Spleen
Wojciech Gorczyca in Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
SMZL is a low-grade B-cell lymphoma involving splenic white pulp with focal infiltration of the red pulp, usually involving also the splenic hilar lymph nodes, bone marrow (BM), and blood [1, 10–14, 21, 22]. SMZL is a disease of the elderly with median age at presentation of 65 years (range 30–90 years). The disease presents as an incidental finding or with symptoms of splenic enlargement, cytopenia (most often anemia), and lymphocytosis. The presence of circulating villous lymphocytes defines splenic lymphoma with villous lymphocytes, which is considered the leukemic counterpart of SMZL. Tumor cells in the blood are detected in 57%–68% of cases [23, 24]. The majority of cases have BM involvement and roughly one-third have liver involvement. Serum paraproteinemia is observed in 10%–28% of cases.
Rheology of Paraproteinemias and Leukemias
Gordon D. O. Lowe in Clinical Blood Rheology, 2019
The paraproteinemias (otherwise known as plasma cell or lymphoid dyscrasias) are uncommon disorders, whose main feature is malignant proliferation of a single clone of B-lymphoid cells, which secrete an immunoglobulin (Ig) in excessive quantities. This monoclonal globulin (M-protein) appears as a narrow-based, prominent “spike” on serum protein electrophoresis. The abnormal protein can be characterized by immunological techniques, identifying its category (IgG, IgA, IgM, IgD, or IgE; free light-chains, or heavy-chain fragments).1 The most common of these disorders is multiple myeloma (MM), in which plasma cells proliferate, infiltrate tissues, and secrete IgG (55%), IgA (20%), light-chains (20%), or IgD or IgE (rarely). In Waldenstrom’s macroglobulinemia (WM), proliferating lymphoid cells secrete IgM. Paraproteinemia may also occur in lymphomas and in chronic lymphatic leukemia.1
Evaluation of the Immune System
John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie in Basic Sciences Endocrine Surgery Rhinology, 2018
Multiple myeloma is a malignancy of plasma cells that produce monoclonal immunoglobulin (paraprotein). Paraproteins may be detectable in both serum and/or urine. Twenty percent of myelomas only produce monoclonal light chains, which may only be detectable in urine as Bence Jones protein or in serum using a serum-free light chain assay. Therefore a full myeloma screen requires both blood for immunoglobulins and electrophoresis plus either serum-free light chains or urine for electrophoresis. This combination of tests will detect all but the very rare non-secretory myelomas. These tests are also required for the investigation of possible AL amyloid. As paraprotein levels may be relatively low and difficult to detect in AL amyloid, close liaison with the laboratory is recommended as more sensitive detection methods such as immunofixation may be required. Although paraproteins are the hallmark of myeloma, Waldenstrom’s macroglobulinaemia and AL amyloid, the majority of paraproteins detected in routine clinical practice are not associated with any identifiable lymphoproliferative disorder and are termed monoclonal gammopathy of uncertain significance (MGUS). Some MGUS may progress to lymphoproliferative disease with an overall rate of 1–2% per annum.
How can we assess and measure prognosis for MALT lymphoma? A review of current findings and strategies
Published in Expert Review of Hematology, 2021
Barbara Kiesewetter, Markus Raderer
In addition to extensive imaging, routine assessments for newly diagnosed MALT lymphoma consists of laboratory tests including differential blood counts and blood chemistry, electrophoresis and immunofixation for detection of paraproteinemia and measurement of beta 2 microglobulin (B2M) [11]. Elevation of lactate dehydrogenase (LDH), documented in 10–20% of patients, is considered to be a poor prognostic sign in all kinds of lymphoid malignancies and has also been associated with worse outcome in MALT lymphoma [10,30]. In addition, LDH > upper limit of normal (UNL) has been reported in patients experiencing histological transformation and should thus raise attention of the treating physician [8]. Paraproteinemia is present in up to one-third of patients but is rather a sequela of plasmacytic differentiation than a prognostic factor [33]. Discrepant results have been reported for B2M with no relevant impact based on currently implemented risk scoring systems, but evidence for prognostic value in retrospective evaluations including a series of 174 patients with non-gastric MALT lymphoma where B2M > UNL had a significant impact on 5-year PFS (92.2% versus 44.6%, p < 0.001) and 5-year OS (99.2% versus 63.8%, p < 0.001) [34] .
Implementation and evaluation of a rehabilitation concept in a patient suffering from Scleredema Adultorum Buschke: a case report
Published in Disability and Rehabilitation, 2018
Michael Mickel, Ahmad Jalili, Christina Gesslbauer, Richard Crevenna
Scleredema Adultorum Buschke (SAB) is a disorder manifesting as non-pitting indurations of the skin that start on the head and spread to other distal areas of the body. Buschke was the first to report this clinical entity in 1902 [1]. Extracutaneous organs such as the muscles, joints, heart, or eyes may also be affected. Based on etiology Graff [2] classified SAB as following: (Type 1) streptococcal infections, the most frequent cause, (Type 2) associated with developing paraproteinemias including multiple myeloma, and (Type 3) associated with diabetes mellitus. In rare cases, SAB can be associated with hyperparathyroidism, connective tissue disease or human immunodeficiency virus infection. SAB is classified as a rare disorder with unknown prevalence [3]. In a review of 163 cases of SAB a percentage frequency is reportet as 55% of total of Type 1, 25% of total of Type 2, and 20% of total of Type 3 [4]. SAB affects all races and there is no gender difference in predilection for the disease [5].
Paraproteins and electrolyte assays: exclusion effect and effect of paraprotein elimination
Published in Scandinavian Journal of Clinical and Laboratory Investigation, 2023
Tapio Lahtiharju, Eerika Lehtisyrjä, Pipsa Kulovesi, Kari Pulkki
Paraproteins are produced by a single pathological B lymphocyte cell clone. Paraproteins are abnormal, homogeneous, and essentially identical (monoclonal) antibodies. Paraproteins are present in myeloma, plasmacytoma, Waldenstrom’s macroglobulinemia, other B-lymphocyte malignancies or MGUS (monoclonal gammopathy) [1]. Paraproteins can affect chemical assays by increasing the viscosity of the sample which will result in decreased pipetting accuracy. Also, paraproteins may bind to various analytes, reagent enzymes and antibodies in samples, resulting in unreliable results [2]. Especially in assays using very alkaline or acidic reagents, the paraproteins may unfold and aggregate or precipitate [2,3]. This phenomenon makes the sample increase its turbidity and the sample becomes cloudy. Paraprotein precipitates interfere with spectrophotometric measurement methods of chemical analysers and the determination of the HIL index by absorbing and scattering light [2,4].
Related Knowledge Centers
- Gamma Globulin
- Monoclonal Gammopathy of Undetermined Significance
- Leukemia
- Blood
- Multiple Myeloma
- Myeloma Protein
- Monoclonality
- Immunoproliferative Disorder
- Tumors of The Hematopoietic & Lymphoid Tissues
- Plasma Cell Dyscrasias
- Monoclonal Gammopathy of Undetermined Significance