Serum free light chains light chain deposition disease with renal chains. Medical search. Frequent questions

When presenting to primary care physicians, patients with MIDD frequently have renal insufficiency, proteinuria and these symptoms are often accompanied by nephrotic syndrome, but patients may also present with acute renal failure. (wikipedia.org)
83% of MIDD patients have renal insufficiency, and while almost all patients will have proteinuria, 40-50% will have proteinuria in the nephrotic range. (wikipedia.org)
This deposition damages the kidney, leading to renal insufficiency. (wikipedia.org)
Common manifestations include lytic lesions in bones that cause pain and/or fractures, renal insufficiency, hypercalcemia, anemia, and recurrent infections. (merckmanuals.com)

The term Bence Jones protein has been used to designate a urinary protein that leaves solution at approximately 56°C under certain conditions of pH and ionic strength and returns to the solution upon further heating to 100°C. The Bence Jones protein represents a homogeneous population of immunoglobulin light chains of either kappa type or lambda type and is the product of a presumed single clone of plasma cells. (medscape.com)
Light chains (molecular weight 22,000 d) are polypeptides synthesized by plasma cells and assembled with heavy chains to form the various classes of immunoglobulins, for example, immunoglobulin G (IgG), immunoglobulin M (IgM), and immunoglobulin A (IgA). (medscape.com)
Monoclonal Immunoglobulin Deposition Disorder, or MIDD, is a disease characterised by the deposition of monoclonal immunoglobulins on the basement membrane of the kidney. (wikipedia.org)
There are three subtypes of MIDD, light chain deposition disease where Immunoglobulin light chains are deposited on the basement membrane, light and heavy chain deposition disease, where both heavy and light chains of immunoglobulins are deposited, and heavy chain deposition disease, where only immunoglobulin heavy chains are deposited. (wikipedia.org)
The diseases vary in what part of the immunoglobulin is deposited, and which measurement techniques are most useful in aiding their diagnosis. (wikipedia.org)
Several chapters are devoted to various glomerulopathies associated with deposition of immunoglobulin light and heavy chains, including those associated with amyloidosis. (edu.au)
Disorders characterized by abnormal proliferation of immunoglobulin-producing cells and abnormal proliferation of immunoglobulin monoclonal (M protein)represent part of the spectrum of disease due to the neoplastic behavior of the B lymphocyte series. (emedicodiary.com)
Paraproteinaemia is a disorder characterized by abnormal proliferation of immunoglobulin-producing cells due to the neoplastic behavior of B-Lymphocytic series with an increase in serum level of homogenous immunoglobulin (monoclonal IG) or its fragments. (emedicodiary.com)
Paraprotein is the presence of a monoclonal immunoglobulin band (M-band) in the serum. (emedicodiary.com)
Plasma cells are derived from B lymphocytes and produce immunoglobulin (Ig) which contains heavy and light chains. (emedicodiary.com)
It is characterized by hyperglobulinemia, excess Bence-Jones proteins (free monoclonal IMMUNOGLOBULIN LIGHT CHAINS) in the urine, skeletal destruction, bone pain, and fractures. (lookformedical.com)
A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. (lookformedical.com)
Two Ig light chains and two Ig heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) make one immunoglobulin molecule. (lookformedical.com)
The major immunoglobulin isotype class in normal human serum. (lookformedical.com)
This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY. (lookformedical.com)
A class of immunoglobulin bearing mu chains (IMMUNOGLOBULIN MU-CHAINS). (lookformedical.com)
A lymphoproliferative disorder characterized by pleomorphic B-LYMPHOCYTES including PLASMA CELLS, with increased levels of monoclonal serum IMMUNOGLOBULIN M. There is lymphoplasmacytic cells infiltration into bone marrow and often other tissues, also known as lymphoplasmacytic lymphoma. (lookformedical.com)

[ 2 ] The term has been associated with various glomerular abnormalities that are caused by the deposition of these monoclonal immunoglobulins (or their heavy-chain or light-chain subunits) and are broadly classified into 2 categories, organized or nonorganized, depending on the pattern of deposition. (medscape.com)

Nephrotoxic effects often develop from overproduction of monoclonal immunoglobulins and free light chains, leading to cast nephropathy (the most common cause of acute kidney injury), light-chain-related proximal tubular injury, and various glomerulopathies such as light-chain deposition disease and amyloid light-chain (AL) amyloidosis. (nejm.org)
Light chain (AL) amyloidosis is the most common type in the U.S. and is caused by a bone marrow disorder. (emedicinehealth.com)
AA amyloidosis occurs as a reaction to another illness, usually severe and chronic inflammatory diseases or infections. (emedicinehealth.com)
Cardiac amyloidosis (CA) is an infiltrative disease that is being increasingly identified in those with HF p EF. (thischangedmypractice.com)
Amyloidosis is a group of heterogeneous disorders characterized by the deposition of misfolded protein that aggregates into fibrils, affecting multiple organs including the heart, neural tissues, kidneys, and the gastrointestinal tract ( 1 ). (thischangedmypractice.com)
The two most common types associated with cardiac deposition are light chain amyloidosis ( AL ), typically associated with plasma cell dyscrasias, and transthyretin amyloidosis ( ATTR ), which may be either acquired (wild-type) or hereditary (due to mutations in the ATTR gene) ( 2 ). (thischangedmypractice.com)
A renal biopsy confirmed amyloidosis, with amyloid protein deposits in the tubules and blood vessels. (medscape.com)
Amyloidosis is caused by the deposition and aggregation of insoluble, misfolded B-pleated protein fibrils in tissue, causing progressive organ dysfunction. (medscape.com)
The two most common types of systemic acquired disease are wild-type transthyretin (ATTR) amyloidosis and monoclonal Ig light chain (AL) amyloidosis . (medscape.com)
AA amyloidosis is another form of acquired systemic disease that results from high levels of serum AA protein, an acute phase reactant associated with chronic inflammation. (medscape.com)
AA amyloidosis affects persons with chronic infections or autoinflammatory diseases and primarily involves the kidneys. (medscape.com)

These diseases include monoclonal gammopathy of undetermined significance, smoldering multiple myeloma, multiple myeloma and Waldenström's macroglobulinemia. (wikipedia.org)
Because of the effect MIDD has on the kidneys, it is classified as a monoclonal gammopathy of renal significance. (wikipedia.org)
LCDD is associated with multiple myeloma in 39-59% of cases, with monoclonal gammopathy of renal significance in 39% of cases and may also be associated with lymphoplasmacytic lymphoma. (wikipedia.org)
[ 1 ] First described in 1848, MM is part of a spectrum of diseases ranging from monoclonal gammopathy of unknown significance ( MGUS ) to plasma cell leukemia. (medscape.com)
Questo spiega come mai la MCN sia descritta sostanzialmente solo in caso di MM (o raramente in corso di altre patologie neoplastiche ematologiche come il linfoma linfoplasmocitico e la leucemia linfatica cronica) ma non nelle condizioni precancerose, come la cosiddetta monoclonal gammopathy of renal significance . (giornaleitalianodinefrologia.it)

Its pathophysiology includes immune complex deposition and a wide range of skin lesions. (lookformedical.com)

Light chains (molecular weight 22,000 d) are polypeptides that are synthesized by plasma cells and form part of immunoglobulins. (medscape.com)
Individual immunoglobulins have either kappa or lambda light chains, but not both. (medscape.com)
The deposition of monoclonal immunoglobulins on the basement membrane of the kidney causes renal impairment. (wikipedia.org)
Monoclonal immunoglobulins are produced in diseases that feature abnormal proliferation of plasma cells. (wikipedia.org)
Abnormal immunoglobulins, especially IGG or IGM, that precipitate spontaneously when SERUM is cooled below 37 degrees Celsius. (lookformedical.com)
Antibodies found in adult RHEUMATOID ARTHRITIS patients that are directed against GAMMA-CHAIN IMMUNOGLOBULINS. (lookformedical.com)

Laboratory workup revealed abnormal renal function with the rapid rise of creatinine from 1.04 mg/dL to 14.5 mg/dL within 3 days. (ijpmonline.org)
Abnormal neuronal function is often seen in various heart diseases, such as heart failure (HF), myocardial ischemia, infarction, and arrhythmias. (slideshare.net)

It can also lead to the other symptoms of nephrotic syndrome such as swelling and low serum albumin. (wikipedia.org)

Techniques commonly used to aid the diagnosis of MIDD include serum protein electrophoresis, urine protein electrophroresis, serum or urine immunofixation, measurement of serum free light chains and renal biopsy. (wikipedia.org)

Serum protein electrophoresis or immunofixation is positive in 25-76% of cases, while urine protein electrophoresis or immunofixation is positive in 42-90% of cases. (wikipedia.org)
Serum or urine protein electrophoresis or immunofixation is positive in 80-100% of cases, while serum free light chain measurement is positive in 100% of cases. (wikipedia.org)
Serum immunofixation revealed an IgA lambda monoclonal band despite normal findings on serum and urine protein electrophoresis. (medscape.com)

It appears that bortezomib may have an effect on the kidneys in reversal of renal failure, other than its anti-myeloma effect. (peoplebeatingcancer.org)

Multiple Myeloma an incurable disease, but I have spent the last 25 years in remission using a blend of conventional oncology and evidence-based nutrition, supplementation, and lifestyle therapies from peer-reviewed studies that your oncologist probably hasn't told you about. (peoplebeatingcancer.org)
Kidney/renal damage for newly diagnosed multiple myeloma patients can be as dangerous as the MM itself. (peoplebeatingcancer.org)
It can be a primary manifestation of multiple myeloma or develop as a terminal complication during the disease. (lookformedical.com)

Plasma cells normally produce a slight excess of light chains that are either excreted or catabolized by the kidney, and only a minute amount of light-chain protein normally appears in the urine. (medscape.com)
The kidney is the major site of metabolism of light-chain proteins. (medscape.com)
However, renal infiltration causes acute kidney injury in only 1% of patients with acute leukemia and in even fewer patients with lymphoma or chronic leukemia. (nejm.org)
Calcium phosphate precipitation in the renal tubules may also contribute to acute kidney injury in patients with severe hyperphosphatemia from the tumor lysis syndrome, especially if the urine is alkaline. (nejm.org)
At the same time, if your creatinine, BUN and/or GFR levels are out of the normal range, please read the studies linked below to learn about the interaction of MM, MM treatment and both of those when compared to renal/kidney health. (peoplebeatingcancer.org)
Vascular pathology related to hypertension was most commonly encountered followed by diabetic kidney disease. (ijpmonline.org)
In the context of significant weight loss, serum creatinine levels may overestimate eGFR, leading to a delayed diagnosis of chronic kidney disease (CKD) and late referral to a specialist. (medscape.com)
The right kidney and enlarged renal pelvis were observed as large signal intensity areas on T2WI. (cd31-signal.com)

Light chains are divided into 2 major classes based on the amino acid sequence in the constant portion of the polypeptide chain and are designated as kappa and lambda. (medscape.com)
These are further divided into at least 10 subtypes (4 kappa and 6 lambda) based on the amino acid sequence in the variable region of the polypeptide chain. (medscape.com)
Lambda light chains usually exist as dimers (44,000 d) and, therefore, are less likely to be filtered and appear in urine. (medscape.com)
At times, light chains of either kappa or lambda type may form tetramers (88,000 d), which are not filtered, and a patient may have light-chain proteinemia without light-chain proteinuria. (medscape.com)
Of patients producing either IgG or IgA, 40% also have Bence Jones proteinuria, which is free monoclonal kappa ( κ ) or lambda ( λ ) light chains in the urine. (merckmanuals.com)
Each Ig molecules have either 2 kappa or 2 lambda light chains. (emedicodiary.com)
There are two major types of light chains, kappa and lambda. (lookformedical.com)

The symptoms of the three subtypes are similar, but Heavy Chain Deposition Disease (HCDD) patient may have greater proteinuria than patients with the other subtypes. (wikipedia.org)
Here, we take the opportunity to report an unusual association of heavy chain deposition disease (HCDD) with clear cell subtypes of renal cell carcinoma in a 48-year-old male of Indian ethnicity. (ijpmonline.org)
To the best of our knowledge, this is one of the first reports demonstrating concurrent heavy chain deposition disease (HCDD) with clear cell subtypes of renal cell carcinoma in a 48- year-old male of Indian ethnicity. (ijpmonline.org)

Light chain deposition disease (LCDD) is the most common of the three subtypes (around 80% of all MIDD), but it and MIDD as a whole are classified as a rare disorder. (wikipedia.org)
Light microscopy cannot distinguish between the three subtypes, but immunofluorescence can. (wikipedia.org)
During the evaluation of resected specimens, oncopathologists check all the pathological parameters enlisted in the standard protocol for reporting such as histological subtypes, grade, stage, etc. [2] The majority of the times peritumoral and distant renal parenchyma is overlooked and the opportunity to identify concurrent familiar or unfamiliar medical renal diseases is missed. (ijpmonline.org)

Smithline et al first used the term light-chain nephropathy in 1976 to describe a case of renal tubular dysfunction with light-chain proteinuria. (medscape.com)
Diagnosis typically requires demonstration of M-protein (sometimes present in urine and not serum but rarely absent entirely) and/or light-chain proteinuria, and excessive plasma cells in the bone marrow. (merckmanuals.com)

The filtered light-chain proteins, reabsorbed by the proximal tubular cells via the tandem megalin/cubilin receptors, are catabolized by lysosomal enzymes. (medscape.com)
Metabolism (catabolism) of these filtered light-chain proteins depends on normal proximal tubular cell function, and damage to these cells can result in increased excretion of light-chain proteins in the urine. (medscape.com)

The most characteristic histologic lesion of light chain deposition disease (LCDD) is nodular glomerulosclerosis, which must be distinguished from diabetic glomerulosclerosis by using electron microscopy. (medscape.com)
Light Chain Deposition Disease (LCDD) features deposition of light chains only. (wikipedia.org)

Very high levels of uric acid in the glomerular filtrate may precipitate in the renal tubules, leading to micro-obstruction and vasoconstriction, as well as renal ischemia and up-regulation of inflammatory cytokines, and resulting in an abrupt decrease in the glomerular filtration rate. (nejm.org)

Kappa light chains usually exist as monomers (22,000 d) and are therefore small enough to be filtered through the glomerulus, but they may exist as dimers. (medscape.com)

Rarely, patients have no M-protein in blood and urine, although the currently used serum free light chain assay now demonstrates monoclonal light chains in many of these formerly so-called nonsecretory patients. (merckmanuals.com)
National Comprehensive Cancer Network (NCCN) Clinical Practice Guidelines also recommend the use of serum free light chain assay and plasma cell fluorescence in situ hybridization (FISH) on bone marrow: del 13, del 17p13, t(4;14), t(11;14), t(14;16), t(14;20), 1q21 amplification, 1p deletion as part of the initial diagnostic workup. (medscape.com)

Normally Ig molecule consists of 4 polypeptide chains 2 heavy chains and 2 light chains. (emedicodiary.com)
Polypeptide chains, consisting of 211 to 217 amino acid residues and having a molecular weight of approximately 22 kDa. (lookformedical.com)

While ATTR-CA is often well tolerated for many years until severe LV wall thickening, diastolic dysfunction, and conduction disease develop, the median survival can be limited to 4 to 6 years from the onset of congestive symptoms (2). (thischangedmypractice.com)
The present case indicates that non-contrast MRI is useful for the evaluation of renal diseases even in elderly patients with renal dysfunction. (cd31-signal.com)

This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. (lookformedical.com)

[3] stated that more than 60% of nephrectomy specimens had pathology in non-neoplastic renal parenchyma which is responsible for the deterioration of renal function disproportionate to nephron loss in the postoperative era. (ijpmonline.org)

The presence of light-chain proteins in the urine is associated with a number of systemic diseases (see Etiology ). (medscape.com)
At least 36 proteins have been identified in humans, with 17 showing systemic involvement and the rest presenting as localized diseases. (medscape.com)

Light microscopy (hematoxylin and eosin stain at 25X power) showing nodular glomerulosclerosis (arrow) and thickening of the basement membrane. (medscape.com)

Light-chain proteins appear in urine in high concentration either when the production of light-chain proteins is markedly increased or when the ability of the proximal tubules to reabsorb all the filtered protein is diminished. (medscape.com)
Despite the similar effect in the heart, AL and ATTR are two patho-physiologically different disease processes leading to CA. In AL disease, the amyloid proteins are light chains produced by clonal plasma cells in the bone marrow ( 4 ). (thischangedmypractice.com)

Using a translational approach to renal manifestations in patients with plasma cell dyscrasias / myelomas, this publication conveys a comprehensive state-of-the-art view of the subject. (edu.au)
Conditions characterized by the presence of M protein (Monoclonal protein) in serum or urine without clinical manifestations of plasma cell dyscrasia. (lookformedical.com)

Paraproteins containing only light chains lead to Bence Jones paraproteinemia, while the presence of only atypical heavy chains leads to heavy chain disease. (lookformedical.com)

More than two-thirds of patients in the belatacept groups remained on CNI- and steroid-free regimens at 12 months and the calculated glomerular filtration rate was 8-10 mL/min higher with either belatacept regimen than with TAC-MMF. (cd31-signal.com)

The mainstay of management of cast nephropathy is the clone-based therapy by reducing production and thereby precipitation of light chains. (giornaleitalianodinefrologia.it)

The excess light chains are secreted into the extracellular fluid and readily pass through the glomerulus. (emedicodiary.com)

Primary renal tumors account for 2%-3% of all human malignancy, and the major subtype is renal cell carcinoma (RCC). (ijpmonline.org)

Current immunosuppressive regimens in renal transplantation typically include calcineurin inhibitors (CNIs) and corticosteroids, both of which have toxicities that can impair recipient and allograft health. (cd31-signal.com)

Light and Heavy Chain Deposition Disease (LHCDD or HLCDD in the scientific literature) features deposition of both light chains and heavy chains. (wikipedia.org)
In myeloma, plasma cells produce monoclonal (M) Ig of a single heavy and light chain commonly referred to as a paraprotein. (emedicodiary.com)
The most common abnormality is the production of excess of light chains over heavy chains. (emedicodiary.com)
Altri quadri possibili sono l'amiloidosi, la light or heavy chain deposition disease , la sindrome di Fanconi, etc. (giornaleitalianodinefrologia.it)

This is most commonly associated with κ free light chains. (wikipedia.org)
Nerve disease commonly involves peripheral sensory and motor nerves and autonomic nerves. (aan.org.au)

Lack of broad awareness among clinicians and the prevalence of concomitant conditions also associated with HF p EF such as hypertension, aortic stenosis, and coronary artery disease can all be major obstacles for appropriate diagnosis and treatment. (thischangedmypractice.com)

In recent years, the knowledge of how renal damage occurs in patients with plasma cell dyscrasias / myeloma has substantially increased. (edu.au)

Osteoporosis Osteoporosis is a progressive metabolic bone disease that decreases bone mineral density (bone mass per unit volume), with deterioration of bone structure. (merckmanuals.com)

Reversal of renal failure was observed in all six patients. (peoplebeatingcancer.org)
Although the emphasis is on the management of cases with renal involvement, a distinct focus on the diseases as a whole and their impact on patients' general health and prognosis has been maintained throughout the discussions. (edu.au)

As the deposits have no organised structure, it is grouped among the non-organised monoclonal gammopathies of renal significance. (wikipedia.org)

Timely diagnosis is important, as the prognosis for untreated AL disease is poor, with median survival less than a year following diagnosis (7). (thischangedmypractice.com)

Renal cell carcinoma (RCC) is the most common subtype of adult renal tumors, and its detection rate in the early stages has been increased in the dawn of advanced imaging modalities. (ijpmonline.org)

We report here six cases of renal failure secondary to MM treated with bortezomib. (peoplebeatingcancer.org)

Reversal of renal failure persisted despite incomplete response to MM in two cases, and progression of disease in one patient. (peoplebeatingcancer.org)
The goal of treatment is to relieve symptoms and slow the progression of the disease. (emedicinehealth.com)

Anatomy1

Diseases16

Chemicals and Drugs19

Analytical, Diagnostic and Therapeutic Techniques and Equipment8

Phenomena and Processes3

Information Science2

Insufficiency4

Immunoglobulin17

Depending on the pattern of deposition1

Amyloidosis11

Monoclonal gammopathy5

Pathophysiology1

Immunoglobulins6

Abnormal2

Nephrotic syndrome1

Biopsy1

Protein electrophoresis3

Kidneys1

Multiple myeloma3

Kidney8

Lambda7

HCDD3

Subtypes3

Proteinuria2

Tubular2

LCDD2

Precipitate1

Kappa1

Assay2

Polypeptide chains2

Dysfunction2

Infections1

Disproportionate1

Systemic2

Microscopy1

Proteins2

Manifestations2

Bence Jones1

Glomerular1

Nephropathy1

Glomerulus1

Primary1

Typically1

Heavy4

Commonly2

Coronary artery1

Occurs1

Bone1

Patients2

Deposits1

Diagnosis1

Adult1

Secondary1

Progression2