Role of serum free light chains in diagnosis and monitoring response to treatment in light chain deposition disease. Answers from experts on light chain deposition disease. It should also be done in all patients who have achieved a complete response to treatment to determine whether they have attained a stringent complete response. Light chain deposition disease an overview sciencedirect. The characteristic appearance of light chain deposition disease by light microscopy is that of a nodular glomerulosclerosis, which strongly resembles diabetic nephropathy, as in this case periodic acidschiff stain. They are associated to different forms of renal disease, like nephropathy due to ig deposition or precipitation. It leads to endstage renal disease and has a high disease. Aug 01, 2012 mri features associated with crystal deposition diseases include size, softtissue signal characteristics, arthritic changes, as well as etiology and common locations of such deposition diseases. Median time from disease to progression to end stage kidney disease is 2. Myeloma is common in patients with lcdd and may be present in 60% of cases, and, as with cast nephropathy, poor outcomes have been reported after kidney transplantation. It may cause enlargement of the liver and spleen as well as enlargement of the lymph nodes in the abdomen. Light chain deposition disease lcdd is a monoclonal immunoglobulin deposition disease that commonly affects kidneys among other organs. Mu heavy chain disease igm heavy chain disease, the rarest of the three heavy chain diseases, most often affects people over 50. The light chains are secreted by a plasma clone and deposit in the alveolar walls, small airways, and vessels.
Pdf the morphological spectrum of light chain deposition disease lcdd may range from normal glomerular morphology to mesangioproliferative to. Unusual presentation of light chain deposition disease. Although the clinical presentation suggested amyloid neuropathy, nerve biopsy showed the immunohistochemical and ultrastructural features typical of light chain deposition disease lcdd. Light chain deposition disease lcdd is a systemic disorder characterised by the pathologic deposition of immunoglobulin light chains, which is histologically distinguished from amyloidosis by failure to stain with congo red. It is a rare disease characterized by deposition of nonamyloid immunoglobulin light chains, and they do not stain with congo red and do not exhibit a fibrillar structure when examined ultrastructurally. An elderly but previously healthy patient presents with weakness and fatigue and was found to have a creatinine of 3. Lcs are normally cleared by the kidneys, but in lcdd, these light chain deposits damage organs and cause disease. Light chain disease is a variant of multiple myeloma in which the malignant population of marrow cells produces free monoclonal light chains. The kidneys are almost always affected while heart, liver and other tissues are occasionally involved. The kidneys are almost always affected and this often leads to kidney failure. Light chain deposition disease lcdd is a rare disease. Heavy chain diseases merck manuals consumer version.
Alamyloidosis and lightchain deposition disease light. Kasagi t1, nobata h, suzuki k, miura n, banno s, takami a, yamashita t, ando y, imai h. In patients with light chains deposition disease, the typically kappa ig fragments accumulate in a disorganized. The true incidence of light chain deposition disease lcdd restricted to the. Pdf monoclonal free light chains flcs are important disease biomarkers in patients with plasma cell proliferative disorders. The specific type of midd is diagnosed by immunofluorescence or immunohistochemistry. Light chain deposition disease lcdd is a rare blood cell disease which is characterized by deposition of fragments of infectionfighting immunoglobulins, called light chains lcs, in the body. Light chain deposition disease lcdd is the deposition of monoclonal, amorphous, noncongophilic light chains in multiple organs that do not exhibit a fibrillar structure when examined ultrastructurally. Light chains are used to make antibodies that the body needs to fight infection. Light chain deposition disease lcdd is the deposition of monoclonal light chains in multiple organs. Some patients with light chain deposition disease will make so many that they overwhelm the kidneys ability to recycle or get rid of them all, and some of those proteins get stuck in. Because free light chains flcs are rapidly cleared from the serum and. Primary cns nonamyloidogenic light chain deposition disease.
A 59yearold male was diagnosed with nephrotic syndrome secondary to lightchain deposition disease. Light chain deposition disease pulmonary manifestations. Lightchain deposition disease kidney international. Renal failure due to light chain deposition disease revista nefrologia. Hrct is an important modality in the evaluation of interstitial lung disease to include cystic lung disease.
The pathogenesis of renal injury and treatment in light chain. This article will illustrate and describe the spectrum of diseases associated with air cysts at highresolution ct hrct. While lcdd can occur in any organ, the kidneys are always. Help you understand more about the serum free light chain assay describe the role of the serum free light chain assay in the diagnosis and monitoring of myeloma answer some of the questions you may have about the serum free light chain assay myeloma uk provides a range of infoguides and infosheets on specific treatment. May 10, 2012 light chain deposition disease lcdd is an uncommon, clonal plasma cell proliferative disorder, in which monoclonal immunoglobulin light chains deposit in various tissues, resulting in organ dysfunction. White blood cells defend the body by fighting infection. The heavy chain in hcd is often incomplete or truncated and a sharp, localized peak may not be seen on the electrophoretic. Light chain deposition disease lcdd in the lung is a rare occurrence. Drug class antimetabolite mechanism of action drugs of this class alter rna and dna, building blocks of all cells, resulting in blunting of the immune system. Treatment of light chain deposition disease with bortezomib. Central nervous system cnsrestricted lcdd is among the rarest manifestations.
Understanding serum free light chain freelite assays judith a. Wed like to understand how you use our websites in order to improve them. Heavy chain deposition disease is when kidneys, liver andor heart dysfunction and failure occur due to deposits of pieces of heavy chains. The interaction between light chain deposition disease lcdd free light chain flcs and mesangial cells mcs. Light chain deposition disease diagnosed with laser micro. If you have problems viewing pdf files, download the latest version of adobe reader.
Light chain deposition disease diagnosed with laser microdissection, liquid chromatography, and tandem mass spectrometry of nodular glomerular lesions. Alamyloidosis and light chain deposition disease light chains induce divergent phenotypic transformations of human mesangial cells. Patients with light chain deposition disease make far too many light chains, which get deposited in lots of different tissues in the body, including the kidney. Sep 26, 2019 light chain deposition disease lcdd is the deposition of monoclonal light chains in multiple organs. Light chain deposition disease affecting the gastrointestinal. Light chain deposition disease is a rather uncommon monoclonal gammopathy with predominantly renal manifestations with presence of monoclonal light chains in serum and urine. Technically, light chain deposition disease lcdd is not considered a cancer.
Light chain deposition disease radiology reference article. Other kidney diseases treated with this medication, continued. The disease is caused when a persons antibodyproducing cells do not function properly and produce abnormal protein fibers made of components of antibodies called light chains. Light chain deposition disease lcdd is a rare disease characterized by deposition of monoclonal nonamyloid light chains in multiple organs. It is a rare disease characterized by deposition of nonamyloid immunoglobulin light chains, which do not stain with congo red and do not exhibit a fibrillar structure when examined ultrastructurally. Jan 06, 2012 i have never heard of light chain deposition disease, but i know that some patients dont show other indicators and have to be checked via light chains. Director of scientific affairs the binding site, inc.
Light chain deposition disease lcdd is characterized by immunoglobulin light chains deposits, most commonly kappa k that is neither. Enlightening light chain deposition disease a related article has been published. Amyloid lightchain al amyloidosis, also known as primary amyloidosis, is the most common form of systemic amyloidosis in the us. We report an unusual histologic manifestation of lcdd in a 55yearold female patient, who presented with nephrotic syndrome and an increased serum creatinine.
Tubular injury, interstitial fibrosis, and intact glomeruli. Natural history and outcome of light chain deposition disease splitscreen. Monoclonal light chain and heavy chain deposition diseases. Serial serum free light chain measurement should be routinely performed in patients with al amyloidosis and multiple myeloma patients with oligosecretory disease. Light chain deposit disease lcdd is an uncommon monoclonal gammopathy which should be considered carefully in patients who have both renal disease and a lymphoplasmacytic disorder capable of producing monoclonal light chainsmyeloma, macroglobulinaemia, lymphoma, chronic lymphatic leukaemia 24. Light chain deposition disease lcdd is a rare condition characterized by the deposition of specific proteins monoclonal light chains in the kidneys and other organs. Diseases treated with this medication this medication is primarily used to prevent kidney transplant rejection and to treat severe rheumatoid arthritis. Lightchain deposition disease lcdd patients have a very high risk of recurrence of the monoclonal kappa or lambda chain deposition in the graft. Data on serum free light chains flcs were not available for all patients at the time of diagnosis, as nephelemetric measurements of serum flcs freelite. Diagnosis and monitoring a case of lightchain deposition. Pdf serum free light chains in clinical laboratory diagnostics. Clinical features, immunopathology, and molecular analysis. Aug 02, 2016 light chain deposition disease lcdd is a rare condition characterized by the deposition of specific proteins monoclonal light chains in the kidneys and other organs.
Early treatment has a significant impact on renal survival in light chain deposition disease lcdd. A 59yearold male was diagnosed with nephrotic syndrome secondary to light chain deposition disease. Clinical findings nephrotic syndrome, heart failure, arrhythmias, liver disease, anorexia, nausea, weight loss. There are two major categories of monoclonal ig deposition diseases. Jump to this post im not able to share much about how maintenance therapy velcade monthly affects times between transplants, none of the members of our support group that have had. The heavy chain diseases hcds are rare b cell proliferative disorders characterized by the production of a monoclonal m protein consisting of a portion of the immunoglobulin heavy chain without a bound light chain. Light chain deposition disease lcdd is the most common of the nonal amyloid monoclonal immunoglobulin deposition diseases midd, which also include subtypes with both light and heavy chains or only heavy chain component. Primary idiopathic cns nonamyloidogenic light chain. Natural history and outcome of light chain deposition disease ncbi. A 65yearold man with igg lambda multiple myeloma developed severe polyneuropathy with prominent thermalpain sensory impairment and autonomic failure.
Gastrointestinal gi involvement has been described in both primary and secondary amyloidosis, but has rarely been reported in lcdd, and only as an incidental finding. The monoclonal immunoglobulin ig deposition diseases are closely related disorders characterized by visceral and soft tissue deposition of aberrant ig, resulting in compromised organ function. Pdf download for primary cns nonamyloidogenic light chain. A nephropathy which presents with nephrotic range proteinuria and rapidly progressive renal failure due to deposition of electrondense material within tubular and glomerular basement membranes. Light chain deposition disease accessed 17 january 2018. Monoclonal light chain and heavy chain deposition diseases definition plasma cell neoplasm that secretes immunoglobulin that deposits in tissues and causes tissue damage but does not form an amyloidtype betapleated sheet structure. People with lcdd make too many light chains, which get deposited in many body tissues. Understanding serum free light chain freelite assays. Dec 09, 2008 light chain deposition disease lcdd is a rare plasma cell dyscrasia characterized by deposition of immunoglobulin fragments.
Monoclonal gammopathy characterized by overproduction and deposition of nonamyloid immunoglobulin light chains in various organs. A case of light chain deposition disease lcdd in a young patient article pdf available in indian journal of clinical biochemistry 264. Light chain deposition disease definition of light chain. Light chain deposition disease blood american society. Patients with lcdd are middleaged or older adults, and men are affected more often. Biopsy performed for kidney injury, acute versus chronic. Econimo l, gaggiotti m, ravera s, re a, peli a, tardanico r et al. Light chain deposition disease genetic and rare diseases. Gout gout has a prevalence of about 20 per patients and an annual incidence of 1 to 2 per patients.
Lcdd was first described in 1976 in two patients with endstage renal disease as granular deposits of free light chains in multiple organs. Pdf a case of light chain deposition disease lcdd in a. Pulmonary manifestations of light chain deposition disease are rare when considering the full disease spectrum of light chain deposition disease pathology. However, it shares some characteristics with cancer, and can be associated with certain types of cancer. Light chain deposition disease neuropathy resembling amyloid. Treatment of light chain deposition disease using bortezomib. Light chain deposition disease lcdd is a rare clinical disorder. A case of light chain deposition disease lcdd in a young patient. Light chain deposition disease and light chain proximal. There was no other evidence of a b cell clonal disorder or amyloidosis. He went into remission with undetectable serum free light chains and remained so for three years at the time of writing this report.
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