic... (Review)
Review
BACKGROUND
Disease complications associated with certain malignancies may be mediated by cells or soluble molecules that traffic in the bloodstream. Because of this, therapeutic apheresis (TA) methodologies have been used to selectively remove or manipulate specific molecules, antibodies, or cellular elements to treat the underlying pathological process. For some disorders, TA is utilized as a rapid-acting and short-term adjunct to conventional chemotherapy or immunotherapy. For others, a series of scheduled treatments is recommended for optimal management. In all cases, the risks, benefits, and costs must be strongly considered.
METHODS
The current literature and published guidelines were reviewed to summarize the use of TA in the management of certain complications of cancer.
RESULTS
Although TA is relatively safe and useful as a first-line or salvage modality for some disorders, few prospective, randomized clinical trials exist and the majority of evidence is derived from observational studies. Expert-based, clinical practice guidelines have been developed to inform hematology/oncology professionals and apheresis physicians about the efficacy and limitations of TA for malignancy-related indications.
CONCLUSIONS
Certain oncological conditions respond to TA and consensus guidelines are available to support clinical decision-making. However, well-designed, prospective intervention trials are needed to better define the role of TA for a variety of disorders.
Topics: Blood Component Transfusion; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Leukapheresis; Neoplasms; Paraproteinemias; Plasma Exchange; Plateletpheresis
PubMed: 25504280
DOI: 10.1177/107327481502200109 -
Aging Dec 2018Plasma cell dyscrasias are a rare heterogeneous group of hematological disorders which are more prevalent in the older part of the population. The introduction of novel... (Review)
Review
Plasma cell dyscrasias are a rare heterogeneous group of hematological disorders which are more prevalent in the older part of the population. The introduction of novel agents, improved understanding of disease biology and better supportive management have improved outcomes considerably and in the era of the aging population the question of how to best manage older patients with plasma cell dyscrasias has never been more relevant. Data on how to treat these patients comes mostly from subgroup analysis as they are underrepresented in clinical trials. This review will cover issues, available evidence and recommendations relevant to diagnosis and management of the older patients with Multiple Myeloma (MM), Waldenstrom Macroglobulinemia (WM) and systemic AL Amyloidosis. What will become increasingly evident is the need to develop and establish the use of disease-specific geriatric assessment (GA) tools. Frailty status assessment using GA tools and moving away from making decisions based merely on chronological age will allow setting clear treatment goals and consequently achieving an optimum balance between effectiveness and toxicity for this complex and heterogeneous group of patients.
Topics: Aged; Frailty; Geriatric Assessment; Humans; Paraproteinemias
PubMed: 30568029
DOI: 10.18632/aging.101707 -
BMC Nephrology Jan 2024It is crucial to identify patients with monoclonal gammopathy of renal significance (MGRS) from those without MGRS but with monoclonal gammopathy and concomitant kidney... (Meta-Analysis)
Meta-Analysis
BACKGROUND
It is crucial to identify patients with monoclonal gammopathy of renal significance (MGRS) from those without MGRS but with monoclonal gammopathy and concomitant kidney diseases. However, there have been few studies with large sample sizes, and their findings were inconsistent. This study aimed to conduct a meta-analysis of MGRS to describe the general characteristics of MGRS and its predictive factors.
METHODS
Cohort or case-control studies published through December 2022 and related to clinicopathological features of MGRS were retrieved from the PubMed, Cochrane Library, Web of Science, Scopus, and Embase databases. Two researchers searched for studies that met the inclusion criteria. In the univariate analysis, fixed- or random- effects models were used to obtain pooled estimates of the weighted mean difference (WMD) and odds ratio (OR) for risk factors. In the multivariate analysis, the ORs of the independent risk factors from each study were pooled after transforming the original estimates.
RESULTS
The meta-analysis included six studies. Univariate analysis showed that the following variables were statistically significant in MGRS: age (WMD = 1.78, 95%CI 0.21-3.35), hypertension (OR = 0.54, 95%CI 0.4-0.73), diabetes (OR = 0.42, 95%CI 0.29-0.59), albumin (WMD = - 0.26, 95%CI - 0.38--0.14), urinary protein level (WMD = 0.76, 95%CI 0.31-1.2), urinary protein ≥ 1.5 g/d (OR = 1.98, 95%CI 1.46-2.68), lambda-chain value (WMD = 29.02, 95%CI 16.55-41.49), abnormal free light-chain ratio (OR = 4.16, 95%CI 1.65-10.47), bone marrow puncture rate (OR = 5.11, 95% CI 1.31-19.95), and abnormal bone marrow outcome rate (OR = 9.63, 95%CI 1.98-46.88). Multivariate analysis showed urinary protein ≥ 1.5 g/d (OR = 2.80, 95%CI 1.53-5.15) and an abnormal free light-chain ratio (OR = 6.98, 95%CI 4.10-11.91) were associated with predictors of MGRS.
CONCLUSIONS
Compared with non-MGRS patients with monoclonal gammopathy and concomitant kidney diseases, patients with MGRS were older, had fewer underlying diseases, more urinary protein, more abnormal free light-chain ratio, and more abnormal bone marrow results. Urinary protein ≥ 1.5 g/d and an abnormal free light-chain ratio were independent risk factors for MGRS.
Topics: Humans; Paraproteinemias; Kidney; Monoclonal Gammopathy of Undetermined Significance; Kidney Diseases; Immunoglobulin Light Chains
PubMed: 38229028
DOI: 10.1186/s12882-024-03458-5 -
Frontiers in Immunology 2022
Topics: Humans; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma
PubMed: 35844583
DOI: 10.3389/fimmu.2022.960829