Light chain deposit diseases light chain deposition disease with renal chains. Medical search. Frequent questions

[ 20 ] Mesangial nodularity within the glomerulus occurs from the increased deposition of extracellular matrix proteins mixed with kappa light chains. (medscape.com)
The presence of light-chain proteins in the urine is associated with a number of systemic diseases (see Causes). (medscape.com)
Amyloidosis is any of a group of disparate conditions characterized by extracellular deposition of insoluble fibrils composed of misaggregated proteins. (msdmanuals.com)
However, some prefibrillar oligomers of amyloidogenic proteins have direct cellular toxicity, an important component of disease pathogenesis. (msdmanuals.com)
In systemic amyloidosis, circulating amyloidogenic proteins form deposits in a variety of organs. (msdmanuals.com)
Localized forms of amyloidosis appear to be caused by local production and deposition of an amyloidogenic protein (most often immunoglobulin light chains) within the affected organ rather than by deposition of circulating proteins. (msdmanuals.com)
At least 36 proteins have been identified in humans, with 17 showing systemic involvement and the rest presenting as localized diseases. (medscape.com)
Different light chains are responsible for two diseases with diametrically opposite mesangial alterations: Light chain deposition disease which results in the expansion of the mesangium due to accumulation of matrix proteins not present in the normal mesangium and AL (light chain-associated) amyloidosis where the native mesangial matrix is replaced by fibrils (amyloid). (heraldopenaccess.us)
Approximately 85% of the monoclonal proteins in patients with plasma cell dyscrasia are associated with renal disease. (heraldopenaccess.us)
Normal light chains are small molecular weight proteins that are filtered through the peripheral capillary walls and are delivered to the proximal tubules where they are catabolized by endosomal activity in the apical portions of the tubules and their amino acids are then returned to the circulation. (heraldopenaccess.us)
The build-up of the amyloid proteins (deposits) can make it difficult for the organs and tissues to work properly. (askhematologist.com)
Amyloidoses are a group of diseases resulting from deposition of amyloid, insoluble fibrils derived from various precursor proteins, into extracellular tissues. (renalfellow.org)
Diagnosis of amyloid type occurs via immunohistochemistry, mass spectrometry, or amino acid sequencing of proteins in amyloid deposits. (renalfellow.org)
FIGURE 339-1 Scheme of the primary cilium and cystic kidney disease proteins. (basicmedicalkey.com)
Topology of autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) proteins polycystin-1, polycystin-2, and fibrocystin/polyductin (FPC) are shown. (basicmedicalkey.com)
PC2 and FPC both interact with kinesin 2 (KIF 3A/B). Localization of disease proteins in the cilium, the transition zone, and the basal body is color coded. (basicmedicalkey.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)

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

[ 10 ] In 25% of patients, an abnormal serum free light chain ratio is noted, even without an abnormal finding with serum and/or urine electrophoresis with immunofixation. (medscape.com)
The physicochemical and conformational characteristics of the abnormal light chains are primarily responsible for the downstream events affecting the mesangial milieu. (heraldopenaccess.us)
Investigations performed in the last 20 years have provided detailed insights into how these abnormal light chains interact with the mesangium to produce glomerular injury. (heraldopenaccess.us)
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)
Amyloid is an abnormal insoluble protein that is produced in the bone marrow and can be deposited in any tissue or organ. (askhematologist.com)
Various subtypes are classified by their abnormal ultrastructures and immune deposits. (lookformedical.com)

The necessary criterion to make the diagnosis of LCDD requires that all tissues to be stained for kappa and light chains and the tissue must exhibit kappa fixation along the tubular basement membrane. (medscape.com)
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)
Specific mutations in human AE1 cause several types of hereditary hemolytic anemias and/or distal renal tubular acidosis. (rcsb.org)

[ 5 , 6 ] LCDD was first described in 1976 in two patients with end-stage renal disease as granular deposits of free light chains in multiple organs, including the kidneys, that did not stain with Congo red. (medscape.com)

Light-chain deposition disease (LCDD) is the deposition of monoclonal light chains in multiple organs. (medscape.com)
LCs are normally cleared by the kidneys, but in LCDD, these light chain deposits damage organs and cause disease. (wikipedia.org)
Light chains may be deposited in many other organs and may or may not result in any symptoms. (wikipedia.org)
Tissue may be taken from affected organs such as the kidneys, liver, heart, nerves, and other organs to confirm amyloid deposits. (emedicinehealth.com)
This causes deposits of amyloid protein in single organs, including the skin (cutaneous amyloidosis). (askhematologist.com)

Amyloid deposits stain pink with hematoxylin and eosin, contain carbohydrate constituents that stain with periodic acid-Schiff dye or with Alcian blue, but most characteristically have apple-green birefringence under polarized light microscopy after Congo red staining. (msdmanuals.com)
Pathologists now know that amyloid, using the Congo red stain introduced in the 1920s, looks pink with normal lighting and demonstrates apple-green birefringence under polarized light. (askhematologist.com)
Apple-green birefringence under polarized light. (askhematologist.com)
The β-pleated sheet formation of fibrils gives them the ability to bind Congo red dye (seen on light microscopy) in an organized, intercalated way that emits apple-green birefringence under polarized light. (renalfellow.org)
3. Congo red stain shows orange-colored positivity by regular microscopy and apple-green birefringence under polarized light. (renalfellow.org)

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)
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)
Primary renal tumors account for 2%-3% of all human malignancy, and the major subtype is renal cell carcinoma (RCC). (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)
What are the useful imaging procedures in patients suspected to have renal cell carcinoma? (diwa-gbr.de)

A single clonal plasma cell is responsible for the overproduction of kappa chains and more rarely, lambda light chains. (medscape.com)
Amyloid light-chain (AL)-amyloidosis consists predominantly of lambda light chains, whereas kappa light chains are predominantly involved in LCDD. (medscape.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)
Individual immunoglobulins have either kappa or lambda light chains, but not both. (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)
Positive lambda light chain stain in a glomerulus. (askhematologist.com)
Although healthy individuals have a preponderance of kappa free light chains (K/λ) = 2:1), the reverse is true in most patients with primary amyloidosis, as excess lambda light chains have a greater propensity to be amyloidogenic. (askhematologist.com)
AL fibrils are most often lambda light chains (four times more likely than kappa light chains). (renalfellow.org)
There are two major types of light chains, kappa and lambda. (lookformedical.com)

and nonfibrillar, powdery, electron-dense deposits in the basements on electron microscopy. (medscape.com)
The most characteristic histologic lesion of light chain deposition disease (LCDD) is nodular glomerulosclerosis, which must be identified from diabetic glomerulosclerosis by using electron microscopy. (medscape.com)
Light microscopy (hematoxylin and eosin stain at 25X power) showing nodular glomerulosclerosis (arrow) and thickening of the basement membrane. (medscape.com)
The various types of amyloid are indistinguishable using light microscopy. (askhematologist.com)
This birefringence is the most widely available gold standard for diagnosis of amyloidosis, however neither light nor electron microscopy can distinguish different types of amyloidosis. (renalfellow.org)
This can sometimes be seen by silver stain on light microscopy. (renalfellow.org)

Distinguishing characteristics - AL amyloidosis is the most prevalent type of amyloid disease in most parts of the United States (US). (renalfellow.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)
Amyloid can deposit in the glomerulus (predominant), tubulointerstitium, or vasculature of the kidney. (renalfellow.org)

Light chain deposition disease (LCDD) is a rare blood cell disease which is characterized by deposition of fragments of infection-fighting immunoglobulins, called light chains (LCs), in the body. (wikipedia.org)
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)
Antibodies found in adult RHEUMATOID ARTHRITIS patients that are directed against GAMMA-CHAIN IMMUNOGLOBULINS. (lookformedical.com)

LCDD is categorized as a monoclonal immunoglobulin deposition disease in the World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues. (medscape.com)
[ 9 ] although in LCDD only light chains will be observed. (medscape.com)
LCDD is a rare disease, so no established guidelines exist and management remains controversial. (medscape.com)
Unlike in AL amyloidosis, in which light chains are laid down in characteristic amyloid deposits, in LCDD, light chains are deposited in non-amyloid granules. (wikipedia.org)
More than 90% of people with LCDD develop kidney failure, often with rapid progression of disease. (wikipedia.org)
In patients with LCDD, a biopsy of the affected organ will show deposited light chains. (wikipedia.org)
After 5 years, about 37% of patients with LCDD are alive and do not have end stage renal disease. (wikipedia.org)
The glomerulopathies associated with monoclonal light chains are Light Chain Deposition Disease (LCDD) and AL (light chainrelated) amyloidosis, each creating completely different alterations in the mesangium. (heraldopenaccess.us)

A renal biopsy confirmed amyloidosis, with amyloid protein deposits in the tubules and blood vessels. (medscape.com)
In all these, light chains purified from the urine of patients with renal biopsy-proven conditions have been used. (heraldopenaccess.us)

Plasma cells normally produce a slight excess of light chains that are either excreted or catabolized by the kidney. (medscape.com)
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)
Vascular pathology related to hypertension was most commonly encountered followed by diabetic kidney disease. (ijpmonline.org)
Kidney disease usually presents as reduced glomerular filtration rate (GFR) and/or proteinuria which may progress to nephrotic syndrome. (renalfellow.org)
Without treatment, GFR may progressively decline and patients may develop end-stage kidney disease (ESKD). (renalfellow.org)
The polycystic kidney diseases are a group of genetically heterogeneous disorders and a leading cause of kidney failure. (basicmedicalkey.com)
The autosomal dominant form of polycystic kidney disease (ADPKD) is the most common life-threatening monogenic disease, affecting 12 million people worldwide. (basicmedicalkey.com)
The autosomal recessive form of polycystic kidney disease (ARPKD) is rarer but affects the pediatric population. (basicmedicalkey.com)
Kidney cysts are often seen in a wide range of syndromic diseases. (basicmedicalkey.com)
Although cysts only occur in 5% of the tubules in the kidney, the enormous growth of these cysts ultimately leads to the loss of normal surrounding tissues and loss of renal function. (basicmedicalkey.com)

Amyloid deposits are composed of small (about 10 nm diameter), insoluble fibrils that form congophilic beta-pleated sheets that can be identified by x-ray diffraction. (msdmanuals.com)

In-vitro , ex-vivo and in-vivo animal models have been created to study these disorders providing excellent platforms to elucidate pathogenesis and provide insightful information that can be translated to the practice of renal pathology, as the In-vitro and invivo platforms corroborate each other. (heraldopenaccess.us)
[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)

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)

Mesangial injury represents a crucial event in the pathogenesis of light chain-associated glomerulopathies in patients with plasma cell dyscrasias. (heraldopenaccess.us)
Pathogenesis - Clonal plasma cells make monoclonal light chains. (renalfellow.org)

This form of amyloidosis is caused by deposits of beta-2 microglobulin that build up in the blood. (askhematologist.com)

AL is caused by overproduction of an amyloidogenic immunoglobulin light chain in patients with a monoclonal plasma cell or other B cell lymphoproliferative disorder. (msdmanuals.com)

Decreasing production of the organ-damaging light chains is the treatment goal. (wikipedia.org)
The amyloid deposits themselves are metabolically inert but interfere physically with organ structure and function. (msdmanuals.com)
AA amyloid deposits develop in all tissues, but the most commonly damaged organ is the kidneys. (emedicinehealth.com)
Amyloid can deposit in almost any organ system, leading to a wide range of clinical presentations. (renalfellow.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)

In addition to the fibrillar amyloid protein, the deposits also contain serum amyloid P component and glycosaminoglycans. (msdmanuals.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)

Polypeptide chains, consisting of 211 to 217 amino acid residues and having a molecular weight of approximately 22 kDa. (lookformedical.com)

In some patients there is involvement of more than one compartment, but the manifestations usually are centered in one of the renal compartments [1-5]. (heraldopenaccess.us)

A monoclonal population of plasma cells can be detected in the bone marrow, and an altered serum-free light chain ratio is present. (medscape.com)
The repair of the damaged mesangium is difficult due to the absence of enough mesangial cells that can participate in the process and also the damage can be so extensive that the intrinsic processes that are available for repair (i.e. recruitment of stem cells from bone marrow and precursor stem cells in renal niches) cannot effectively carry out the recovery. (heraldopenaccess.us)
Light chain (AL) amyloidosis is the most common type in the U.S. and is caused by a bone marrow disorder. (emedicinehealth.com)

A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. (lookformedical.com)

The glomerulopathic light chains interact with mesangial cells where purported receptors regulate the downstream cellular mechanisms that will be activated and result in glomerular alterations. (heraldopenaccess.us)

The findings that have been elucidated have paved the way for the design of novel therapeutic interventions aimed at stopping, slowing down or avoiding renal damage in patients with plasma cell dyscrasias. (heraldopenaccess.us)

Alzheimer Disease Alzheimer disease causes progressive cognitive deterioration and is characterized by beta-amyloid deposits and neurofibrillary tangles in the cerebral cortex and subcortical gray matter. (msdmanuals.com)

AA amyloidosis affects persons with chronic infections or autoinflammatory diseases and primarily involves the kidneys. (medscape.com)

Deposits can build up in many different tissues, but it most commonly affects bones, joints, and tendons. (askhematologist.com)
This is caused by deposits of normal TTR in the heart and other tissues. (askhematologist.com)

It is a rare disease characterized by deposition of nonamyloid immunoglobulin light chains, which unlike amyloid do not stain with Congo red and do not exhibit a fibrillar structure when examined ultrastructurally. (medscape.com)

The deposition of large antigen-antibody complexes leading to tissue damage causes IMMUNE COMPLEX DISEASES. (lookformedical.com)

About half of people with light chain deposition disease also have a plasma cell dyscrasia, a spectrum of diseases that includes multiple myeloma, Waldenström's macroglobulinemia, and the monoclonal gammopathy of undetermined significance premalignant stages of these two diseases. (wikipedia.org)
It can be a primary manifestation of multiple myeloma or develop as a terminal complication during the disease. (lookformedical.com)

The most common type of amyloidosis and used to be called primary amyloidosis results when light chains are produced in excess by clonal or frankly malignant plasma cells. (askhematologist.com)
Defects in the primary cilia are linked to a wide spectrum of human diseases, collectively termed ciliopathies. (basicmedicalkey.com)
This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. (lookformedical.com)

Many genetic defects are linked to a higher chance of amyloid disease. (askhematologist.com)

Skin is a common site of deposition and can manifest as asymptomatic plaques, fissures or nodules. (askhematologist.com)
1. Amyloid deposits are weakly periodic acid-Schiff (PAS) positive and silver negative (unlike Kimmelstiel-Wilson nodules in diabetes and hyaline deposits in arterioles which are both positive for PAS). (renalfellow.org)

Normal and tubulopathic light chains do not interact with the mesangium thus mesangial homeostasis is maintained intact. (heraldopenaccess.us)

Two Ig light chains and two Ig heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) make one immunoglobulin molecule. (lookformedical.com)

The median time to progression to end stage renal disease is 2.7 years. (wikipedia.org)
The goal of treatment is to relieve symptoms and slow the progression of the disease. (emedicinehealth.com)

Previously known as secondary amyloidosis , this condition is the result of another chronic infectious or inflammatory diseases such as rheumatoid arthritis, Crohn's disease, or ulcerative colitis. (askhematologist.com)

Altri quadri possibili sono l'amiloidosi, la light or heavy chain deposition disease , la sindrome di Fanconi, etc. (giornaleitalianodinefrologia.it)
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)
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)

Anatomy2

Diseases10

Chemicals and Drugs21

Analytical, Diagnostic and Therapeutic Techniques and Equipment5

Phenomena and Processes7

Information Science3

Proteins17

Pathophysiology1

Abnormal7

Tubular3

Granular deposits1

Organs5

Apple-green birefringence under polarized light5

Cell carcinoma5

Lambda10

Microscopy6

Type of amyloid1

Glomerulus2

Immunoglobulins3

LCDD8

Biopsy2

Kidney10

Insoluble fibrils1

Pathology2

Fibrils1

Monoclonal Ig light1

Pathogenesis2

Amyloidosis is caused1

Immunoglobulin light1

Organ4

Subtypes1

Protein2

Polypeptide1

Involvement1

Bone3

Disproportionate1

Glomerular1

Dyscrasias1

Deterioration1

Kidneys1

Tissues2

Fibrillar1

Antibody1

Multiple myeloma2

Primary3

Amyloid disease1

Nodules2

Mesangial1

Molecule1

Progression2

Rheumatoid1

Heavy3