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Haematological Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
A paraprotein is an abnormal monoclonal immunoglobulin produced by a malignant clone of antibody-producing B cells (usually plasma cells). Multiple myeloma is a malignant tumour of plasma cells, terminal cells of the B-cell lineage, which secrete immunoglobulins (antibodies), thus myeloma is typically associated with the presence of a paraprotein. Other clonal disorders of B cells can also be associated with a paraprotein: these are also classified (with myeloma) as paraproteinaemias. Very high paraproteins can be associated with the hyperviscosity syndrome.
Other Common Peripheral Neuropathies
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
Amyloid neuropathy is defined as widespread amyloidosis that affects multiple nerves. It could be familial such as familial amyloid polyneuropathy (FAP) or non-familial (primary or secondary subtypes). FAP is associated with apolipoprotein Ig-light chain. It usually occurs as an isolated neuropathy. Primary amyloidosis occurs in 50% of cases and is associated with paraprotein deposition and underlying plasma cell diseases such as multiple myeloma. The clinical diagnosis is always established before the biopsy. Patients may present with painful symmetrical distal sensorimotor neuropathy associated with muscle weakness. Findings of monoclonal free-light chain or plasma cells in bone marrow biopsy and urinary test are suggestive for primary amyloidosis. Secondary amyloidosis is the rarest causative variant of amyloid neuropathy because it is not always present with peripheral neuropathy. Secondary amyloidosis is commonly associated with systemic chronic inflammatory diseases.
Multiple Myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
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, hypercalcemia, and renal failure are medical emergencies requiring immediate management. Patients with a paraprotein found on routine testing and who have no clinical symptoms and no anemia, hypercalcemia, or renal impairment can be seen less urgently. Appropriate initial investigations are summarized in Table 31.1.
Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review
Published in Renal Failure, 2023
Li Zhu, Lei Wang, Hongxia Shi, Lei Jiang, Xin Li, Chunying Shao, Yu Yan, Bao Dong, Wanzhong Zou, Li Zuo
After admission, the patient’s serum creatinine level pre-dialysis fell to 5.63 mg/dL (normal, 0.51–0.95 mg/dL) without any specific treatment. The serum calcium level was 2.21 mmol/L (reference, 2.20–2.65 mmol/L). A morning urinalysis revealed 2+ protein, 2+ glucose, urine specific gravity of 1.006, and normal blood glucose. Renal glycosuria, with normal serum phosphorus, normal serum uric acid, low serum bicarbonate levels, and continuous hypokalemia under severe renal insufficiency raised clinical suspicion of Fanconi syndrome. The protein level in a 24-h urine sample was elevated to 2.66 g/day (normal, 0.03–0.14 g/day). Urine protein electrophoresis revealed mixed overflow proteinuria, tubular proteinuria, and glomerular proteinuria. Serum protein electrophoresis revealed monoclonal gamma paraprotein (8.4 g/L). Both serum and urine immunofixation electrophoresis revealed restricted immunoglobulin G kappa (IgGkappa) and kappa light chain. Serum free light chain results were κ > 4850 mg/L (normal, 3.30–19.40 mg/L), free λ 61.8 mg/L (normal, 5.71–26.3 mg/L), and κ/λ > 78.48 (normal, 0.26–1.65). Ultrasound showed bilateral renal atrophy (right kidney, 8.7 cm in length; left kidney, 8.8 cm in length). Although the patient did not suffer any trauma, an X-ray of the lumbar spine revealed a pathological fracture of L1, which was not seen three weeks ago. MM was suspected due to the presence of monoclonal gamma paraprotein, a pathological fracture, anemia, and renal impairment. Kidney and bone marrow biopsies were performed.
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].
IgG4 plasma cell myeloma without clinical evidence of IgG4-related disease: a report of two cases
Published in Hematology, 2020
Deonne Thaddeus V. Gauiran, Krista M. Marcon, Mari L. DeMarco, Angela W. S. Fung, Grace van der Gugten, Andre Mattman, Mollie N. Carruthers, Kevin W. Song, Luke Y. C. Chen
Since the advent of IgG4-RD, there have been few reports of IgG4 myeloma in the literature. One retrospective study of 158 bone marrow biopsies from patients with IgG myeloma identified six cases (4%) that were IgG4 subtype on immunohistochemical staining [14]. Four of the six cases were men with a mean age of 64 years (range 53–87). All of these patients had elevated serum paraprotein (mean 24 g/L, range 5–42 g/L). In their report, the authors noted that three cases had plasmablastic morphology and two had necrotizing fasciitis. None of these six cases had evidence of IgG4-RD, although the authors did not report the actual serum IgG4 concentration in this case series. One case report described a case of kappa-restricted IgG4-positive myeloma with IgG4 levels of 48.8 g/L but did not provide any clinical or laboratory information about possible features of IgG4-RD [16]. Kato et al. reported another case of kappa-restricted myeloma with increased monoclonal IgG4 (15.6 g/L) complicated by pancreatic ductal adenocarcinoma (initially misdiagnosed as localized autoimmune pancreatitis) [15]. Only a few of these reports provided detailed information about response to treatment. One very recent study, published while this present paper was under review, describes 3 patients with IgG4 myeloma out of 80 patients with IgG myeloma from a Japanese cohort [27]. Larger studies of IgG myeloma with subclassification of the serum paraproteins and plasma cell immunohistochemistry may provide insight into whether there are differences in the clinical presentation and prognosis for IgG1, IgG2, IgG3 and IgG4 myeloma.