Patient survival from the time that symptoms begin has ranged from 1 month to 10 years. While lcdd can occur in any organ, the kidneys are always involved. The deposits are derived from monoclonal light chains only in lcdd, monoclonal light and heavy chains. Persons with light chain disease may develop lytic bone lesions, hypercalcemia, impaired kidney function, and amyloidosis. Light chain deposition disease an overview sciencedirect. Aug 15, 2018 renal failure, bone disease, and systemic light chain al amyloidosis appear to be more frequent in patients with light chain multiple myeloma lcmm. While lcdd can occur in any organ, the kidneys are always. 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. Approximately 500 mg of free light chains is produced by the normal lymphoid system 80% of flc is extravascular two thirds of light chain production is kappa. Lcmm has an earlier average age of onset and appears to have a poorer prognosis when compared to igg or iga variant 3, 17. Amyloidosis information a general overview for patients. Technically, light chain deposition disease lcdd is not considered a cancer.
Bone marrow can be deferred in patients with lowrisk monoclonal gammopathy of undetermined signi. There was no other evidence of a b cell clonal disorder or amyloidosis. If there is more of one type of light chain than the other, the ratio will be different, which can be a sign of myeloma. A model of glomerulosclerosis defined at the molecular level find, read. It occurs in older children and young adults and is associated with gastrointestinal symptoms, such as malabsorption, intestinal obstruction, and diarrhea. 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. A precise morphologic and clinical description of lcdd. Light chain cast nephropathy myeloma kidney is a disease resulting from acute tubular injury fig. Measure of disease and treatment response in light chain multiple myeloma patients using urine and serum assays. Less than 5% of patients with light chain amyloidosis involving the heart have isolated cardiac involvement and it is the presence of associated noncardiac symptoms that will point to a systemic disease rather than a purely cardiac pathology. Diagnosis and monitoring a case of lightchain deposition. A 59yearold male was diagnosed with nephrotic syndrome secondary to lightchain deposition disease. Light chain deposition disease lcdd is characterized by the deposition of monotypic immunoglobulin light chains in the kidney, resulting in renal dysfunction. Systemic light chain deposition disease presenting as multiple pulmonary nodules.
Lightchain deposition disease lcdd is the most common form of ig deposition disease and has been considered as a contraindication to transplant. Symptoms can be related to the disease as it affects your body as a whole, such as weakness and fatigue, weight loss, bone pain, or numbnesstingling of your arms or legs. Neurofilament light chain in blood and csf as marker of. Light chains are used to make antibodies that the body needs to fight infection. People with lcdd make too many light chains, which get deposited in many body tissues.
Light chain deposition disease neuropathy resembling. Immunoglobulin light chain al amyloidosis previously referred to as primary amyloidosis, light chain deposition disease lcdd, and heavy chain deposition disease hcdd are monoclonal plasma cell proliferative disorders that are characterized by tissue deposits of light chain or heavy chain fragments, leading to organ dysfunction. Normally, they are present in equal amounts in the blood, giving a ratio of 1 to 1. Al amyloidosis amyloid light chain cleveland clinic. The median survival from diagnosis was 30 mo for 52 patients with kappalcd and 10 mo for 45 patients with lambdalcd p less than 0. Light chain deposition disease lcdd is a rare plasma cell dyscrasia characterized by deposition of immunoglobulin fragments. 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. Pdf on aug 1, 2001, p m ronco and others published light chain deposition disease. Light chain disease definition of light chain disease by. The kidneys are almost always affected and this often leads to kidney failure. Natural history and outcome of light chain deposition.
Light chain deposition disease lcdd is the most common form of ig deposition disease and has been considered as a contraindication to transplant. Light andor heavy chain deposition disease, high dose therapy, autologous stem cell transplantation. A case of cardiac light chain deposition disease in a patient. In large part this is related to the effects of light chains in other organs and not necessarily due to kidney disease. It is a malignant disease characterized by abnormal proliferation of plasma cells and monoclonal immunoglobulins or free light chains flc. Light chain deposition disease pulmonary manifestations. Lightchain deposition disease kidney international. View the article pdf and any associated supplements and figures for a period of 48 hours. Lcs are normally cleared by the kidneys, but in lcdd, these light chain deposits damage organs and cause disease. 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.
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. A typical antibody is composed of two immunoglobulin ig heavy chains and two ig light chains. Lcdd is a clonal plasma cell proliferative disorder in which fragments of light chains. Renal involvement is the most common clinical manifestation. Alpers, md2 clinical and pathologic features light chain deposition disease lcdd is the most common of the nonal amyloid monoclonal immunoglobulin deposition diseases midd. Each year approximately 30005000 new cases of light chain al amyloidosis are diagnosed in the united states, with many more cases of agerelated and inherited transthyretin amyloidosis attr also diagnosed. Learn about amyloid light chain amyloidosis from cleveland clinic. Aug 16, 2018 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.
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. However, it shares some characteristics with cancer, and can be associated with certain types of cancer. Monoclonal immunoglobulin deposition disease light chain deposition disease monoclonal. 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. Natural history and outcome of light chain deposition disease.
Systemic manifestations include weight loss, easy bruising, brittle and. Cardiac involvement of light chain deposition disease, also known as cardiac nonamyloidotic immunoglobin deposition disease cidd, is a rare clinical entity, where clinical outcome is very variable and best treatment approaches are not well known. Ninetyseven patients with light chain disease lcd were studied. Serum free light chain assay14 pages 1112 kappa free light chain 0. However, when there is light chain restriction to a single light chain isotype e.
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. 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 is a rather uncommon monoclonal gammopathy with predominantly renal manifestations with presence of monoclonal light chains in serum and urine. The immunoglobulin light chain is the small polypeptide subunit of an antibody immunoglobulin. Light chain disease is a variant of multiple myeloma in which the malignant population of marrow cells produces free monoclonal light chains but no heavy chain or complete immunoglobulin. Multiple myeloma early detection, diagnosis, and staging. The presented rare form of double myeloma disease with initial renal insufficiency underscores the importance of careful observation and teamwork that can alter the course of this serious disease. A case of atypical light chain deposition diseasediagnosis and. Patients with lcdd are middleaged or older adults, and men are affected. Light chain deposition disease genetic and rare diseases.
Amyloidosis is generally a disease of middleaged people and older, although the disease has been seen in individuals in their thirties. It is recommended for ongoing monitoring of patients with oligosecretory multiple myeloma in which the amount of secreted monoclonal protein is very low, light. 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. Castleman disease is an uncommon and heterogeneous lymphoproliferative disorder for which management is rapidly evolving. Natural history and outcome of light chain deposition disease ncbi. Hcd, mediterranean lymphoma is considered a variant of malttype lymphoma. Differentiating immunoglobulin lightchain al from transthyretinrelated ttr cardiac amyloidoses is imperative given the implications for prognosis, therapy, and genetic counseling. Patients commonly have an underlying plasma cell dyscrasia, and produce excess levels of monoclonal light chains. The outcome of patients with light chain deposition disease remains uncertain.
Among the systemic diseases associated with immunopro liferative syndromes is a recently identified lightchain depo sition disease lcdd li, 21. Pulmonary manifestations of light chain deposition disease. 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. Serial serum free light chain measurement should be routinely performed in patients with al amyloidosis and multiple myeloma patients with oligosecretory disease. Light chain deposition diseasecausessymptomstreatment. L l f flc i t d ith th b flevels of flcs are associated with the number of. Pulmonary manifestations of light chain deposition disease are rare when considering the full disease spectrum of light chain deposition disease pathology. A condition in which plasma cells secrete immunoglobulin light chains of only one type, kappa or lambda. Light chain deposition disease lcdd a systemic disorder that involves the immune system, lcdd is caused by an excess buildup of immunoglobulin light chains in the tissues and organs.
Monoclonal gammopathy characterized by overproduction and deposition of nonamyloid immunoglobulin light chains in various 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. See also gammopathy, heavy chain disease, multiple myeloma. 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. Patients with lcdd are middleaged or older adults, and men are affected more often. 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. Diagnosis and management of castleman disease jacob d. 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.
Treatment generally involves chemotherapy, corticosteroids and, in some cases, radiation therapy. The symptoms a person has vary based on the reason for the light chain production. Maia,1,2,4,10 oliver preische,1,5 juliane schelle,1,2,3 anja apel,1,6 stephan a. 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. In 2015, an estimated 28,850 new cases of multiple myeloma were.
Pdf a case of light chain deposition disease lcdd in a. The kidneys are almost always affected while heart, liver and other tissues are occasionally involved. Castleman disease is an uncommon lymphoproliferative disorder characterized as either unicentric. A 65yearold man with igg lambda multiple myeloma developed severe polyneuropathy with prominent thermalpain sensory impairment and autonomic failure. This issues inaugural conference on light chain deposition disease is discussed by david salant, vaishali. Monoclonal immunoglobulin deposition disease is a group of multisystem disorders characterized by deposition of monoclonal immunoglobulin light or heavy chains in various organs. 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. Light chain deposition disease lcdd is a rare illness with, as yet, no clear evidencebased guidelines for its treatment. Light chain deposition disease lcdd is a rare condition characterized by extracellular light chain deposition in tissues. It is recommended for ongoing monitoring of patients with oligosecretory multiple myeloma in which the amount of secreted monoclonal protein is very low, light chain myeloma, light chain al amyloidosis, and lcdd. International myeloma working group updated criteria for. We validated the discriminatory ability of 99m tcpyrophosphate scintigraphy in al versus ttr cardiac amyloidoses in a population of 45 subjects with biopsy. These three subtypes are distinguished depending on the components of the deposits.
Light chain deposition disease lcdd in the lung is a rare occurrence. Sep 26, 2019 light chain deposition disease lcdd is the deposition of monoclonal light chains in multiple organs. Heavy chain disease an overview sciencedirect topics. 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. A 59yearold male was diagnosed with nephrotic syndrome secondary to light chain deposition disease. Lightchain deposition disease lcdd is the deposition of monoclonal light chains in multiple organs. The disease often is difficult to recognize because of its broad range of manifestations and what often are vague symptoms. 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. Renal failure, bone disease, and systemic light chain al amyloidosis appear to be more frequent in patients with light chain multiple myeloma lcmm. Light chain deposition disease is a systemic disease characterized by deposition of immunoglobin light chains in various organs. The specific type of midd is diagnosed by immunofluorescence or immunohistochemistry.
Light chain deposition disease accessed 17 january 2018. Here we leverage the unique characteristics of the dominantly inherited. The clinical syndromes at presentation include nephroticrange proteinuria with or without renal dysfunction, hepatomegaly. Lightchain al amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Dec 24, 2015 monoclonal immunoglobulin deposition disease is a group of multisystem disorders characterized by deposition of monoclonal immunoglobulin light or heavy chains in various organs. Aug 02, 2016 technically, light chain deposition disease lcdd is not considered a cancer. Ahlimmunoglobulin heavy and lightchain amyloidosis.
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