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Clinical Medicine (London, England) Oct 2018Monoclonal gammopathy of undetermined significance (MGUS) is characterised by the presence of a monoclonal paraprotein in the blood, without the characteristic end organ... (Review)
Review
Monoclonal gammopathy of undetermined significance (MGUS) is characterised by the presence of a monoclonal paraprotein in the blood, without the characteristic end organ damage seen in multiple myeloma. MGUS is more common in older age groups and has a risk of progression to myeloma of 1% per year. Population screening is not currently recommended, but retrospective studies have suggested improvements in myeloma outcomes in those under MGUS follow-up; in addition, MGUS has associated complications, including fracture, osteoporosis, renal disease and infection, which can be treated. Given this increasing evidence of disease related directly to MGUS, strategies for early identification might be needed. In this review, we discuss the complications of MGUS and whether MGUS fulfils the criteria needed to implement a screening programme. We also highlight areas where more evidence is needed, including identification of a higher risk population to make screening more practical and economically viable.
Topics: Disease Progression; Early Detection of Cancer; Fractures, Bone; Humans; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Risk Factors
PubMed: 30287433
DOI: 10.7861/clinmedicine.18-5-391 -
Frontiers in Immunology 2020Factor H (FH), a member of the regulators-of-complement-activation (RCA) family of proteins, circulates in human plasma at concentrations of 180-420 mg/L where it...
Factor H (FH), a member of the regulators-of-complement-activation (RCA) family of proteins, circulates in human plasma at concentrations of 180-420 mg/L where it controls the alternative pathway (AP) of complement in the fluid phase and on cell surfaces. When the regulatory function of FH is impaired, complement-mediated tissue injury and inflammation occur, leading to diseases such as atypical hemolytic uremic syndrome (a thrombotic microangiopathy or TMA), C3 glomerulopathy (C3G) and monoclonal gammopathy of renal significance (MGRS). A pathophysiological cause of compromised FH function is the development of autoantibodies to various domains of the FH protein. FH autoantibodies (FHAAs) are identified in 10.9% of patients with aHUS, 3.2% of patients with C3G, and rarely in patients with MGRS. The phenotypic variability of FHAA-mediated disease reflects both the complexity of FH and the epitope specificity of FHAA for select regions of the native protein. In this paper, we have characterized FHAA epitopes in a large cohort of patients diagnosed with TMA, C3G or MGRS. We explore the epitopes recognized by FHAAs in these diseases and the association of FHAAs with the genetic deletion of both copies of the gene to show how these disease phenotypes are associated with this diverse spectrum of autoantibodies.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Atypical Hemolytic Uremic Syndrome; Autoantibodies; Biomarkers; Child; Child, Preschool; Complement C3b Inactivator Proteins; Complement Factor H; Epitopes; Female; Gene Deletion; Genetic Predisposition to Disease; Glomerulonephritis; Humans; Infant; Male; Middle Aged; Paraproteinemias; Phenotype; Prevalence; Retrospective Studies; United States; Young Adult
PubMed: 33384694
DOI: 10.3389/fimmu.2020.607211 -
Acta Ophthalmologica Mar 2023The aim of this pilot study was to assess oxygen saturation in retinal blood vessels in patients with monoclonal gammopathies (MGs).
PURPOSE
The aim of this pilot study was to assess oxygen saturation in retinal blood vessels in patients with monoclonal gammopathies (MGs).
METHODS
Thirty-one patients with MGs (11 women and 20 men, mean age 65.9 ± 8.9 years) were enrolled during 2016-2020. The patients were diagnosed at the Haemato-Oncology Department and subsequently examined at the Ophthalmology Department before initiating systemic therapy. All patients were subjected to automatic retinal oximetry (Oxymap ehf.) and had their fundus photographed (Topcon TRC-50DX retinal camera). We assessed the association between retinal oxygen saturation (SatO ) - arterial SatO , venous SatO and arterio-venous (AV) difference-and MGs parameters: serum monoclonal immunoglobulin (M-protein) level and serum immunoglobulin-free light chains (FLC kappa and lambda), total protein, serum viscosity, haemoglobin, albumin, lactate dehydrogenase, C-reactive protein, creatinine and serum calcium level. Hyperviscosity-related retinopathy was also evaluated.
RESULTS
Statistical analysis showed a significant positive correlation (r = 0.462; p = 0.009) between the AV difference and the haemoglobin level. A significant, medium strong negative correlation was found between the AV difference and the serum levels of the monoclonal light lambda chains (r = -0.450; p = 0.011). Contrary to expectations, no statistically significant correlation was found between retinal oxygen saturation and the total protein or viscosity.
CONCLUSION
This study found correlation between retinal oxygen saturation and certain parameters in the blood of patients with MGs. Increasing levels of monoclonal immunoglobulin seem to reduce oxygen absorption in retinal arterioles, resulting in a lower AV difference, particularly in patients with a high free light chain level.
Topics: Male; Humans; Female; Middle Aged; Aged; Pilot Projects; Oxygen Saturation; Retina; Retinal Vessels; Oxygen; Oximetry; Paraproteinemias
PubMed: 36004612
DOI: 10.1111/aos.15239 -
Tidsskrift For Den Norske Laegeforening... Sep 2021Monoclonal gammopathy is a frequent finding and may be associated with severe cancer such as myelomatosis and other B-cell lymphoproliferative disorders. However, the...
Monoclonal gammopathy is a frequent finding and may be associated with severe cancer such as myelomatosis and other B-cell lymphoproliferative disorders. However, the monoclonal component can also be the direct cause of serious disease, namely monoclonal gammopathy of clinical significance (MGCS). MGCS is most likely significantly underdiagnosed and is consequently also undertreated. In order to achieve a good therapeutic outcome, it is crucial that the condition is recognised at an early stage, so that treatment can be initiated before the patient has developed irreversible organ damage. Increased awareness of MCGS is therefore essential.
Topics: Humans; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Paraproteinemias
PubMed: 34596996
DOI: 10.4045/tidsskr.20.0981 -
International Journal of Molecular... Mar 2024Multiple myeloma (MM) is a plasma cell disorder representing the second most common blood cancer [...].
Multiple myeloma (MM) is a plasma cell disorder representing the second most common blood cancer [...].
Topics: Humans; Multiple Myeloma; Paraproteinemias; Hematologic Neoplasms; Neoplasms, Second Primary
PubMed: 38612612
DOI: 10.3390/ijms25073799 -
Blood Jan 2018Monoclonal gammopathy of undetermined significance (MGUS) is, in many ways, a unique hematologic entity. Unlike most hematologic conditions in which the diagnosis is... (Review)
Review
Monoclonal gammopathy of undetermined significance (MGUS) is, in many ways, a unique hematologic entity. Unlike most hematologic conditions in which the diagnosis is intentional and credited to hematologists, the discovery of MGUS is most often incidental and made by nonhematologists. MGUS is considered an obligate precursor to several lymphoplasmacytic malignancies, including immunoglobulin light-chain amyloidosis, multiple myeloma, and Waldenström macroglobulinemia. Therefore, long-term follow-up is generally recommended. Despite its high prevalence, there is surprisingly limited evidence to inform best clinical practice both at the time of diagnosis and during follow-up. We present 7 vignettes to illustrate common clinical management questions that arise during the course of MGUS. Where evidence is present, we provide a concise summary of the literature and clear recommendations on management. Where evidence is lacking, we describe how we practice and provide a rationale for our approach. We also discuss the potential harms associated with MGUS diagnosis, a topic that is rarely, if ever, broached between patients and providers, or even considered in academic debate.
Topics: Aged; Bone Marrow; Bone and Bones; Disease Management; Disease Progression; Female; Humans; Male; Middle Aged; Paraproteinemias
PubMed: 29183887
DOI: 10.1182/blood-2017-09-807560 -
The Chinese Journal of Dental Research 2015To provide an overview of internal organ involvement (IOI) in immunoglobulin G4-related sialadenitis (IgG4-RS) patients, with a focus on the prevalence and clinical... (Review)
Review
OBJECTIVE
To provide an overview of internal organ involvement (IOI) in immunoglobulin G4-related sialadenitis (IgG4-RS) patients, with a focus on the prevalence and clinical features of IOI, the analysis of serum IgG4 levels in patients with or without IOI, and the usefulness of positron emission tomography (PET) for examination of the whole body.
METHODS
A systematic search was performed using PubMed, CNKI, Wanfang Data and CQVIP databases.
RESULTS
A total of 99 articles, including 493 IgG4-RS cases, were analysed in this study. The male-to-female ratio was 1.57:1 and the mean age was 61.67 years. IOI was observed in 71.6% patients, including lesions of the pancreas (38.5%), the biliary system and liver (17.8%), distant lymphadenopathy (20.3%), the respiratory system (15.6%), the urinary system (12.0%) and retroperitoneal fibrosis (11.4%). The lesions could occur homeochronously or metachronously with IgG4-RS. The serum IgG4 levels in the IOI-positive and IOI-negative groups were 1,131 ± 952 mg/dL and 659 ± 843 mg/dL, respectively (P < 0.01). The prevalence of IOI and the number of involved internal organs between the PET and the non-PET groups showed no significant difference (P = 0.399 and P = 0.823, respectively), but were significantly higher in the PET group, amongst patients whose first symptom or chief complaint was salivary gland swelling (P = 0.002 and P = 0.001, respectively).
CONCLUSION
IOI is common in IgG4-RS and almost every organ can be affected. High levels of serum IgG4 represent a potential indicator of IOI. Furthermore, PET is a useful tool for evaluation of the whole body.
Topics: Humans; Autoimmune Diseases; Immunoglobulin G; Paraproteinemias; Positron-Emission Tomography; Sialadenitis; Viscera
PubMed: 26167546
DOI: No ID Found -
Indian Journal of Pathology &... 2023POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis of POEMS syndrome can be a challenge. A good history, physical...
POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis of POEMS syndrome can be a challenge. A good history, physical examination, and appropriate testing can aid in establishing its diagnosis. We are presenting the case of a 75-year-old man who was diagnosed with POEMS syndrome.
Topics: Male; Humans; Aged; POEMS Syndrome; Paraproteinemias
PubMed: 37530355
DOI: 10.4103/ijpm.ijpm_857_21 -
Turkish Journal of Haematology :... Dec 2017Multiple myeloma (MM) is one of the most important clonal malignant plasma cell disorders and renal involvement is associated with poor prognosis. Although there are... (Review)
Review
Multiple myeloma (MM) is one of the most important clonal malignant plasma cell disorders and renal involvement is associated with poor prognosis. Although there are several reasons for renal impairment in MM, the main cause is the toxic effects of monoclonal proteins. Although cast nephropathy is the best known and unchallenged diagnosis for hematologists and pathologists, the renal effects of monoclonal gammopathy can be various. Monoclonal gammopathy of renal significance was proposed by the International Kidney and Monoclonal Gammopathy Research Group for renal lesions in monoclonal gammopathy in recent years. Renal lesions in monoclonal gammopathy can be grouped as follows: light chain (cast) nephropathy, acute tubular injury/necrosis, tubulointerstitial nephritis, amyloidosis, monoclonal Ig deposition diseases, immunotactoid glomerulopathy, type I cryoglobulinemia, proliferative glomerulonephritis with monoclonal IgG deposits, C3 glomerulopathy with monoclonal gammopathy, and crystal-storing histiocytosis, considering the previous and new terminology. In this study, renal involvement of monoclonal gammopathies, in terms of previous and new terminology, was reviewed.
Topics: Amyloidosis; Animals; Humans; Kidney; Kidney Diseases; Multiple Myeloma; Paraproteinemias; Plasma Cells
PubMed: 28832010
DOI: 10.4274/tjh.2017.0197 -
Journal of the American Academy of... Dec 2014
Topics: Aged, 80 and over; Clitoris; Female; Humans; Necrobiotic Xanthogranuloma; Necrosis; Paraproteinemias; Skin Ulcer; Thigh; Vulvar Diseases
PubMed: 25454046
DOI: 10.1016/j.jaad.2014.04.039