Conversely, tubular involvement in the form of myelomalike casts or basement membrane deposition of monoclonal light chain lightchain deposition disease is unusual. However, it is rarely encountered as a localized pulmonary disease. Central nervous system cnsrestricted lcdd is among the rarest manifestations. A 59yearold male was diagnosed with nephrotic syndrome secondary to light chain deposition disease. This eosinophilic material corresponds to monotypic immunoglobulin light chains. Cardiac involvement is usually described in the advanced stages of the disease. Dec 24, 2015 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. Light chain deposition disease is often associated with multiple myeloma or lymphoproliferative disease, but as many as 50% of patients have no evidence of neoplastic plasma cell proliferation. The kidneys are almost always affected while heart, liver and other tissues are occasionally involved. Jun 28, 2018 the immunofluorescence in cases of monoclonal immunoglobulin deposition disease is characterized by linear tubular basement membrane staining.
Light chain deposition disease accessed 17 january 2018. The kidneys are almost always affected and this often leads to kidney failure. In these cases, the positive staining is restricted to the monoclonal immunoglobulin leading to disease. Light chain deposition diseasecausessymptomstreatment. Dec 09, 2008 light chain deposition disease lcdd is a rare plasma cell dyscrasia characterized by deposition of immunoglobulin fragments. Background light chain deposition disease lcdd is usually a systemic disorder characterised by nonamyloid monoclonal immunoglobulin light chain deposition in tissues. 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. Lightchain deposition disease lcdd is the most common form of ig deposition disease and has been considered as a contraindication to transplant. Monoclonal immunoglobulin deposition disease light chain deposition disease monoclonal. However, it shares some characteristics with cancer, and can be associated with certain types of cancer. Light chain deposition disease radiology reference article.
While lcdd can occur in any organ, the kidneys are always. In large part this is related to the effects of light chains in other organs and not necessarily due to kidney disease. Light chain deposition disease an overview sciencedirect topics. No vascular immune deposits were identified with the special stains. The deposition of light chain immunoglobulins mainly affects the kidneys, which have different characteristics than other tissues. D renal biopsy from a patient with hc deposition disease. Light chain deposition disease lcdd is a rare clinical disorder. The photomicrographs here show strong staining for kappa compared to lambda in a case of kappa light chain deposition disease. Light chain deposition disease lcdd infrequently affects the lungs and usually causes damage to the parenchyma, while bronchial involvement appears to be very rare. Unusual presentation of light chain deposition disease. Sep 26, 2019 light chain deposition disease lcdd is the deposition of monoclonal light chains in multiple organs. 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. Apr 06, 2020 light chain deposition disease lcdd is a monoclonal gammopathy of clinical significance 1 that is characterized by the formation of unstructured tissue deposits of the monoclonal immunoglobulin light chain.
Patients commonly have an underlying plasma cell dyscrasia, and produce excess levels of monoclonal light chains. Light chain deposition disease lcdd is a rare disease characterized by deposition of monoclonal nonamyloid light chains in multiple organs. Methods we investigated the clinical, radiological and pathological. The pathogenesis of renal injury and treatment in light chain. Pdf on aug 1, 2001, p m ronco and others published light chain. 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. Light chain deposition disease lcdd is a rare condition characterized by extracellular light chain deposition in tissues.
The deposition of light chain immunoglobulins mainly affects the kidneys. Light chain deposition disease an overview sciencedirect. 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. Alamyloidosis and lightchain deposition disease light. The outcome of patients with light chain deposition disease remains uncertain. Light chain deposition disease presenting as paroxysmal. Renal involvement is the most common clinical manifestation. 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. In clinical and pathologic terms, light, light and heavy, and heavy chain deposition. 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 in the lung is a rare occurrence. Cast nephropathy and lightchain deposition disease in. Pdf the pathogenesis of renal injury and treatment in light. To the best of our knowledge, lcdd has not been previously reported from saudi arabia.
Lightchain deposition disease lcdd is caused by an underlying clonal plasma cell dyscrasia in which monoclonal immunoglobulin light chains lcs are deposited in. In support of the proposed change, the 2009 international myeloma working group guidelines. Light chains are used to make antibodies that the body needs to fight infection. Light chain deposition disease involving the airways. 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. Lightchain deposition disease kidney international. Fig 5 glomerular capillary loop, mesangial staining, and linear tubular staining are characteristic of light chain deposition disease.
This study reports the characteristic clinical ocular findings seen in advanced lcdd upon development of ocular fundus changes. We present in this report, a 38yearold saudi male who presented with clinical features suggestive of hypertensive nephropathy but kidney biopsy later revealed the diagnosis of 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. A 55yearold woman was admitted to our emergency department because of palpitations.
A case of atypical light chain deposition diseasediagnosis and. Backgroundpurpose light chain deposition disease lcdd is a rare condition characterised by deposition of monoclonal immunoglobulin light chains lcs in tissues, resulting in varying degrees of organ dysfunction. Lcs are normally cleared by the kidneys, but in lcdd, these light chain deposits damage organs and cause disease. Pdf light chain deposition disease is a rather uncommon monoclonal. Patient survival from the time that symptoms begin has ranged from 1 month to 10 years. A nodular lightchain deposition disease arrows, focus of tubular atrophy and interstitial fibrosis arrowhead with thickening and vivid periodic acidschiff stain of the basement membrane of the tubules at the same area, small epithelial cyst asterisk pas.
Biopsyproven resolution of renal lightchain deposition. Light chain deposition disease lcdd is a rare illness with, as yet, no clear evidencebased guidelines for its treatment. Alamyloidosis and light chain deposition disease light chains induce divergent phenotypic transformations of human mesangial cells. Light chain deposition disease lcdd is a rare disease. Pulmonary manifestations of light chain deposition disease. Dec 29, 2007 light chain deposition disease lcdd can involve the heart and cause severe heart failure. Among the systemic diseases associated with immunopro. Immunoglobulin light heavychain deposition disease. Light, light and heavy, and heavy chain deposition diseases belong to a family of diseases that include light chain alamyloid, nonamyloid fibrillary and immunotactoid glomerulonephritis, and cryoglobulinemic glomerulonephritis, in which monoclonal ig or their subunits become deposited in kidney. Light chain deposition disease is nephropathy caused by tissue deposition of the constant component of monotypic immunoglobulin light chains and producing. Monoclonal gammopathy characterized by overproduction and deposition of nonamyloid immunoglobulin light chains in various organs.
Pulmonary cystic disorder related to light chain deposition. Clinical features, immunopathology, and molecular analysis. Light chain deposition disease arkana laboratories. Lcdd is recognized as a multisystem disease, in which the kidneys and liver are often affected. Ten months after excision of a poorly differentiated vaginal carcinoma, a routine chest computed tomography ct. To date, the therapeutic approach for the treatment of lcdd has no evidencebased consensus, and clinical experience of reported cases guides current disease management strategies. Light, light and heavy, and heavychain deposition diseases belong to a family of diseases that. A condition in which plasma cells secrete immunoglobulin light chains of only one type, kappa or lambda. Light chain deposition disease genetic and rare diseases. Treatment of light chain deposition disease with bortezomib. This is the first report to describe this entity in. 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. Retinal pigment epithelial detachments and tears, and. Oct 17, 2005 light chain deposition disease lcdd, a rare disease recognized in 1976 by randall, is characterized by the deposition of a nonfibrillary, amorphous material that does not have.
Light chain deposition disease lcdd is a systemic disorder typically characterized by nonamyloid monoclonal immunoglobulin light chain deposition in tissues. Jun 21, 2012 conversely, tubular involvement in the form of myelomalike casts or basement membrane deposition of monoclonal light chain lightchain deposition disease is unusual. People with lcdd make too many light chains, which get deposited in many body tissues. Aug 02, 2016 technically, light chain deposition disease lcdd is not considered a cancer. Diagnosis and monitoring a case of lightchain deposition. There was no other evidence of a b cell clonal disorder or amyloidosis. 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. A case of atypical light chain deposition diseasediagnosis. Midd are characterised by nonamyloid deposition of immunoglobulin in various organs leading to dysfunction. We report the occurrence of cast nephropathy associated with lightchain deposition disease in 2 patients with waldenstrom macroglobulinemia, which resulted in severe and. 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. Monoclonal immunoglobulin deposition occurs in tissues as congo red binding fibrils in light chain amyloidosis, as less structured deposits in light chain deposition disease, and as similar but distinct deposits in light and heavy chain deposition disease.
We report the case of a woman in whom restrictive cardiomyopathy due to lcdd presented with paroxysmal atrial fibrillation. Treatment of light chain deposition disease using bortezomib. Light chain deposition disease lcdd is the most common form of ig deposition disease and has been considered as a contraindication to transplant. We report an unusual histologic manifestation of lcdd in a 55yearold female patient, who presented with nephrotic syndrome and an increased serum creatinine. Alamyloidosis and lightchain deposition disease light chains induce divergent phenotypic transformations of human mesangial cells. Either kappa or lambda light chain paraprotein may cause light chain deposition disease, although kappa more commonly is the culprit antibody to kappa light chain, immunofluorescence. Fulltext pdf coexistence of myeloma cast nephropathy, light chain deposition disease, and nonamyloid fibrils in a patient with multiple. Portal hypertension related to light chain deposition disease. Primary idiopathic cns nonamyloidogenic light chain. Light chain deposition disease american journal of kidney. Nov 25, 2019 light chain deposition disease lcdd is a rare clinical disorder. The present authors report the case of a 64yrold female with lcdd characterised by asymptomatic airway involvement.