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Multiple Myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
This is a rare syndrome in which a serum paraprotein (M-component) is associated with polyneuropathy (P), organomegaly (O), endocrinopathy (E), and skin changes (S). Approximately 50% of cases are associated with multiple myeloma and the remainder with solitary plasmacytomas or more subtle plasma cell dyscrasias. The cardinal feature is a severe sensorimotor neuropathy with osteosclerotic bone lesions, hepatomegaly and lymphadenopathy, hormonal abnormalities, and skin hyperpigmentation. Treatment is difficult, but local radiotherapy for localized plasmacytomas and chemotherapy for generalized disease are recommended. There are now a number of reports of neurological improvement or stabilization following high-dose therapy and stem-cell transplantation.
B Cells and Humoral Immunity
Published in Constantin A. Bona, Francisco A. Bonilla, Textbook of Immunology, 2019
Constantin A. Bona, Francisco A. Bonilla
There are three major monoclonal gammopathies: multiple myeloma, Waldenstrom’s macro globulinemia, and the heavy chain diseases. These conditions are also referred to as plasma cell dyscrasias.
Lymphocyte and plasma cell malignancies
Published in Gabriel Virella, Medical Immunology, 2019
Juan Carlos Varela, Gabriel Virella
Plasma cell dyscrasias include three main diseases: MGUS, smoldering myeloma, and multiple myeloma. Each of these diseases is associated with the presence of monoclonal paraproteins and plasma cells in the bone marrow, but only multiple myeloma is associated with end-organ damage as described later.
Identification of AL proteins from 10 λ-AL amyloidosis patients by mass spectrometry extracted from abdominal fat and heart tissue
Published in Amyloid, 2023
Julian Baur, Natalie Berghaus, Sarah Schreiner, Ute Hegenbart, Stefan O. Schönland, Sebastian Wiese, Stefanie Huhn, Christian Haupt
The formation and deposition of fibrils derived from immunoglobulin light chains (LC) is a hallmark of AL amyloidosis [1]. It is the most common form of systemic amyloidosis in industrialised countries and usually results from an underlying plasma cell dyscrasia in which malignant plasma cells overproduce a monoclonal LC [2]. The annual incidence is approximately 10 new patients per 1 million population in the Western world [2]. The clinical and pathological disease manifestations are diverse and AL amyloid deposits can be found in different tissues and organs, but frequently occur in the heart and kidneys [2]. Many patients present with non-specific symptoms, leading to late diagnosis of the disease. For example, once symptoms of heart failure appear, the prognosis is poor and the median survival rate is < 7 months in patients with advanced cardiac involvement [3].
Kidney-limited AL amyloidosis: a case report and review of the literature
Published in Journal of Community Hospital Internal Medicine Perspectives, 2021
Sara Velayati, Alexander Belkin, Gurwinder Sidhu Kumar, Zubin J Tharayil, Neeru Kumar, Samir Patel
AL systemic amyloidosis occurs as a result of abnormal light chain protein deposition, of which 80% are associated with the Lambda (λ) chain and involve almost all vital organs. The heart and kidneys, with 82% and 68% of cases reported, respectively, are the most common sites of deposition. Additionally, the liver, nervous system, and gastrointestinal tract are often involved, and multiple organ systems are frequently affected [5]. Localized amyloidosis is rare but has been reported in the upper airways, orbits, urinary tracts, skin, and nails; this almost never progresses to systemic disease. Amyloidosis can also be accompanied by other plasma cell dyscrasias such as multiple myeloma (MM) or monoclonal gammopathy of undetermined significance (MGUS); these should be evaluated and excluded at the time of diagnosis. As noted, systemic disease commonly involves multiple organs, and there is a predilection for one organ to be affected more than others. The kidneys are frequently involved in systemic amyloidosis, but it is extremely rare for the kidneys to be the sole organ involved in the disease process [6].
C3 glomerulonephritis associated with monoclonal gammopathy: a retrospective case series study from a single institute in China
Published in Renal Failure, 2021
Xin Zhang, Xiao-Juan Yu, Dan-yang Li, Su-xia Wang, Fu-de Zhou, Ming-hui Zhao
Monoclonal gammopathy, often associated with renal disorder, consists of a heterogeneous group of diseases characterized by the abnormal clonal proliferation of Ig-producing B-lymphocytes or plasma cells, including classic malignancies such as multiple myeloma and Waldenström macroglobulinemia; and the premalignant plasma cell dyscrasia termed MGUS (monoclonal gammopathy of undetermined significance) [2]. The terminology MGRS (monoclonal gammopathy of renal significance) is introduced to describe the clonal proliferative disorder that produces a nephrotoxic monoclonal Ig and does not meet previously defined hematological criteria for treatment of a specific malignancy [3,4]. Occasionally, C3G is accompanied by monoclonal gammopathy, which proposes that monoclonal immunoglobulins might cause kidney injury indirectly through interfering AP [5–9]. Monoclonal λ-dimer functioning as anti-CFH autoantibody has also been reported [10]. The studies describing C3G patients with monoclonal gammopathy [5,6,9,11,12] show chemotherapy could improve most patients’ outcomes. However, as far as we know, there is no study describing the characteristics of Chinese patients of C3GN with monoclonal gammopathy. In this retrospective study, we report in detail 19 Chinese patients of C3GN combined with monoclonal Ig in serum and (or) urine, we also review the clinicopathological features, complement abnormalities, treatment, and follow-up of these patients.