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British Medical Journal Feb 1980
Topics: Age Factors; Aged; Bence Jones Protein; Humans; Liver Diseases; Middle Aged; Multiple Myeloma; Paraproteinemias; Prognosis
PubMed: 7357337
DOI: No ID Found -
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 -
Practical Neurology Dec 2021Paraproteinaemic neuropathies comprise a heterogeneous group of neuro-haematological conditions with some distinct neurological, haematological and systemic phenotypes.... (Review)
Review
Paraproteinaemic neuropathies comprise a heterogeneous group of neuro-haematological conditions with some distinct neurological, haematological and systemic phenotypes. The spectrum of disease varies from mild to severe, indolent to rapidly progressive and from small fibre sensory involvement to dramatic sensorimotor deficits. The haematological association may be overlooked, resulting in delayed treatment, disability, impaired quality of life and increased mortality. However, the presence of an irrelevant benign paraprotein can sometimes lead to inappropriate treatment. In this review, we outline our practical approach to paraproteinaemic disorders, discuss the utility and limitations of diagnostic tests and the distinctive clinical phenotypes and touch on the complex multidisciplinary management approaches.
Topics: Humans; Neurology; Paraproteinemias; Peripheral Nervous System Diseases; Quality of Life
PubMed: 34282034
DOI: 10.1136/practneurol-2020-002837 -
Seminars in Nephrology Nov 2022Pathogenic roles of monoclonal immunoglobulins in kidney disease have been attributed previously to malignant plasma cell and lymphoproliferative disorders such as... (Review)
Review
Pathogenic roles of monoclonal immunoglobulins in kidney disease have been attributed previously to malignant plasma cell and lymphoproliferative disorders such as multiple myeloma, lymphoplasmacytic lymphoma, chronic lymphocytic leukemia, or amyloid light chain amyloidosis. Improved technology, advancements in molecular diagnostics, and highly sensitive imaging techniques have established the need to redefine monoclonal gammopathies and the kidney disorders that are associated with monoclonal immunoglobulins regardless of tumor burden. This has led to the establishment of monoclonal gammopathy with renal significance (MGRS). MGRS was defined by the International Kidney and Monoclonal Gammopathy Research Group in 2012 as a clonal proliferative disorder that produces a nephrotoxic monoclonal immunoglobulin and does not meet previously defined hematological criteria for treatment of a specific malignancy. MGRS encompasses a wide array of pathologies with knowledge surrounding its incidence, prognosis, and management continuously increasing. This review examines the current evidence on the diagnosis, prognosis, pathogenesis, and therapy of plasma cell dyscrasias and related MGRS.
Topics: Humans; Paraproteinemias; Kidney Diseases; Kidney; Monoclonal Gammopathy of Undetermined Significance
PubMed: 37257390
DOI: 10.1016/j.semnephrol.2023.151352 -
WMJ : Official Publication of the State... Dec 2007The differential diagnosis for hypophosphatemia is long, and involves complex, overlapping physiological systems. Practitioners are often guilty, however, of simply... (Review)
Review
The differential diagnosis for hypophosphatemia is long, and involves complex, overlapping physiological systems. Practitioners are often guilty, however, of simply supplementing phosphate without fully investigating the etiology of the problem. The purpose of this case presentation is to illustrate a case of spurious hypophosphatemia that initially led to unnecessary phosphate replacement in a woman with undiagnosed multiple myeloma. An 85-year-old African American woman was admitted to the hospital for congestive heart failure exacerbation. The patient was incidentally found to be profoundly hypophosphatemic and was also diagnosed with multiple myeloma at this hospitalization. Normal phosphorus levels were difficult to maintain despite aggressive replacement. A serum sample initially reported to have an abnormally low phosphorus concentration on the Beckman CX7 analyzer was reanalyzed with the Kodak Ektachem 700 system, revealing the phosphorus concentration to be towards the higher limit of the normal range on the same sample. We conclude that clinicians should proceed with caution before aggressively treating abnormal phosphorus levels in patients with known paraproteinemia. Conversely, unexplained phosphorus abnormalities should bring disorders associated with paraproteinemia, such as multiple myeloma, into the differential diagnosis. Knowledge of how various phosphorus assays are affected by paraproteins is essential to guiding diagnosis and treatment. We also review mechanisms of reported interference with common assays.
Topics: Aged, 80 and over; Diabetes Mellitus, Type 2; Diagnosis, Differential; Female; Heart Failure; Humans; Hypertension; Hypophosphatemia; Multiple Myeloma; Paraproteinemias; Phosphorus
PubMed: 18237074
DOI: No ID Found -
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 -
Clinical Laboratory 2015Lymphoplasmacytic lymphoma is a type of B-cell Non-Hodgkin's lymphoma. The monoclonal immunoglobulin of IgA type is rarely seen in the clinical practice.
BACKGROUND
Lymphoplasmacytic lymphoma is a type of B-cell Non-Hodgkin's lymphoma. The monoclonal immunoglobulin of IgA type is rarely seen in the clinical practice.
METHODS
We report a patient with anemia, thrombocytopenia, hepatomegaly, and splenomegaly. Serum immuno-electrophoresis, bone marrow morphology, and flow cytometry assays were used for the diagnosis of this patient.
RESULTS
The monoclonal gammopathy was detected in the serum protein electrophoresis. The serum immunoelectrophoresis was of IgA-λ type and serum IgA level was high. The bone marrow aspiration exhibited a large number of prominent lymphoplasmacytoid lymphocytes. Immunophenotype of lymphoplasmacytoid lymphocytes showed: CD38+, CD138+, CD19+, CD20+. The patient was diagnosed as Lymphoplasmacytic Lymphoma with IgA paraproteinemia.
CONCLUSIONS
For patients whose bone marrow aspiration reveals lymphoplasmacytoid lymphocytes while the monoclonal immunoglobulin is not of IgM type, further diagnosis should be performed to determine the characteristic of the tumor cells, in order to diagnose if it is a rare type of LPL.
Topics: Biomarkers; Biomarkers, Tumor; Biopsy; Bone Marrow Examination; Humans; Immunoelectrophoresis; Immunoglobulin A; Immunophenotyping; Lymphoma, B-Cell; Male; Middle Aged; Paraproteinemias; Predictive Value of Tests
PubMed: 26299085
DOI: 10.7754/clin.lab.2015.150117 -
Cutis May 1996Necrobiotic xanthogranuloma (NXG) is a rare skin disease mimicking necrobiosis lipoidica diabeticorum clinically and histologically. However, its association with...
Necrobiotic xanthogranuloma (NXG) is a rare skin disease mimicking necrobiosis lipoidica diabeticorum clinically and histologically. However, its association with paraproteinemia, and the evolution of hematologic malignancies in some cases, established NXG as a separate clinicopathologic entity. A typical case with indolent course is described.
Topics: Aged; Aged, 80 and over; Biopsy; Collagen; Female; Foam Cells; Giant Cells; Humans; Necrobiosis Lipoidica; Paraproteinemias; Skin; Xanthomatosis
PubMed: 8726711
DOI: No ID Found -
Journal of the American Academy of... Sep 1980Eight patients had multiple xanthomatous plaques and subcutaneous nodules that had a predilection for the periorbital area, flexures, and trunk and that tended to...
Eight patients had multiple xanthomatous plaques and subcutaneous nodules that had a predilection for the periorbital area, flexures, and trunk and that tended to ulcerate. Skin biopsy specimens showed a combination of xanthogranulomatous nodules with necrobiosis. All patients had an accompanying dysproteinemia, which was a monoclonal IgG paraprotein in six. Hyperlipidemia, low serum complement, and cryoglobulinemia were variable features. Five patients had leukopenia. Bone marrow examination showed myeloma in two patients, a lymphoproliferative process in one, and some atypical plasma cells in two. Cutaneous necrobiotic xanthogranuloma is a distinctive histologic pattern most frequently related to plasma cell dyscrasias, and it should be distinguished from normolipemic plane xanthoma and other necrobiotic granulomas.
Topics: Adult; Aged; Antineoplastic Agents; Blood Protein Electrophoresis; Female; Granuloma; Humans; Immunoglobulin G; Male; Middle Aged; Paraproteinemias; Skin; Xanthomatosis
PubMed: 7451693
DOI: 10.1016/s0190-9622(80)80189-7 -
Veterinary Clinical Pathology Dec 2022Hyperglobulinemia is reported in 26% of canine chronic B-cell lymphocytic leukemia (B-CLL) cases. However, few cases have been characterized by protein electrophoresis...
BACKGROUND
Hyperglobulinemia is reported in 26% of canine chronic B-cell lymphocytic leukemia (B-CLL) cases. However, few cases have been characterized by protein electrophoresis and immunofixation (IF), and the incidence of a monoclonal protein (M-protein) is unknown using these techniques.
OBJECTIVE
To characterize and determine the proportion of canine B-CLL cases with an M-protein using plasma protein electrophoresis (PPE), routine and free light chain (fLC) IF, and to assess if productive B-CLL cases express MUM1/IRF4 by cell tube block (CTB).
METHODS
PPE, routine (targeting IgG, IgA, IgM, IgG4, and light chain) and fLC IF were performed using 48 dog B-CLL plasma samples from patients diagnosed via peripheral blood flow cytometry. CTB was performed on a separate cohort of 15 patients.
RESULTS
Hyperproteinemia (>7.5 g/dL) was present in 17/48 cases (35%). An M-protein was detected in 32/48 cases (67%). Of these, 19/32 cases (59%) had only complete (monoclonal heavy and light chain) M-proteins detected, 10/32 cases (31%) had both complete and fLC M-proteins detected, and 3/32 cases (9%) had only an fLC M-protein detected. IgM was the most common clonal immunoglobulin isotype detected (23 cases). CD21 cell counts were higher in cases with detectable M-protein. Plasma fLC IF suggested β-γ region interference, likely caused by clotting proteins. All B-CLL cases consistently expressed PAX5 and did not express MUM1/IRF4.
CONCLUSIONS
Most B-CLL cases had an M-protein and were not hyperproteinemic. Most cases with paraproteins had a complete IgM monoclonal gammopathy; a subset had documented fLCs. The prognostic significance of heavy and fLC presence should be evaluated.
Topics: Dogs; Animals; Leukemia, Lymphocytic, Chronic, B-Cell; Immunoglobulin Light Chains; Immunoelectrophoresis; Paraproteinemias; Immunoglobulin M; Dog Diseases
PubMed: 35883213
DOI: 10.1111/vcp.13156