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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 -
Clinical Chemistry and Laboratory... Jun 2016Monoclonal immunoglobulins are markers of plasma cell proliferative diseases and have been described as the first (and perhaps best) serological tumor marker. The unique... (Review)
Review
Monoclonal immunoglobulins are markers of plasma cell proliferative diseases and have been described as the first (and perhaps best) serological tumor marker. The unique structure of each monoclonal protein makes them highly specific for each plasma cell clone. The difficulties of using monoclonal proteins for diagnosing and monitoring multiple myeloma, however, stem from the diverse disease presentations and broad range of serum protein concentrations and molecular weights. Because of these challenges, no single test can confidently diagnose or monitor all patients. Panels of tests have been recommended for sensitivity and efficiency. In this review we discuss the various disease presentations and the use of various tests such as protein electrophoresis and immunofixation electrophoresis as well as immunoglobulin quantitation, free light chain quantitation, and heavy-light chain quantitation by immuno-nephelometry. The choice of tests for inclusion in diagnostic and monitoring panels may need to be tailored to each patient, and examples are provided. The panel currently recommended for diagnostic screening is serum protein electrophoresis, immunofixation electrophoresis, and free light chain quantitation.
Topics: Bence Jones Protein; Biomarkers, Tumor; Blood Protein Electrophoresis; Blood Viscosity; Cryoglobulins; Humans; Immunoelectrophoresis; Immunoglobulin Light Chains; Multiple Myeloma; Myeloma Proteins; Paraproteinemias; Practice Guidelines as Topic
PubMed: 26509779
DOI: 10.1515/cclm-2015-0580 -
European Review For Medical and... Sep 2022Bence Jones proteinuria (BJP) refers to monoclonal free immunoglobulin light chains detected in urine, deriving from the clonal expansion of plasma cells in the bone... (Review)
Review
OBJECTIVE
Bence Jones proteinuria (BJP) refers to monoclonal free immunoglobulin light chains detected in urine, deriving from the clonal expansion of plasma cells in the bone marrow in patients with plasma cell dyscrasias, associated with monoclonal gammopathies of uncertain origin. This review summarizes routinely diagnostic procedures to assess BJP highlighting critical steps of pre-analytical, analytical, and post-analytical phases.
QUALITATIVE AND QUANTITATIVE METHODS
The best option for BJP detection is the first morning void urine sample and immunofixation electrophoresis detection technique (IFE) the recommended method, with the employment of specific polyvalent antisera. Other qualitative tests for a quick evaluation of BJP are currently available. Densitometric analysis performed on the 24-hour urine is the recommended method to quantify BJP. To overcome the 24-hour collection, it is possible to use morning urine sample and correlate the assessed value of BJP to creatininuria. In addition to the traditional ones, we here reviewed screening methods currently used to avoid false negatives and reduce the time around test (TAT), together with immunochemical quantification methods for increased sensitivity, after checking BJP by IFE. Mass spectrometry emerges as a new challenge in the determination of BJP.
CONCLUSIONS
The employment of different based-assays methods may be useful for diagnostic purposes to improve the accuracy of BJP monitoring in monoclonal gammopathies.
Topics: Bence Jones Protein; Humans; Immune Sera; Immunoglobulin Light Chains; Neoplasms; Paraproteinemias; Proteinuria
PubMed: 36196726
DOI: 10.26355/eurrev_202209_29779 -
Hematology. American Society of... Dec 2020"Monoclonal gammopathy of clinical significance" (MGCS) is the term used to describe nonmalignant monoclonal gammopathies causing important disease. MGCS is the...
"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.
Topics: Aged; Biopsy; Disease Management; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Kidney; Male; Paraproteinemias; Rituximab; Skin
PubMed: 33275738
DOI: 10.1182/hematology.2020000122 -
International Journal of Molecular... May 2021Vitamin D is a steroid hormone that is essential for bone mineral metabolism and it has several other effects in the body, including anti-cancer actions. Vitamin D... (Review)
Review
Vitamin D is a steroid hormone that is essential for bone mineral metabolism and it has several other effects in the body, including anti-cancer actions. Vitamin D causes a reduction in cell growth by interrupting the cell cycle. Moreover, the active form of vitamin D, i.e., 1,25-dihydroxyvitamin D, exerts various effects via its interaction with the vitamin D receptor on the innate and adaptive immune system, which could be relevant in the onset of tumors. Multiple myeloma is a treatable but incurable malignancy characterized by the growth of clonal plasma cells in protective niches in the bone marrow. In patients affected by multiple myeloma, vitamin D deficiency is commonly correlated with an advanced stage of the disease, greater risk of progression, the development of pathological fractures, and a worse prognosis. Changes in the vitamin D receptor often contribute to the occurrence and progress of deficiencies, which can be overcome by supplementation with vitamin D or analogues. However, in spite of the findings available in the literature, there is no clear standard of care and clinical practice varies. Further research is needed to better understand how vitamin D influences outcomes in patients with monoclonal gammopathies.
Topics: Animals; Disease Progression; Humans; Immune System; Models, Biological; Paraproteinemias; Risk Factors; Vitamin D
PubMed: 34066482
DOI: 10.3390/ijms22094922 -
British Journal of Haematology Jun 2016Pure red cell aplasia (PRCA) is a rare disorder characterized by inhibition of erythroid precursors in the bone marrow and normochromic, normocytic anaemia with...
Pure red cell aplasia (PRCA) is a rare disorder characterized by inhibition of erythroid precursors in the bone marrow and normochromic, normocytic anaemia with reticulocytopenia. Among 51 PRCA patients, we identified 12 (24%) patients having monoclonal gammopathy, monoclonal gammopathy of undetermined significance or smouldering multiple myeloma, with presence of monoclonal protein or abnormal serum free light chains and atypical bone marrow features of clonal plasmacytosis, hypercellularity and fibrosis. Thus far, three patients treated with anti-myeloma based therapeutics have responded with reticulocyte recovery and clinical transfusion independence, suggesting plasma cells play a key role in the pathogenesis of this specific monoclonal gammopathy-associated PRCA.
Topics: Adult; Aged; Bone Marrow; Dexamethasone; Diagnosis, Differential; Female; Humans; Immunoglobulins; Lenalidomide; Male; Middle Aged; Multiple Myeloma; Paraproteinemias; Plasma Cells; Red-Cell Aplasia, Pure; Reticulocyte Count; Reticulocytes; Thalidomide; Young Adult
PubMed: 26999424
DOI: 10.1111/bjh.14012 -
Blood Advances Jul 2020Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) syndrome is associated with increased risk for ischemic stroke...
Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) syndrome is associated with increased risk for ischemic stroke (IS). Because POEMS syndrome is rare, little is known regarding the underlying mechanism and prognosis for IS in patients in whom it occurs. The medical records of patients with POEMS syndrome were screened between January 2018 and January 2000 at Peking Union Medical College Hospital to identify those with IS. The baseline characteristics, IS features, and patient outcomes were analyzed. Forty-one (8.0%) of 510 POEMS patients were documented to have IS. Patients with IS were older, had a higher percentage of Overall Neuropathy Limitation Scale score >4, and had a higher level of fibrinogen compared with those who did not have IS. Ninety-three percent of IS events occurred before or within 3 months after a diagnosis of POEMS. Of 41 occurrences of IS, 29 (46.3%) were multifocal. Recurrent IS was observed in 13 (31.7%) of 41 patients, but not in patients with successful anti-plasma cell therapy. The 3-year overall survival rate in patients with IS was 71.0% and for those without IS, it was 88.5% (P = .002). We showed that 8.0% of POEMS patients had IS, and most IS events occurred in POEMS patients not being treated effectively. Having IS was a predictor of unfavorable prognosis. Early diagnosis, immediate initiation of treatment for POEMS, and control of POEMS syndrome is key to reducing the occurrence of IS, improving survival, and preventing recurrence of IS.
Topics: Brain Ischemia; Humans; Ischemic Stroke; POEMS Syndrome; Paraproteinemias; Stroke
PubMed: 32722780
DOI: 10.1182/bloodadvances.2020001865 -
American Society of Clinical Oncology... 2016Multiple myeloma (MM) is an incurable disease that progresses from a premalignant stage termed monoclonal gammopathy of undetermined significance (MGUS) and an... (Review)
Review
Multiple myeloma (MM) is an incurable disease that progresses from a premalignant stage termed monoclonal gammopathy of undetermined significance (MGUS) and an intermediate stage of smoldering multiple myeloma (SMM). Recent major advances in therapy with more effective and less toxic treatments have brought reconsideration of early therapeutic intervention in management of SMM, with the goal of reducing progression of the disease before the occurrence of end-organ damage to MM and improving survival. Key to this effort is accurate identification of patients at high risk of progression who would truly benefit from early intervention. In this review, we discuss the current definitions, risk factors, risk stratification, prognosis, and management of MGUS and SMM, as well as new emerging therapeutic options under active investigation.
Topics: Disease Management; Disease Progression; Humans; Multiple Myeloma; Neoplasm Staging; Paraproteinemias; Plasma Cells; Prognosis; Risk Factors
PubMed: 27249747
DOI: 10.1200/EDBK_159010 -
Hellenic Journal of Nuclear Medicine 2023Plasma cell disorders are a heterogeneous group caused by the monoclonal proliferation of lymphoplasmacytic cells in the bone marrow. Multiple Myeloma (MM) is the most...
Plasma cell disorders are a heterogeneous group caused by the monoclonal proliferation of lymphoplasmacytic cells in the bone marrow. Multiple Myeloma (MM) is the most serious and prevalent plasma cell dyscrasia, with a median age of onset of 60 years.MM displays significant genetic, biological and clinical heterogeneity with subsequent imaging heterogeneity, evident in contemporary imaging modalities (PET/CT and MRI). Evidence suggests that MM is always preceded by precursor stages of monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma.
Topics: Humans; Middle Aged; Positron Emission Tomography Computed Tomography; Fluorodeoxyglucose F18; Plasma Cells; Paraproteinemias; Multiple Myeloma; Disease Progression
PubMed: 37658561
DOI: No ID Found -
Indian Journal of Ophthalmology Aug 2020
Topics: Corneal Diseases; Humans; Multimodal Imaging; Paraproteinemias; Vision Disorders
PubMed: 32709812
DOI: 10.4103/ijo.IJO_2029_19