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 Skin: mirror of marrow- case of POEMS syndrome

2017 ◽  
Vol 3 (2) ◽  
pp. 286
Author(s):  
R. B. Chavan ◽  
V. A. Belgaumkar ◽  
A. S. Salunke ◽  
S. S. Tharewal ◽  
N. M. Bansal
Keyword(s):  
B Cells ◽  
Plasma Cell ◽  
Monoclonal Gammopathy ◽  
Poems Syndrome ◽  
Skin Disorders ◽  
Monoclonal Immunoglobulin ◽  
Skin Changes ◽  
Monoclonal Gammopathies ◽  
Clonal Proliferation ◽  
Plasma Cell Dyscrasias

<p class="abstract"><span lang="EN-IN">Monoclonal gammopathy is clonal proliferation and accumulation of immunoglobulin producing B-cells. A variety of skin disorders are associated with an increased level of monoclonal immunoglobulin proteins. Synonyms such as monoclonal gammopathies, paraproteinemias, plasma cell dyscrasias and dysproteinemias are used to designate gammaglobinopathies. Here in we report a case of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome presenting with sclerodermoid features.</span></p>

scholarly journals Ig VH gene mutational patterns indicate different tumor cell status in human myeloma and monoclonal gammopathy of undetermined significance

Blood ◽  
1996 ◽  
Vol 87 (2) ◽  
pp. 746-755 ◽  
Author(s):  
SS Sahota ◽  
R Leo ◽  
TJ Hamblin ◽  
FK Stevenson
Keyword(s):  
Multiple Myeloma ◽  
Tumor Cell ◽  
Plasma Cell ◽  
Monoclonal Gammopathy ◽  
Somatic Hypermutation ◽  
Wide Spectrum ◽  
Malignant Potential ◽  
Gene Sequences ◽  
Vh Gene ◽  
Undetermined Significance

Plasma cell tumors display a wide spectrum of clinical progression, ranging from aggressive multiple myeloma to a benign form known as monoclonal gammopathy of undetermined significance (MGUS), which requires no treatment. Because both diseases involve mature Ig- secreting plasma cells, the reason for this variation in malignant behavior is unclear. However, assessment of malignant potential is desirable for choice of treatment protocols. Ig variable (VH) gene sequences analysis has previously shown the tumor cell of multiple myeloma to be postfollicular, with mutated homogeneous clonal sequences indicating no continuing exposure to the somatic hypermutation mechanism, and this was confirmed in 7 of 7 patients. Comparison of the VH gene sequences in the monoclonal cells in MGUS yielded a different result, with 3 of 7 patients demonstrating mutated heterogeneous sequences consistent with the tumor cells remaining under the influence of the mutator. In 1 of 3 of these patients, an IgM-positive precursor cell was identified that expressed heterogeneous VH sequences similar to those of the isotype-switched plasma cell. These results indicate that the clonal cells in MGUS differ from those in myeloma and suggest that the difference may reflect malignant potential.

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.

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 Comparative Study of Immune Status to Infectious Agents in Elderly Patients with Multiple Myeloma, Waldenstrom's Macroglobulinemia, and Monoclonal Gammopathy of Undetermined Significance

2011 ◽  
Vol 18 (6) ◽  
pp. 969-977 ◽  
Author(s):  
Johanna Karlsson ◽  
Björn Andréasson ◽  
Nahid Kondori ◽  
Evelina Erman ◽  
Kristian Riesbeck ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Elderly Patients ◽  
Humoral Immunity ◽  
Monoclonal Gammopathy ◽  
Study Groups ◽  
Waldenström’S Macroglobulinemia ◽  
Content Type ◽  
Waldenstrom's Macroglobulinemia ◽  
Waldenström's Macroglobulinemia ◽  
Undetermined Significance

ABSTRACTWhereas patients with multiple myeloma (MM) have a well-documented susceptibility to infections, this has been less studied in other B-cell disorders, such as Waldenstrom's macroglobulinemia (WM) and monoclonal gammopathy of undetermined significance (MGUS). We investigated the humoral immunity to 24 different pathogens in elderly patients with MM (n= 25), WM (n= 16), and MGUS (n= 18) and in age-matched controls (n= 20). Antibody titers against pneumococci, staphylococcal alpha-toxin, tetanus and diphtheria toxoids, and varicella, mumps, and rubella viruses were most depressed in MM patients, next to lowest in WM and MGUS patients, and highest in the controls. In contrast, levels of antibodies specific for staphylococcal teichoic acid,Moraxella catarrhalis, candida, aspergillus, and measles virus were similarly decreased in MM and MGUS patients. Comparable titers in all study groups were seen againstHaemophilus influenzaetype b (Hib), borrelia, toxoplasma, and members of the herpesvirus family. Finally, a uniform lack of antibodies was noted againstStreptococcus pyogenes, salmonella, yersinia, brucella, francisella, and herpes simplex virus type 2. To conclude, although MM patients displayed the most depressed humoral immunity, significantly decreased antibody levels were also evident in patients with WM and MGUS, particularly againstStaphylococcus aureus, pneumococci, and varicella. Conversely, immunity was retained for Hib and certain herpesviruses in all study groups.

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