scholarly journals Monoclonal gammopathies of clinical significance

Hematology ◽  
2020 ◽  
Vol 2020 (1) ◽  
pp. 380-388
Author(s):  
Angela Dispenzieri
Keyword(s):  
Differential Diagnosis ◽  
Clinical Significance ◽  
Monoclonal Gammopathy ◽  
Monoclonal Gammopathies ◽  
Skin Biopsies ◽  
Unexplained Symptoms ◽  
Symptoms And Signs ◽  
Important Disease ◽  
Iv Immunoglobulin ◽  
Undetermined Significance

Abstract “Monoclonal gammopathy of clinical significance” (MGCS) is the term used to describe nonmalignant monoclonal gammopathies causing important disease. MGCS is the differential diagnosis for any patient presenting with what appears to be a monoclonal gammopathy of undetermined significance but is also experiencing other unexplained symptoms. Broadly, these conditions can be separated into symptoms and signs referable to the nerves, the kidneys, and the skin. The first step in making these diagnoses is to consider them. With a particular condition in mind, the next step is to order those tests that can help confirm or dismiss a particular diagnosis. Nearly all of the renal and dermatologic conditions are diagnosed by renal and skin biopsies, respectively. The importance of a highly competent renal pathologist and dermatopathologist cannot be underestimated. Biopsy is less specific for the neuropathic conditions. Because several of the MGCSs are syndromes, recognizing other manifestations is also key. Treatment recommendations for many of these conditions are anecdotal because of their rarity, but for several of the conditions, IV immunoglobulin, rituximab, and plasma cell–directed therapy are the best options.

Differential Diagnosis of Monoclonal Gammopathies

1999 ◽  
Vol 123 (2) ◽  
pp. 108-113 ◽  
Author(s):  
Raymond Alexanian ◽  
Donna Weber ◽  
Frank Liu
Keyword(s):  
Multiple Myeloma ◽  
Differential Diagnosis ◽  
Clinical Features ◽  
Plasma Cell ◽  
Monoclonal Gammopathy ◽  
Monoclonal Immunoglobulin ◽  
Monoclonal Gammopathies ◽  
Plasma Cell Dyscrasias ◽  
Waldenström's Macroglobulinemia ◽  
Undetermined Significance

Abstract Several disorders are associated with a monoclonal immunoglobulin detected by serum or urine electrophoresis, the most common being a monoclonal gammopathy of undetermined significance, multiple myeloma, Waldenström’s macroglobulinemia, and amyloidosis. The clinical features of these conditions, as well as other similar entities, are described in this review. The objective is to demonstrate the importance of electrophoretic studies in the differential diagnosis of plasma cell dyscrasias and in guiding the decision for rational therapies.

scholarly journals Differential diagnosis of monoclonal gammopathy of undetermined significance

Hematology ◽  
2012 ◽  
Vol 2012 (1) ◽  
pp. 595-603 ◽  
Author(s):  
Giampaolo Merlini ◽  
Giovanni Palladini
Keyword(s):  
Differential Diagnosis ◽  
Plasma Cell ◽  
Monoclonal Gammopathy ◽  
Cell Clone ◽  
Organ Damage ◽  
Organ Injury ◽  
Al Amyloidosis ◽  
Monoclonal Protein ◽  
Undetermined Significance

Abstract Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic plasma cell disorder occurring in 4.2% of adults > 50 years of age, which can progress into symptomatic diseases either through proliferation of the plasma cell clone, giving rise to multiple myeloma and other lymphoplasmacellular neoplasms, or through organ damage caused by the monoclonal protein, as seen in light-chain amyloidosis and related conditions. Differential diagnosis of asymptomatic and symptomatic monoclonal gammopathies is the determinant for starting therapy. The criteria for determining end-organ damage should include markers of organ injury caused by the monoclonal protein. Patient assessment and optimal follow-up are now performed using risk stratification models that should also take into account the risk of developing AL amyloidosis. Patients with low-risk MGUS (approximately 40% of all MGUS patients) need limited assessment and very infrequent follow-up. The ongoing development of novel molecular biomarkers and advanced imaging techniques will improve the identification of high-risk patients who may benefit from early therapeutic intervention through innovative clinical trials.

scholarly journals Recommendations for use of Tumor Markers in Monoclonal Gammopathies

2007 ◽  
Vol 26 (2) ◽  
pp. 165-172
Author(s):  
Marijana Dajak
Keyword(s):  
Tumor Markers ◽  
Monoclonal Gammopathy ◽  
Clinical Biochemistry ◽  
M Protein ◽  
Monoclonal Gammopathies ◽  
Undetermined Significance

Preporuke za Primenu Tumorskih Markera Kod Monoklonskih GamapatijaMonoklonske gamapatije čine grupu poremećaja koji se karakterišu klonskom proliferacijom plazma ćelija. M protein je tumorski marker specifičan za monoklonske gamapatije jer odražava klonsku produkciju imunoglobulina. Monoklonske gamapatije uključuju: multipli mijelom,Waldenström-ovu makroglobulinemiju (WM), nesekretorni mijelom, prikriveni (smoldering) multipli mijelom, monoklonsku gamapatiju od neodređenog značaja (MGUS,Monoclonal gammopathy of undetermined significance), primarnu sistemsku amiloidozu i bolest teških lanaca. Dijagnoza multiplog mijeloma je zasnovana na detekciji M proteina u serumu i/ili urinu, infiltraciji plazma ćelija u koštanoj srži i litičkim koštanim lezijama na radiografiji skeleta. Prema NACB (National Academy of Clinical Biochemistry) preporukama, tumorski markeri za dijagnozu,screening, identifikaciju klonaliteta, praćenje bolesti i prognostičku evaluaciju kod monoklonskih gamapatija su: elektroforeza proteina u serumu i/ili urinu, imunofiksacija u serumu i/ili urinu, slobodni laki lanci (SLL) u serumu i/ili urinu, viskoznost seruma i β2-mikroglobulin. Imunofiksacija se koristi za identifikaciju klonaliteta (tipa) M proteina primećenog na elektroforezi i kada postoji sumnja bez obzira na normalan proteinski elektroforetogram. Posebno je korisna za prepoznavanje i razlikovanje biklonskih ili triklonskih gamapatija. Viskoznost seruma trebalo bi određivati ako pacijent ima znake i simptome sindroma hiperviskoznosti. WM je najčeŠći uzrok hiperviskoznosti, ali se takođe može pojaviti i kod pacijenata sa velikim nivoima monoklonskog IgA ili IgG. Automatizovana imunoodređivanja SLL u serumu su osetljivija od tradicionalne elektroforetske metode i imunofiksacije za detekciju mijeloma monoklonskih lakih lanaca, nesekretornog mijeloma i AL amiloidoze. Osim toga, odnos SLL u serumu je nezavisan faktor rizika za nastanak maligne progresije kod pacijenata sa MGUS. Određivanje SLL u serumu i elektroforeze proteina seruma kao testova prve linije za razmatranje prisustva mogućih poremećaja B ćelija daje dodatnu dijagnostičku informaciju.

Serum ß2-Microglobulin lin Patients with Monoclonal Gammopathies

1987 ◽  
Vol 2 (3) ◽  
pp. 169-172 ◽  
Author(s):  
Samuele Di Giovanni ◽  
Gaetano Valentini ◽  
Emma Ravazzolo ◽  
Paolo Carducci ◽  
Paolo Giallonardo ◽  
...  
Keyword(s):  
Monoclonal Gammopathy ◽  
Serum Samples ◽  
Monoclonal Protein ◽  
Β2 Microglobulin ◽  
Monoclonal Gammopathies ◽  
Protein Levels ◽  
Neoplastic Process ◽  
The Mean ◽  
Beta 2 Microglobulin ◽  
Undetermined Significance

Beta-2-microglobulin concentrations were determined in serum samples from 45 patients with benign and malignant monoclonal gammopathies. In the group of patients suffering from multiple myeloma or Waldenström macroglobulinemia the mean β2-microglobulin level was significantly higher than in the group with monoclonal gammopathy of undetermined significance. Values above 3 mg/L were highly indicative of a neoplastic process and were observed in all the Waldenström patients and in > 90% of myeloma patients. No significant correlation was noticed between β2-microglobulin and monoclonal protein levels in any of the groups examined.

scholarly journals Gamapatias Monoclonais de Significado Indeterminado: Critérios de Diagnóstico e Acompanhamento Clínico

2014 ◽  
Vol 27 (5) ◽  
pp. 661
Author(s):  
Joana Parreira ◽  
Paulo Lúcio ◽  
Cristina João ◽  
Ana Macedo ◽  
Ana Bela Sarmento ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multiple Myeloma ◽  
Clinical Management ◽  
Monoclonal Gammopathy ◽  
Long Term Care ◽  
Term Care ◽  
Monoclonal Gammopathies ◽  
Undetermined Significance

<p>The Portuguese group of multiple myeloma of the Portuguese Society of Hematology proposes a national protocol for diagnosis and clinical follow-up of monoclonal gammopathies. The proposed protocol aims to standardize clinical management of monoclonal gammopathies. Furthermore, it would also define the major risk factors for progression to Multiple Myeloma that require a precocious close articulation between general practitioners and a Hematology Clinic.</p><p><br /><strong>Keywords: </strong>Monoclonal Gammopathy of Undetermined Significance; Prognosis; Disease Progression; Long-Term Care; Portugal.</p>

scholarly journals Monoclonal Gammopathy of Undetermined Significance (MGUS)—Not So Asymptomatic after All

Cancers ◽  
2020 ◽  
Vol 12 (6) ◽  
pp. 1554 ◽  
Author(s):  
Oliver C. Lomas ◽  
Tarek H. Mouhieddine ◽  
Sabrin Tahri ◽  
Irene M. Ghobrial
Keyword(s):  
Cardiovascular Disease ◽  
Primary Care ◽  
Multiple Myeloma ◽  
Monoclonal Gammopathy ◽  
Lymphoproliferative Disease ◽  
Current Evidence ◽  
Secondary Immunodeficiency ◽  
Symptoms And Signs ◽  
Undetermined Significance ◽  
Morbid Conditions

Monoclonal Gammopathy of Undetermined Significance (MGUS) is considered to be a benign precursor condition that may progress to a lymphoproliferative disease or multiple myeloma. Most patients do not progress to an overt condition, but nevertheless, MGUS is associated with a shortened life expectancy and, in a minority of cases, a number of co-morbid conditions that include an increased fracture risk, renal impairment, peripheral neuropathy, secondary immunodeficiency, and cardiovascular disease. This review aims to consolidate current evidence for the significance of these co-morbidities before considering how best to approach these symptoms and signs, which are often encountered in primary care or within a number of specialties in secondary care.

scholarly journals Advances in the understanding of IgM monoclonal gammopathy of undetermined significance

F1000Research ◽  
2017 ◽  
Vol 6 ◽  
pp. 2142 ◽  
Author(s):  
Jonas Paludo ◽  
Stephen M Ansell
Keyword(s):  
B Cell ◽  
Monoclonal Gammopathy ◽  
Immunoglobulin M ◽  
The Other ◽  
Pivotal Role ◽  
Monoclonal Gammopathies ◽  
Waldenstrom Macroglobulinemia ◽  
Recent Advances ◽  
Malignant Condition ◽  
Undetermined Significance

Among monoclonal gammopathies of undetermined significance (MGUSs), the immunoglobulin M (IgM) MGUS subtype stands as a unique entity and plays a pivotal role as a pre-malignant condition for multiple B-cell non-Hodgkin lymphomas, most notably Waldenström macroglobulinemia (WM). A relationship between IgM MGUS and WM has been proposed for decades. However, insight regarding the pathobiology of these two conditions improved significantly in recent years, strengthening the hypothesis that WM and IgM MGUS are different stages of the same disease. Therefore, the understanding of IgM MGUS and that of WM are interconnected and advances in one will likely impact the other. Furthermore, IgM MGUS has been more commonly recognized as the underlying etiology of IgM-related disorders. In this review, we explore recent advances in the understanding of the pathobiology of IgM MGUS and WM and the treatment of common IgM-related disorders.

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