Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia
Wojciech Gorczyca in Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Lymphoplasmacytic lymphoma (LPL) is a rare low-grade lymphoma characterized by a mixture of small lymphocytes, lymphocytes with plasmacytoid features, and plasma cells, which do not fulfill the criteria for any of the other small B-cell lymphoid neoplasms [1–16]. It involves the bone marrow (BM) and occasionally the lymph node, spleen, and rarely blood. Monoclonal paraprotein (usually IgM) is often present, but is not required for the diagnosis of LPL. In most patients, adenopathy develops slowly over many years. Extramedullary sites may be involved as well, although most of these cases represent marginal zone B-cell lymphoma with plasmacytic differentiation. The most common extramedullary sites include the lymph nodes, soft tissue spleen, skin, lung, tonsil, gastrointestinal (GI) tract, and liver [17]. Patients present with weakness and fatigue due to anemia usually have IgM serum paraprotein and may display hyperviscosity (up to 30%), neuropathy, cryoglobulinemia, or coagulopathy. Some patients have more lymphomatous manifestation with adenopathy or extranodal infiltrates.
Multiple myeloma
Pat Price, Karol Sikora in Treatment of Cancer, 2014
Initial investigation of a patient with suspected myeloma should include the screening tests indicated in Table 31.1, followed by further tests to confirm the diagnosis. The urgency with which these should be carried out depends on the method of presentation. Symptomatic patients with suspected myeloma require urgent specialist referral. Spinal cord compression, hypercalcaemia and renal failure are medical emergencies requiring immediate admission to hospital. Patients with a paraprotein found on routine testing and who have no clinical symptoms and no anaemia, hypercalcaemia or renal impairment do not necessarily require urgent referral, but specialist advice should be sought. Appropriate initial investigations are summarized in Table 31.1 (permission granted from BCSH).
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.
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].
Quality of life in multiple myeloma: considerations and recommendations
Published in Expert Review of Hematology, 2019
Samantha Seitzler, Elizabeth Finley-Oliver, Christine Simonelli, Rachid Baz
MM is characterized by the uncontrolled expansion of malignant plasma cells in the bone marrow and the production of a monoclonal immunoglobulin. Bone marrow involvement often results in anemia, lytic bone disease, bone destruction, fractures and hypercalcemia; while the paraprotein may result in renal insufficiency. These so-called CRAB criteria represent an indication to start systemic therapy. While some patients with MM may be asymptomatic at the time of diagnosis, the hallmarks of the disease include fatigue, bone pain, frequent infections, hypercalcemia, renal failure, and peripheral neuropathy [6]. Furthermore, considering that the median age of patients at the time of diagnosis is nearly 70 years of age, comorbid conditions are often coexistent with the MM diagnosis [1]. It is imperative that patient’s comorbidities as well as symptoms of MM be managed effectively so that a patient’s quality of life (QOL) may not be negatively impacted [2,6]. While it is imperative that health-care providers strive to keep patients QOL at the forefront of treatment decisions, data pertaining to QOL in MM remains limited in contrast to other aspects of the disease. Indeed, when performing a Medline search of ‘quality of life’ and ‘Myeloma’ MeSH terms between 2010 and 2015, one notes 112 manuscripts and this contrasts sharply with over 13,000 manuscripts with ‘Myeloma’ as the sole MeSH term.
A look at treatment strategies for relapsed multiple myeloma
Published in Expert Review of Anticancer Therapy, 2018
Giusy Cetani, Mario Boccadoro, Stefania Oliva
Disease-specific characteristics should be taken into account in particular clinical manifestations: some patients may experience a biochemical relapse based on an increased level of monoclonal paraprotein without any organ damage. In this asymptomatic biochemical relapse setting, a ‘watch and wait’ approach is recommended. However, some indications for starting treatment at biochemical relapse were recently discussed by the IMWG [12], in particular: doubling of the serum M-protein; increase of serum M-protein by ≥10 g/L; increase of urine M-protein by ≥500 mg/24 h or an increase of involved serum FLC level by ≥200 mg/L (plus abnormal ratio) by two measurements, 2 months apart. Other factors should be considered when deciding whether to start treatment at biochemical relapse: aggressive disease at diagnosis; a short treatment-free interval with a suboptimal response to the previous treatment; imminent risk for organ dysfunction, such as previous light chain-induced renal impairment, aggressive bone lesions or unfavorable cytogenetics [13].
Related Knowledge Centers
- Antibody
- Immunoglobulin Light Chain
- Monoclonal Antibody
- Monoclonal Gammopathy of Undetermined Significance
- Plasma Cell
- Cancer
- Multiple Myeloma
- Monoclonality
- Monoclonal Gammopathy of Undetermined Significance
- Hemorheology
- Kidney Disease