• LCDD should be distinguished from Fanconi syndrome, myeloma cast nephropathy, cryoglobulinemia, and amyloidosis, all of which are also associated with monoclonal proteins and may present similarly. (medscape.com)
  • Amyloid light-chain (AL)-amyloidosis consists predominantly of lambda light chains, whereas kappa light chains are predominantly involved in LCDD. (medscape.com)
  • AL amyloidosis is an immunoglobulin light chain associated amyloidosis that involves the deposition of misfolded amyloid light chain (AL) immunoglobulins. (wikipedia.org)
  • Other types of amyloidosis related MGRS disorders include heavy chain amyloidosis (deposition of only heavy amyloid chains in the kidney)(AH) and heavy and light chain amyloidosis (AHL)(deposition of heavy and light chains). (wikipedia.org)
  • Light microscopy findings in AL, AH or AHL amyloidosis include acellular deposits in the glomeruli and blood vessels that stain pale eosinophilic, Congo red positive and the presence of apple green birefringence on polarized light. (wikipedia.org)
  • Founded on the secretion of a monoclonal immunoglobulin (M-protein), plasma cell proliferative disorders are classified as monoclonal gammopathies and include multiple myeloma (MM), Waldenström's macroglobulinemia (WM), amyloidosis (AL), light chain deposition disease (LCDD), POEMS syndrome, and premalignant diseases such as monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM). (degruyter.com)
  • Standard treatment of L chain-type amyloidosis aims to reduce production of the monoclonal immunoglobulin precursor via chemotherapy or, occasionally, via radiation therapy or surgical resection of a localized amyloidogenic plasmacytoma. (medscape.com)
  • Chemotherapy is based on the principle that, as in myeloma, proliferation of a plasma cell clone causes L chain-type amyloidosis. (medscape.com)
  • Many more studies have been published on the treatment of myeloma than of L chain-type amyloidosis, because myeloma is more common and the response to therapy can be more easily monitored. (medscape.com)
  • In L chain-type amyloidosis, determining the response to therapy is difficult and requires indirect measurements of end-organ damage, serial biopsies, or serial P component scans when available. (medscape.com)
  • After melphalan and prednisone were demonstrated to be useful for myeloma, the regimen was tried for L chain-type amyloidosis. (medscape.com)
  • [ 13 , 14 ] Taken together, these studies demonstrated a survival benefit of melphalan and prednisone compared with placebo in L chain-type amyloidosis. (medscape.com)
  • Many experts consider melphalan plus prednisone to be standard therapy for L chain-type amyloidosis for patients not enrolled in a clinical trial, and it is the only regimen that has been shown to prolong survival compared with no chemotherapy. (medscape.com)
  • [ 15 ] Nevertheless, other chemotherapeutic regimens used for multiple myeloma are also expected to benefit patients with L chain-type amyloidosis and are reasonable therapeutic options for this disease. (medscape.com)
  • Improvement can occur in nearly any organ, but improvement in L chain-type amyloidosis neuropathy is rare. (medscape.com)
  • In two studies of patients with L chain-type amyloidosis treated with melphalan-containing regimens, 5% of patients developed myelodysplasia within 3 years of treatment. (medscape.com)
  • Primary systemic amyloidosis (AL) is characterized by multiorgan deposition of monoclonal immunoglobulin light chain. (elsevierpure.com)
  • Amyloidosis is any of a group of disparate conditions characterized by extracellular deposition of insoluble fibrils composed of misaggregated proteins. (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)
  • In immunoglobulin light chain amyloidosis (AL), each patient has a unique monoclonal immunoglobulin light chain (LC) that forms amyloid deposits. (elsevierpure.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)
  • 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)
  • 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)
  • 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)
  • AL amyloidosis often occurs in persons with monoclonal gammopathy and typically affects the heart and kidneys, although the peripheral and autonomic nervous systems, gastrointestinal tract, and other organs may be involved. (medscape.com)
  • AL amyloidosis, or primary systemic amyloidosis, results when plasma cells from bone marrow overproduce light chain proteins that misfold and form chains, proteins that in turn build up in the bloodstream, deposit on organs and eventually interrupt normal function to the point of organ failure. (drugdiscoverynews.com)
  • PRX004, for its part, targets and clears misfolded (toxic) forms of the TTR amyloid protein found in transthyretin-mediated amyloidosis (ATTR amyloidosis), a rare, progressive disease characterized by the deposition of aggregates of misfolded amyloid. (drugdiscoverynews.com)
  • This disease used to be called SSA or SCA, which stood for Senile Systemic Amyloidosis and Senile Cardiac Amyloidosis, respectively, which are now outdated terminologies. (amyloidosis.org)
  • Deposits of TTR amyloid can be found throughout the body, so it is a systemic amyloidosis disease. (amyloidosis.org)
  • It is often overlooked as an amyloidosis disease because so many people experience heart problems in their later years. (amyloidosis.org)
  • On the other hand, a patient with AL (light chain) Amyloidosis who shows cardiomyopathy symptoms will often be treated with chemotherapy - and this treatment is harmful to wild-type ATTR patients with cardiomyopathy. (amyloidosis.org)
  • These scans need to be coupled with blood tests to evaluate for the substrate for AL amyloid (e.g. light chains) and in the absence of any evidence of light chains and a positive scan, are able to diagnose ATTR cardiac amyloidosis. (amyloidosis.org)
  • However, if there is evidence of light chains on blood testing, then a biopsy is still needed to establish the diagnosis and type of amyloidosis. (amyloidosis.org)
  • [3] The proteinopathies include such diseases as Creutzfeldt-Jakob disease and other prion diseases , Alzheimer's disease , Parkinson's disease , amyloidosis , multiple system atrophy , and a wide range of other disorders. (wikipedia.org)
  • They show that plasma cells from patients with light-chain amyloidosis (AL) most closely resemble secondary lymphoid organ plasma cells (SLO-PCs), whereas multiple myeloma (MM) cells are closer to peripheral blood plasma cells (PB-PCs) and newborn bone marrow plasma cells (BM-PCs). (ashpublications.org)
  • [ 20 ] Mesangial nodularity within the glomerulus occurs from the increased deposition of extracellular matrix proteins mixed with kappa light chains. (medscape.com)
  • Monoclonal gammopathy of renal significance (MGRS) are a group of kidney disorders that present with kidney damage due to nephrotoxic monoclonal immunoglobulins (M proteins) secreted by clonal plasma cells or B cells. (wikipedia.org)
  • There are several separate conditions designated as MGRS which are associated with kidney damage (either directly or indirectly) due to monoclonal immunoglobulins (M proteins). (wikipedia.org)
  • In monoclonal gammopathies, M-proteins are visualized as a restricted area of migration in the electrophoretic pattern which can be gated and quantified [ 4 ]. (degruyter.com)
  • However, some prefibrillar oligomers of amyloidogenic proteins have direct cellular toxicity, an important component of disease pathogenesis. (msdmanuals.com)
  • 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)
  • At least 36 proteins have been identified in humans, with 17 showing systemic involvement and the rest presenting as localized diseases. (medscape.com)
  • Monoclonal IgD-was not really measurable on serum proteins electrophoresis, and total IgD level had not been measured at period of diagnosis. (isct-eu2018.com)
  • Dialogue Monoclonal gammopathy includes a heterogeneous band of disorders seen as a clonal proliferation of Ig creating B lymphocytes or plasma cells.5,6 The proliferating cells secrete Ig, which may be detected in the blood or urine as monoclonal Ig (M proteins). (isct-eu2018.com)
  • The M proteins usually includes a weighty string and a light string ( em /em - or em /em -light chains), although occasionally, the neoplastic cells might synthesize just the heavy or light chain alone. (isct-eu2018.com)
  • 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)
  • proteinopathic adj ), or proteopathy , protein conformational disorder , or protein misfolding disease , is a class of diseases in which certain proteins become structurally abnormal, and thereby disrupt the function of cells , tissues and organs of the body. (wikipedia.org)
  • [9] Subsequent research has shown that many different proteins can form amyloid, and that all amyloids show birefringence in cross- polarized light after staining with the dye Congo red , as well as a fibrillar ultrastructure when viewed with an electron microscope . (wikipedia.org)
  • Excessive amounts of IgM M-proteins (monoclonal immunoglobulin protein, which may consist of both heavy and light chains or of only one type of chain) can also accumulate in other disorders, causing manifestations similar to macroglobulinemia. (merckmanuals.com)
  • Less commonly, patients develop hyperviscosity due to the large amounts of high molecular weight monoclonal IgM proteins circulating in plasma, but most patients do not develop problems related to high IgM levels. (merckmanuals.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)
  • Antibodies found in adult RHEUMATOID ARTHRITIS patients that are directed against GAMMA-CHAIN IMMUNOGLOBULINS. (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)
  • Those three candidates are also monoclonal antibodies, though all are being advanced against different indications. (drugdiscoverynews.com)
  • Treatment includes plasma exchange as needed for hyperviscosity and systemic therapy with alkylating drugs, corticosteroids, nucleoside analogs, Bruton's tyrosine kinase (BTK) inhibitors, venetoclax , or monoclonal antibodies. (merckmanuals.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)
  • A panel of assays combining SPEP with IFE/ISUB, serum free light chains (FLC), and urine electrophoresis and IFE/ISUB has the highest degree of clinical sensitivity for the diagnosis of these disorders [ 6 ]. (degruyter.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)
  • 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)
  • 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)
  • In myeloma, the level of serum or urine monoclonal protein usually serves as a quantitative marker of tumor burden. (medscape.com)
  • In all these, light chains purified from the urine of patients with renal biopsy-proven conditions have been used. (heraldopenaccess.us)
  • Serum immunofixation revealed an IgA lambda monoclonal band despite normal findings on serum and urine protein electrophoresis. (medscape.com)
  • A 24-hour urine collection revealed the current presence of a monoclonal (IgD) C area (typical spectra amount of 58) and moderate spectra amounts of elements of complement elements, Isoliensinine indicating accumulation and activation of the different parts of the classic and terminal pathways of enhance. (isct-eu2018.com)
  • Paraprotein is the presence of a monoclonal immunoglobulin band (M-band) in the serum. (emedicodiary.com)
  • These AL light chains are misfolded leading to the disordered deposition of amyloid multimers and fibrils in the glomeruli and blood vessels of the kidney. (wikipedia.org)
  • As with hereditary forms of the disease (hATTR) wild-type ATTR causes problems due to the breaking apart, misfolding and deposition of amyloid protein fibrils in healthy tissue. (amyloidosis.org)
  • In the first case, the clonal plasma cells do not accumulate but induce the deposition of light chains in various organs as amyloid fibrils. (ashpublications.org)
  • The deposition of amyloid fibrils is independent of the tumor burden, which is true for all monoclonal gammopathies of clinical significance. (ashpublications.org)
  • People with MGUS have a monoclonal gammopathy (presence of monoclonal immunoglobulins) but does not meet the criteria for the clonal burden nor the presence of end organ damage seen in hematologic malignancies. (wikipedia.org)
  • Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) involves monoclonal immunoglobulins (usually IgG) depositing in the glomeruli and activating compliment leading to glomerular inflammation. (wikipedia.org)
  • [ 10 ] Approximately 50-60% of patients with LCDD have associated multiple myeloma and 17% have monoclonal gammopathy of unknown significance (MGUS) or no evidence of neoplastic plasma cell proliferation. (medscape.com)
  • MGRS is associated with monoclonal gammopathy of undetermined significance (MGUS). (wikipedia.org)
  • The prevalence and incidence of MGRS in the general population or in specific populations is not known but it is more prevalent in those over the age of 50 as there is a monoclonal protein (M-protein) present in 3% of those 50 and years older and 5% of those 70 years and older (the presence of MGUS), placing those 50 and older at increased risk of MGRS. (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)
  • [ 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)
  • Autologous stem cell transplantation (SCT) for AL achieves superior response rates compared to chemotherapy alone but patients with end-stage renal disease (ESRD) may be excluded from consideration. (elsevierpure.com)
  • in end-stage renal disease ( 1-3 ). (who.int)
  • Multiple myeloma is a cancer of plasma cells that produce monoclonal immunoglobulin and invade and destroy adjacent bone tissue. (merckmanuals.com)
  • In myeloma, plasma cells produce monoclonal (M) Ig of a single heavy and light chain commonly referred to as a paraprotein. (emedicodiary.com)
  • Using therapeutic monoclonal antibody-spiked sera and a pooled beta-migrating M-protein, we aimed to assess SPEP limitations and variability across 16 laboratories in three continents. (degruyter.com)
  • The deposition of large antigen-antibody complexes leading to tissue damage causes IMMUNE COMPLEX DISEASES. (lookformedical.com)
  • DUBLIN- Prothena Corp . is changing tack on its pipeline in the wake of PRX003, a monoclonal antibody being advanced in psoriasis, failing to provide a sufficiently significant clinical benefit in a Phase 1b study to support further advancement. (drugdiscoverynews.com)
  • Micrograph of a section of the cerebral cortex from a person with Alzheimer's disease , immunostained with an antibody to amyloid beta (brown), a protein fragment that accumulates in amyloid plaques and cerebral amyloid angiopathy . (wikipedia.org)
  • Multiple myeloma (MM) is a plasma cell malignancy in which monoclonal plasma cells proliferate in bone marrow, resulting in an overabundance of monoclonal paraprotein (M protein), destruction of bone, and displacement of other hematopoietic cell lines. (medscape.com)
  • Light-chain deposition disease (LCDD) is the deposition of monoclonal light chains in multiple organs. (medscape.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)
  • 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)
  • A single clonal plasma cell is responsible for the overproduction of kappa chains and more rarely, lambda light chains. (medscape.com)
  • Each Ig molecules have either 2 kappa or 2 lambda light chains. (emedicodiary.com)
  • 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)
  • 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)
  • 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)
  • Non-Hodgkin Lymphomas Non-Hodgkin lymphomas are a heterogeneous group of disorders involving malignant monoclonal proliferation of lymphoid cells in lymphoreticular sites, including lymph nodes, bone marrow, the. (merckmanuals.com)
  • Primary sites of disease include peripheral blood, bone marrow. (merckmanuals.com)
  • A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. (lookformedical.com)
  • 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)
  • The excess light chains are secreted into the extracellular fluid and readily pass through the glomerulus. (emedicodiary.com)
  • Background: The amyloidoses are protein misfolding diseases characterized by the deposition of amyloid that leads to cell death and tissue degeneration. (elsevierpure.com)
  • By comparing these physiological TPs, the authors show that tumor PCs of 46 MM, 37 AL, and 6 monoclonal gammopathies of undetermined significance express signatures from SLO-PCs, PB-PCs, newborn BM-PCs, and long-lived BM-PCs. (ashpublications.org)
  • Most kidney diseases associated with MGRS are glomerular disorders. (wikipedia.org)
  • Monoclonal immunoglobulin deposition disease (MIDD) involves light chains with unusual characteristics of the variable domain (such as a positive charge, abnormal glycosylation, hydrophobic residues) depositing in the tubular, vascular or glomerular basement membranes of the nephron. (wikipedia.org)
  • On light microscopy MIDD is seen as a nodular glomerulosclerosis with thickening of the tubular basement membrane with linear deposits along the glomerular, tubular vascular basement membranes seen on immunofluorescence. (wikipedia.org)
  • 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)
  • 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)
  • Serum protein electrophoresis (SPEP) is used to quantify the serum monoclonal component or M-protein, for diagnosis and monitoring of monoclonal gammopathies. (degruyter.com)
  • The origin of monoclonal gammopathies is an old and never resolved debate: is it circulating postgerminal center B cells or long-lived BM-PCs? (ashpublications.org)
  • Its pathophysiology includes immune complex deposition and a wide range of skin lesions. (lookformedical.com)
  • As awareness of the disease increases, wild-type ATTR average age at diagnosis is 75. (amyloidosis.org)
  • Mesangial injury represents a crucial event in the pathogenesis of light chain-associated glomerulopathies in patients with plasma cell dyscrasias. (heraldopenaccess.us)
  • 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)
  • INTRODUCTION: Light chain deposition disease is a systemic disorder characterised by tissue deposition of monoclonal immunoglobulin light chains without tinctorial properties. (hal.science)
  • The term MGRS was introduced in 2012 by the International Kidney and Monoclonal Gammopathy Research Group (IKMG). (wikipedia.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)
  • Kidney Biopsy Analysis The kidney biopsy analysis was IgD heavy-chain deposition disease. (isct-eu2018.com)
  • 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)
  • Regimens most likely to benefit patients with this disease are the same as those that are useful for myeloma (eg, melphalan plus prednisone). (medscape.com)
  • Myeloma is a disease of middle and old age (50-70 years), male predominance. (emedicodiary.com)
  • After myeloma, macroglobulinemia is the 2nd most common malignant disorder associated with a monoclonal gammopathy. (merckmanuals.com)
  • Due to the wide range of disease presentations, identification of the M-protein by serum protein electrophoresis (SPEP) may be the first clue to the diagnosis of a monoclonal gammopathy followed by subsequent isotyping via immunofixation (IFE) or immunosubtraction (ISUB). (degruyter.com)
  • MGRS disorders also have a greater than 90% rate of recurrence if the monoclonal gammopathy is not eliminated either before or immediately after a renal transplant. (wikipedia.org)
  • Parkinson's disease (PD) is one of the most common movement disorders characterized by decreased of dopaminergic neurons. (nature.com)
  • Used using the renal biopsy results collectively, the analysis was supported by the info of IgD heavy-chain deposition disease. (isct-eu2018.com)
  • Nerve disease commonly involves peripheral sensory and motor nerves and autonomic nerves. (aan.org.au)
  • This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. (lookformedical.com)
  • Defects in the primary cilia are linked to a wide spectrum of human diseases, collectively termed ciliopathies. (basicmedicalkey.com)
  • CASE REPORT: We report a case of light chain deposition disease associated with plasma cell dyscrasia presenting as sicca syndrome with salivary glands hypertrophy and polyneuropathy successfully treated by high dose melphalan and autologous blood stem transplantation. (hal.science)
  • Plasma cells are derived from B lymphocytes and produce immunoglobulin (Ig) which contains heavy and light chains. (emedicodiary.com)
  • Normally Ig molecule consists of 4 polypeptide chains 2 heavy chains and 2 light chains. (emedicodiary.com)
  • Malaria remains one of the world's deadliest infectious diseases, and in 2021 was responsible for 241 million clinical infections and 627,000 deaths worldwide (WHO, 2021), primarily among young children in sub-Saharan Africa. (nature.com)
  • As for PRX002, it targets α-synuclein, the protein whose misfolded aggregates are implicated in Parkinson's disease. (drugdiscoverynews.com)
  • Prothena announced the launch of a Phase 2 study of PRX002 in individuals with early Parkinson's disease on July 5. (drugdiscoverynews.com)
  • Iron deposits are neuropathological hallmark of Parkinson's disease (PD). (nature.com)
  • Light microscopy findings in LCPT include proximal tubular swelling with electron microscopy findings showing proximal tubule light chain crystals or lysosomal inclusions. (wikipedia.org)
  • Light microscopy shows a membranoproliferative, endocapillary proliferative or membranous glomerulonephropathy with electron dense deposits in the glomeruli being present on electron microscopy. (wikipedia.org)
  • 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)
  • LMD/MS. Light microscopy displaying (A) glomeruli designated for dissection and (B) clear space after microdissection (hematoxylin and eosin, 20). (isct-eu2018.com)
  • this causes the light chains to form intracytoplasmic aggregates in the proximal tubule of the nephron. (wikipedia.org)
  • AL sequences from patients with different levels of secreted light chain have distinct differences in the location of non-conservative mutations, suggesting that for patients with very low levels of light chains and advanced amyloid deposition, the location of non-conservative mutations rather than the amount of free light chain in circulation may determine the amyloidogenic propensity of light chains. (elsevierpure.com)
  • 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)
  • Altri quadri possibili sono l'amiloidosi, la light or heavy chain deposition disease , la sindrome di Fanconi, etc. (giornaleitalianodinefrologia.it)
  • The physicochemical and conformational characteristics of the abnormal light chains are primarily responsible for the downstream events affecting the mesangial milieu. (heraldopenaccess.us)