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Clinics in Geriatric Medicine May 2021This article provides an overview of the clinical features, diagnosis, and treatment of the major paraprotein-related peripheral neuropathies, including monoclonal... (Review)
Review
This article provides an overview of the clinical features, diagnosis, and treatment of the major paraprotein-related peripheral neuropathies, including monoclonal gammopathy of undetermined significance, Waldenström macroglobulinemia, POEMS syndrome, multiple myeloma, transthyretin amyloidosis, and light chain amyloidosis. For each paraprotein neuropathy, the epidemiology, demographics, systemic findings, and electrophysiologic features are presented. Pharmacologic treatment of transthyretin amyloid polyneuropathy also is reviewed.
Topics: Aged; Humans; Monoclonal Gammopathy of Undetermined Significance; Paraproteinemias; Peripheral Nervous System Diseases; Waldenstrom Macroglobulinemia
PubMed: 33858612
DOI: 10.1016/j.cger.2021.01.004 -
Annals of Allergy, Asthma & Immunology... Jan 2019
Review
Topics: Blood Proteins; Electrophoresis; Humans; Immunoglobulin Heavy Chains; Immunoglobulin Light Chains; Inflammation; Paraproteinemias
PubMed: 30579431
DOI: 10.1016/j.anai.2018.08.004 -
The Veterinary Clinics of North... Jan 2023This review provides current information on myeloma-related disorders, a group of plasma cell or immunoglobulin (Ig) secreting neoplasms including multiple myeloma,... (Review)
Review
This review provides current information on myeloma-related disorders, a group of plasma cell or immunoglobulin (Ig) secreting neoplasms including multiple myeloma, extramedullary plasmacytoma (both cutaneous and noncutaneous variants), solitary osseous plasmacytoma, Waldenström macroglobulinemia/lymphoplasmacytic lymphoma, Ig-secretory B-cell lymphoma, plasma cell leukemia, and monoclonal gammopathy of undetermined significance. The diagnostic procedures commonly used to characterize myeloma-related disorders, including cytopathology, histopathology, polymerase chain reaction for antigen receptor rearrangement, flow cytometry, and electrophoretic techniques are outlined and discussed.
Topics: Animals; Multiple Myeloma; Plasmacytoma; Paraproteinemias; Waldenstrom Macroglobulinemia
PubMed: 36270842
DOI: 10.1016/j.cvsm.2022.07.009 -
Blood Apr 2023
Topics: Humans; Monoclonal Gammopathy of Undetermined Significance; Paraproteinemias; Thrombosis
PubMed: 36626584
DOI: 10.1182/blood.2022018797 -
European Journal of Haematology Jan 2021Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare condition defined by monoclonal plasma cell disorder,... (Review)
Review
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare condition defined by monoclonal plasma cell disorder, peripheral neuropathy, and other systemic symptoms. The pathophysiology of POEMS syndrome is unknown, but the overproduction of vascular endothelial growth factor (VEGF) appears to be an important contributory element. The diagnosis of POEMS syndrome requires the presence of both mandatory criteria (ie, polyneuropathy and a monoclonal plasma cell disorder), at least one major criterion (ie, osteosclerotic bone lesions, Castleman disease, or elevated serum or plasma levels of vascular endothelial growth factor), and at least one of the six minor criteria. POEMS syndrome lacks a standard treatment, but patients with limited sclerotic bone lesions are typically treated with radiation therapy. In contrast, those with widespread lesions receive chemotherapy and hematopoietic stem cell transplantation.
Topics: Combined Modality Therapy; Disease Management; Disease Susceptibility; Humans; Organ Specificity; POEMS Syndrome; Phenotype; Prognosis
PubMed: 32889731
DOI: 10.1111/ejh.13514 -
Clinical Journal of the American... Jan 2018Dysproteinemia is characterized by the overproduction of an Ig by clonal expansion of cells from the B cell lineage. The resultant monoclonal protein can be composed of... (Review)
Review
Dysproteinemia is characterized by the overproduction of an Ig by clonal expansion of cells from the B cell lineage. The resultant monoclonal protein can be composed of the entire Ig or its components. Monoclonal proteins are increasingly recognized as a contributor to kidney disease. They can cause injury in all areas of the kidney, including the glomerular, tubular, and vascular compartments. In the glomerulus, the major mechanism of injury is deposition. Examples of this include Ig amyloidosis, monoclonal Ig deposition disease, immunotactoid glomerulopathy, and cryoglobulinemic GN specifically from types 1 and 2 cryoglobulins. Mechanisms that do not involve Ig deposition include the activation of the complement system, which causes complement deposition in C3 glomerulopathy, and cytokines/growth factors as seen in thrombotic microangiopathy and precipitation, which is involved with cryoglobulinemia. It is important to recognize that nephrotoxic monoclonal proteins can be produced by clones from any of the B cell lineages and that a malignant state is not required for the development of kidney disease. The nephrotoxic clones that do not meet requirement for a malignant condition are now called monoclonal gammopathy of renal significance. Whether it is a malignancy or monoclonal gammopathy of renal significance, preservation of renal function requires substantial reduction of the monoclonal protein. With better understanding of the pathogenesis, clone-directed strategies, such as rituximab against CD20 expressing B cell and bortezomib against plasma cell clones, have been used in the treatment of these diseases. These clone-directed therapies been found to be more effective than immunosuppressive regimens used in nonmonoclonal protein-related kidney diseases.
Topics: Animals; Autoimmunity; B-Lymphocytes; Cell Lineage; Complement Activation; Cytotoxicity, Immunologic; Humans; Immunoglobulin G; Kidney Diseases; Kidney Glomerulus; Paraproteinemias; Prognosis; Risk Factors
PubMed: 29114004
DOI: 10.2215/CJN.00560117 -
European Journal of Ophthalmology Sep 2023Plasma cell dyscrasias are a wide range of severe monoclonal gammopathies caused by pre-malignant or malignant plasma cells that over-secrete an abnormal monoclonal... (Review)
Review
Plasma cell dyscrasias are a wide range of severe monoclonal gammopathies caused by pre-malignant or malignant plasma cells that over-secrete an abnormal monoclonal antibody. These disorders are associated with various systemic findings, including ophthalmological disorders. A search of PubMed, EMBASE, Scopus and Cochrane databases was performed in March 2021 to examine evidence pertaining to ocular complications in patients diagnosed with plasma cell dyscrasias. This review outlines the ocular complications associated with smoldering multiple myeloma and monoclonal gammopathy of undetermined significance, plasmacytomas, multiple myeloma, Waldenström's macroglobulinemia, systemic amyloidosis, Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes (POEMS) syndrome, and cryoglobulinemia. Although, the pathological mechanisms are not completely elucidated yet, wide-ranging ocular presentations have been identified over the years, evolving both the anterior and posterior segments of the eye. Moreover, the presenting symptoms also help in early diagnosis in asymptomatic patients. Therefore, it is imperative for the treating ophthalmologist and oncologist to maintain a high clinical suspicion for identifying the ophthalmological signs and diagnosing the underlying disease, preventing its progression through efficacious treatment strategies.
Topics: Humans; Paraproteinemias; Eye; Eye Diseases; Treatment Outcome
PubMed: 36760117
DOI: 10.1177/11206721231155974 -
Revista Medica de Chile Aug 2019Hematological neoplasms are tumors of cells in different states of maturation and differentiation. Since monoclonal gammopathies (MG) refer to B mature lymphocyte... (Review)
Review
Hematological neoplasms are tumors of cells in different states of maturation and differentiation. Since monoclonal gammopathies (MG) refer to B mature lymphocyte neoplasms, lymphogenesis should be well known. We must keep in mind that the last stage of maturation of these lymphocytes is the plasma cell. This is how a MG could appear in the context of a plasma cell neoplasm, such as multiple myeloma or amyloidosis, but also in relation to a lymphoma. A monoclonal peak is produced by mature B lymphocytes or plasma cells that secrete a monoclonal protein (Immunoglobulin), and represents a MG. But it must be emphasized that, in the correct clinical context, a hypogammaglobulinemia can represent a MG as well. Another important point is the understanding and interpretation of requested tests, such as protein electrophoresis (PEP), immunofixation (IFx) or serum free light chains (sFLC). The current MG screening panel includes these three studies (PEF, IFx, sFLC), although a simpler panel measuring PEF and sFLC has also been proposed, but not yet formally validated. Therefore, screening done only with PEP is insufficient.
Topics: B-Lymphocytes; Blood Protein Electrophoresis; Humans; Neoplasms, Plasma Cell; Paraproteinemias; Paraproteins
PubMed: 31859969
DOI: 10.4067/S0034-98872019000801036 -
Deutsche Medizinische Wochenschrift... Jun 2022Monoclonal gammopathies are a frequently diagnosed entity. However, the diagnosis is not always clinically relevant. The diagnosis of a monoclonal gammopathy requires... (Review)
Review
Monoclonal gammopathies are a frequently diagnosed entity. However, the diagnosis is not always clinically relevant. The diagnosis of a monoclonal gammopathy requires serum electrophoresis, immunofixation and free light chain measurement. Sometimes, monoclonal gammopathies occur in the course of transient or autoimmune inflammation. Further diagnostics should only be performed after risk assessment according to Mayo criteria. In non-low risk patients, a symptomatic myeloma has to be ruled out via SLiM-CRAB criteria. The diagnostic work-up should include whole-body MRI and a bone marrow puncture as well as a 24 h urine sample. If it does not imply myeloma, the diagnosis of MGUS is confirmed and a follow-up after 6 months is recommended. After that, low-risk patients only need SLiM-CRAB screening at clinical signs of progression. All other patients should receive serologic follow-ups once a year. Importantly, MGUS patients show higher morbidity. Amongst a higher prevalence of osteoporosis and immunodeficiency, a wide array of MGUS-associated diseases such as AL amyloidosis, deposition diseases and Fc binding-dependent effects can occur. This article gives an overview over the work-up, observation and caveats of monoclonal gammopathy of (un)known significance.
Topics: Humans; Immunoglobulin Light Chains; Immunoglobulin Light-chain Amyloidosis; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Paraproteinemias
PubMed: 35636418
DOI: 10.1055/a-1664-7353 -
European Journal of Haematology Apr 2018Electronic consultation (e-consult) is an important component of care for patients in the Veterans Health Administration who require subspecialty consultation but not... (Review)
Review
IMPORTANCE
Electronic consultation (e-consult) is an important component of care for patients in the Veterans Health Administration who require subspecialty consultation but not urgent face-to-face evaluation. Monoclonal gammopathy of undetermined significance (MGUS) is a common reason for e-consult. While often benign, MGUS requires careful evaluation and persistent surveillance over time.
OBJECTIVE
To identify areas to improve MGUS care delivery by e-consult.
METHODS
We performed a retrospective review of our e-consult database and identified a cohort of 152 MGUS patients triaged for e-consult over a 5-year period (2010-2014).
RESULTS
The median time to completion of an e-consult was 2 days. Ninety-six percent of MGUS e-consults had a hemoglobin >10 g/dL, and 90% had a creatinine <2 mg/dL. While the majority of e-consults were low risk, paraprotein surveillance varied over time and tracked with consult utilization. With a median follow-up of 44 months, there were 6 documented progression events, representing a mean rate of progression of 1% per year.
CONCLUSIONS
E-consult is a helpful mechanism for the evaluation of MGUS, reducing the need for outpatient appointments. However, timely risk stratification and persistent surveillance over time are critical for e-consult to work well.
Topics: Aged; Biomarkers; Biopsy; Delivery of Health Care; Disease Management; Electronic Health Records; Female; Follow-Up Studies; Hematologic Tests; Humans; Male; Middle Aged; Monoclonal Gammopathy of Undetermined Significance; Paraproteinemias; Quality Improvement; Remote Consultation; Retrospective Studies; Risk Assessment
PubMed: 29281132
DOI: 10.1111/ejh.13019