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Renal Disease; Fluid and Electrolyte Disorders
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
In amyloidosis there is extracellular deposition of protein fibrils in many organs (see Chapter 11, Diabetes mellitus, obesity, lipoprotein disorders and other metabolic diseases), including the kidney in up to 80% of patients. The fibrils contain antibody light chains in AL amyloidosis associated with myeloma or a paraproteinaemia (see Chapter 15, Haematological disease) or the acute phase reactant protein serum amyloid A protein in AA amyloidosis associated with chronic inflammatory disease.
Diagnostic Approach in Nerve Biopsy
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
Inflammation types and sites differ from disease to disease. However, the inflammation is considered serious when it occurs in the perineurium or endoneurium. Perivascular inflammatory cuffs are sometimes non-specific. Types of inflammation can be distinguished using immunolabelling markers such as CD45 and CD68. Although paraproteinemia is associated with minimal inflammation, the diagnosis requires ultrastructural examination of EM sections.
Chronic Leukemias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Scott J. Graham, James D. Cotelingam
The differential diagnosis of PCL includes Waldenstrom’s macroglobulinemia, CLL, and B-PLL, all of which may have circulating lymphoplasmacytoid cells and paraproteinemia. Immunophenotypic analysis, together with clinical and radiographic data, usually leads to accurate diagnosis.
Clonal heterogeneity of polymorphic B-cell lymphoproliferative disease, EBV-positive, iatrogenic/immune senescence: implications on pathogenesis and treatment
Published in Hematology, 2022
Yu-Yan Hwang, Rex Au-Yeung, Rock Y.Y. Leung, Eric Tse, Yok-Lam Kwong
The presence on presentation of three paraproteins, two with lambda light chain and one with kappa light chain, was also of interest. Intriguingly, the nasopharyngeal and marrow lesions all showed lambda-light chain restriction, which corresponded to the circulating IgGλ and IgAλ paraproteins. However, there was another IgGκ paraprotein that persisted for a considerable duration after chemotherapy. The origin of this paraprotein remained obscure, but could conceivably be from a non-biopsied lesion shown on PET/CT. This lesion was likely to be EBV-negative, as plasma EBV DNA remained undetectable during persistence of the IgGκ paraprotein. Biclonal paraproteinemia in plasma cell malignancies is uncommon, reported to occur only in 0.2% of 1027 cases in one series [5]. The presence of multiple paraproteins with different light chains further underlined the clonal heterogeneity present in our case.
Managing complications secondary to Waldenström’s macroglobulinemia
Published in Expert Review of Hematology, 2021
Ilias Pessach, Meletios A. Dimopoulos, Efstathios Kastritis
IgM-related peripheral neuropathy is a common complication of WM, and about 25% of WM patients, may have symptoms of peripheral neuropathy. In clinical practice, a significant proportion of patients with WM will have neuropathy as the only indication to start therapy. Most antibody-targeted paraprotein-associated neuropathies that are presenting in WM are typically length dependent and slowly progressive so that in case of a rapidly progressing peripheral neuropathy (i.e in less than 6 months), other possible causes should be considered; in case of cranial neuropathies, CNS infiltration or other causes should be sought [56]. Patients with known IgM paraproteinemia and peripheral neuropathy should undergo nerve conduction studies and electromyography. Depending on the neurological presentations, serological evaluation for autoimmune antibodies (including those against MAG and GM1) should be requested, and serum should be screened for type I and type II cryoglobulin activity.
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] .