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Multiple Myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
A third of patients present with significant renal impairment, and the majority will be affected at some point during the course of the illness with 10% of patients on dialysis. The main cause of renal failure in myeloma is deposition of Bence Jones protein in the renal tubules with histological features of “myeloma kidney” (fractured distal tubular casts with a surrounding chronic inflammatory infiltrate including giant cells). The physicochemical properties of individual immunoglobulin light chains are important. Many other factors may contribute to renal failure, and these include hypercalcemia, infection, dehydration, hyperuricemia, amyloid deposition, and non-steroidal anti-inflammatory drugs.
The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Lynne V. Abruzzo, L. Jeffrey Medeiros
Immunophenotypic studies have demonstrated that MCLs express monotypic surface IgM and IgD, and pan-B-cell antigens. In contrast to other B-cell neoplasms, MCLs are more likely to express lambda than kappa immunoglobulin light chains. Like CLL/SLL, the neoplastic cells express the CD5 antigen. However, CLL/SLL is usually CD23-positive and MCL is usually CD23-negative. In addition, the density of CD20 antigen and immunoglobulin on the surface of MCL cells is characteristically dense, or “bright” by flow cytometry.
Multiple myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2014
Renal impairment is also common in myeloma patients.65 Approximately one-third of the patients present with significant renal impairment67 and it will affect approximately half at some point during the course of the illness.65,68 A small number of patients (3%–12%) present with renal failure severe enough to require dialysis.69 The main cause of renal failure in myeloma is deposition of BJP in the renal tubules. The classic histological features of ‘myeloma kidney’ are of fractured distal tubular casts with a surrounding chronic inflammatory infiltrate including giant cells. The physicochemical properties of individual immunoglobulin light chains result in differing degrees of renal impairment. Less frequently, light-chain deposition may produce a form of glomerulonephritis (light-chain deposition disease). Many other factors may contribute to renal failure and these include hypercalcaemia, infection, dehydration, hyperuricaemia, amyloid deposition and non-steroidal anti-inflammatory drugs.
Strong positive light chain immunostaining in a patient with transthyretin amyloidosis
Published in Hematology, 2023
Jiao Chen, Haifei Chen, Lingyun Zhou, Danbo Liu, Fang Du, Hongxian Xiang
Congo red staining followed by IHC or immunofluorescence using fibril protein-specific antibodies is critical for diagnosing amyloidosis and confirming fibril protein type. However, the assessment of amyloid by IHC is qualitative, non-standard and requires operator expertise [5, 6]. Many antibodies associated with IHC lack specificity, occasionally resulting in simultaneous positivity for ATTR and immunoglobulin light chains (kappa or lambda light chains) [7]. In addition, currently available antibodies are not applicable to rare forms of amyloidosis [4]. Diagnosis of amyloidosis is a great challenge for clinicians owing to the complex multisystem manifestations of the disease. Similarly, our patient successively visited four different departments and was diagnosed with peripheral neuropathy, lumbar spinal stenosis and cardiomyopathy due to unknown cause. In such clinical scenarios, a multidisciplinary team approach is indubitably superior to a single disciplinary approach.
Combined serum free light chain predicts prognosis in acute kidney injury following cardiovascular surgery
Published in Renal Failure, 2022
Wenji Wang, Lulu Zhang, Tianye Yang, Shaojun Ma, Qi Zhang, Peng Shi, Feng Ding
The immunoglobulin light chains, as part of the immunoglobulins, are produced by plasma cells. There are two isotypes of light chains, kappa (κ) and lambda (λ) [7]. Monoclonal production of free light chains (FLCs) is important in almost all plasma cell disorders, such as multiple myeloma, amyloidosis, and monoclonal gammopathy of undetermined significance [8]. Recently, elevated concentrations of serum polyclonal combined FLCs (cFLC), as a potential biomarker of activation of the B-lymphocyte, have been investigated in a number of non-hematological malignant and inflammatory disorders including diabetes, CKD, and systemic lupus erythematosus (SLE) [9–11]. Nonspecific increases in cFLC occurred in a similar to the nonspecific increases in C reactive protein seen with inflammation[12]. The elevation of cFLC is also the result of reduced removal by impaired renal function [7,13]. Furthermore, elevated cFLC was demonstrated as a predictor of poorer overall survival in the general population, in patients with heart failure and in patients with CKD, as well [14–16].
A special case of hypertrophic cardiomyopathy with a differential diagnosis of isolated cardiac amyloidosis or junctophilin type 2 associated cardiomyopathy
Published in Acta Clinica Belgica, 2021
Sévérine De Bruijn, Xavier Galloo, Gilles De Keulenaer, Edgard A. Prihadi, Christiane Brands, Mark Helbert
Systemic amyloidosis is a collective name for a number of diseases characterized by extracellular precipitation and tissue infiltration by ‘amyloid’ proteins, which arise as a consequence of misfolding of precursor proteins that aggregate into insoluble fibrillar structures [5]. Precipitation of these misfolded proteins can occur in virtually any organ including the kidneys, liver, autonomic nervous system and the heart, disrupting the normal organ function [6]. Until now, five proteins have been identified to cause cardiac amyloidosis (CA): immunoglobulin light chain, immunoglobulin heavy chain, transthyretin (TTR), serum amyloid A and mutant apolipoprotein AI [7]. When the amyloid precursor protein consists of monoclonal light chains, usually lambda (λ) light chains, the diagnosis of immunoglobulin light chain (AL) amyloidosis or primary amyloidosis is made. Misfolded serum amyloid A protein, which is an acute-phase reactant, lies at the root of AA or secondary amyloidosis. The latter is associated with a variety of chronic inflammatory disorders, such as rheumatoid arthritis, Crohn’s disease and familial Mediterranean fever. AA amyloidosis is rarely associated with clinically overt heart disease [8]. Transthyretin amyloidosis (ATTR) arises from the deposition of misfolded hepatic TTR proteins [9]. Finally, only a few cases have been reported in which heavy chain – and/or apolipoprotein AI-derived amyloids are the culprit proteins [10,11].