Glomerulonephritisinflammation in the kidneys a patient education tool. Pdf on aug 1, 2001, p m ronco and others published light chain deposition disease. We report the case of a woman in whom restrictive cardiomyopathy due to lcdd presented with paroxysmal atrial fibrillation. This booklet focuses on the plasma cell type of mgus, which comprises 85% of all mgus cases. Technically, light chain deposition disease lcdd is not considered a cancer. Lcdd is a clonal plasma cell proliferative disorder in which fragments of light chains. In conclusion, elastolysis is common to cystic lung light chain deposition disease and other cystic lung disorders, suggesting its implication in cyst formation. It is highly likely that many people have mgus and never know it. Treatment of idiopathic light chain deposition disease. It is recommended for ongoing monitoring of patients with oligosecretory multiple myeloma in which the amount of secreted monoclonal protein is very low, light. Light chain deposition disease is a rare condition, in which the light chains or the infection fighting proteins are deposited in the major organs, mainly kidneys. 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. Even patients with advanced chronic kidney disease ckd. A concurrence of light and heavy chain deposition disease and diabetic nephropathy article pdf available in journal of the medical association of thailand chotmaihet thangphaet 9010.
Lightchain deposition disease lcdd is a rare condition that is characterized by an excessive nonamyloid deposition of light chains in various tissues, which can lead to organ dysfunction. Pdf a concurrence of light and heavy chain deposition. Enlightening light chain deposition disease american society of. Light chain deposition disease lcdd in the lung is a rare occurrence. Monoclonal immunoglobulin deposition disease midd a disease caused by. Light chain deposition disease lcdd light chains usually kappa are deposited in a more haphazard fashion, but most selectively in small blood vessels of the eyes and kidneys. Glossary and abbreviations the iwmf vision statement support everyone affected by waldenstroms macroglobulinemia while advancing the search for a cure. Light chain deposition disease lcdd is one manifestation of monoclonal immunoglobulin deposition disease in the kidney, first reported in 1973 and confirmed by randall et al who published in 1976 the first description of lcdd the incidence of lcdd in patients with plasma cell dyscrasia is approximately 5%. The subtypes are indistinguishable by light microscopy.
Diagnosis and monitoring a case of lightchain deposition disease in the kidney using a new, sensitive immunoassay. Mgus occurs in 3%4% of the population over the age of 50. Unusual presentation of light chain deposition disease. Light chain amyloidosis al and light chain deposition disease lcdd are systemic diseases caused by an abnormal production of. A 55yearold woman was admitted to our emergency department because of palpitations. Dysproteinemias and glomerular disease loyola medicine. We report an unusual histologic manifestation of lcdd in a 55yearold female patient, who presented with nephrotic syndrome and an increased serum creatinine.
People with lcdd make too many light chains, which get deposited in many body tissues. Light chains are used to make antibodies that the body needs to fight infection. Light chain deposition disease loculated pneumothoraces neurofibromatosis type 1 mimics of pulmonary cysts include pulmonary cavities, emphysema, loculated pneumothoraces, honeycomb lung, and bronchiectasis figure 1. Case report renal amyloidosis complicated by light chain. In myeloma cast nephropathy, the monoclonal in this case. Other renal pathologies, such as tma, membranous glomerulopathy and mcd have a less clear relationship with the wm tumor clone. Light chain deposition disease lcdd is a rare disease characterized by deposition of monoclonal nonamyloid light chains in multiple organs. Light chain deposition disease lcdd is characterized by the deposition of monotypic immunoglobulin light chains in the kidney, resulting in renal dysfunction. Lightchain deposition disease lcdd is the most common form of ig deposition disease and has been considered as a contraindication to transplant. Light chain deposition disease lcdd is a rare condition characterized by the deposition of specific proteins monoclonal light chains in the. Light chain deposition disease lcdd is categorized in the family of monoclonal immunoglobulin depo sition diseases midd in the who classification of. However, it shares some characteristics with cancer, and can be associated with certain types of cancer. Light chain deposition disease lcdd is a rare systemic condition caused by monoclonal proliferation of terminally differentiated blymphocytes with production of free light chains and their.
Natural history and outcome of light chain deposition disease ncbi. This protein misfolding disorder can affect the heart, kidney, skin, stomach, small and large intestines, nerves, and liver causing these organs and tissues to thicken and eventually lose function. Diagnosis and monitoring a case of lightchain deposition disease in. To explore the clinicopathologic characteristics and outcomes of hcdd in chinese individuals, we report on a case series assembled in a single center in china. Light chain deposition disease presenting as paroxysmal. If you have problems viewing pdf files, download the latest version. Al amyloidosis amyloid light chain cleveland clinic. Heavy chain deposition disease hcdd is a rare consequence of monoclonal immunoglobulin deposition disease that has not been well characterized in nonwhite populations. The pathologist viewing the biopsy can tell it is light chain deposition disease because it has a different appearance from other deposition diseases.
Same idea as light chain deposition disease but caused by the heavy chains of antibodies heavy chain. Pdf treatment of light chain deposition disease with. Moreover, in cystic lung light chain deposition disease, a role of metalloproteinases in elastolysis is strongly indicated by the metalloproteinase expression and activity pattern. Light chain deposition disease lcdd can involve the heart and cause severe heart failure. The kidneys are almost always affected while heart, liver and other tissues are occasionally involved. Light chain deposition disease genetic and rare diseases. 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. Cardiac involvement is usually described in the advanced stages of the disease. In other deposition diseases, the chains will appear to form a pattern and are found in one particular section of the kidney. Imwg criteria for the diagnosis of myeloma and guidelines.
The lightchain deposition disease lcdd belongs to the family of monoclonal immunoglobulin deposition diseases, caused by a dyscrasia underlying plasma. The iwmf mission statement to offer mutual support and encouragement to the waldenstroms macroglobulinemia community and others with an interest in the disease. Light chain deposition diseasecausessymptomstreatment. A model of glomerulosclerosis defined at the molecular level find, read. Globulin light chains are filtered at the glomerulus can exceed 1020 gramsday and are toxic to the tubular cells, negative dipstick dt globulins not albumin light chain are partially reabsorbed damaging to proximal tubular cells and delivered distally, combing with tammhorsfell protein produced in the thick. Light chain deposition disease genetic and rare diseases nih.
Pathomechanisms of cyst formation in pulmonary light chain. Light chain deposition disease lcdd is a rare plasma cell dyscrasia characterized by deposition of immunoglobulin fragments. Multiple myeloma objectives and mgus of hematopoietic. Generally, the kidney is the most affected organ in lcdd, and liver. As this condition can severely affect the kidneys, it is important to know about the causes, symptoms, treatment and survival rate in light chain deposition disease. In this issue of blood, sayed et al report on a series of 53 patients with light chain lc deposition disease lcdd prospectively followed for a median of 6. It is a rare disease with about 3,000 new cases per year in the united states. Learn about amyloid light chain amyloidosis from cleveland clinic.
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