The specific type of midd is diagnosed by immunofluorescence or immunohistochemistry. Lcmm has an earlier average age of onset and appears to have a poorer prognosis when compared to igg or iga variant 3, 17. Light chain deposition disease pulmonary manifestations. 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. Learn about amyloid light chain amyloidosis from cleveland clinic. It is a malignant disease characterized by abnormal proliferation of plasma cells and monoclonal immunoglobulins or free light chains flc. Systemic manifestations include weight loss, easy bruising, brittle and. Natural history and outcome of light chain deposition. 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. Light chain deposition diseasecausessymptomstreatment. L l f flc i t d ith th b flevels of flcs are associated with the number of. The disease often is difficult to recognize because of its broad range of manifestations and what often are vague symptoms.
Jan 21, 2019 neurofilament light chain nfl is a promising fluid biomarker of disease progression for various cerebral proteopathies. Bone marrow can be deferred in patients with lowrisk monoclonal gammopathy of undetermined signi. 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. 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. 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. It occurs in older children and young adults and is associated with gastrointestinal symptoms, such as malabsorption, intestinal obstruction, and diarrhea. Ahlimmunoglobulin heavy and lightchain amyloidosis. High dose chemotherapy in light chain or light and heavy chain. Patients with lcdd are middleaged or older adults, and men are affected more often. Treatment generally involves chemotherapy, corticosteroids and, in some cases, radiation therapy. Monoclonal gammopathy characterized by overproduction and deposition of nonamyloid immunoglobulin light chains in various organs.
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. A 59yearold male was diagnosed with nephrotic syndrome secondary to light chain deposition disease. 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. Serial serum free light chain measurement should be routinely performed in patients with al amyloidosis and multiple myeloma patients with oligosecretory disease. The symptoms a person has vary based on the reason for the light chain production.
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. Lightchain deposition disease lcdd is the deposition of monoclonal light chains in multiple organs. Light chain deposition disease lcdd is a rare plasma cell dyscrasia characterized by deposition of immunoglobulin fragments. Castleman disease is an uncommon and heterogeneous lymphoproliferative disorder for which management is rapidly evolving. A precise morphologic and clinical description of lcdd. People with lcdd make too many light chains, which get deposited in many body tissues. The outcome of patients with light chain deposition disease remains uncertain. Light chain deposition disease lcdd is a rare condition characterized by extracellular light chain deposition in tissues. Serum free light chain assay14 pages 1112 kappa free light chain 0.
However, it shares some characteristics with cancer, and can be associated with certain types of cancer. 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. 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. The immunoglobulin light chain is the small polypeptide subunit of an antibody immunoglobulin. While lcdd can occur in any organ, the kidneys are always. This issues inaugural conference on light chain deposition disease is discussed by david salant, vaishali. Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues.
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 is a systemic disease characterized by deposition of immunoglobin light chains in various organs. International myeloma working group updated criteria for. 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. A case of cardiac light chain deposition disease in a patient.
Light chain deposition disease lcdd is the most common form of ig deposition disease and has been considered as a contraindication to transplant. While lcdd can occur in any organ, the kidneys are always involved. If you have problems viewing pdf files, download the latest version. 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. There was no other evidence of a b cell clonal disorder or amyloidosis. Light chain deposition disease lcdd in the lung is a rare occurrence. Light chain deposition disease an overview sciencedirect. 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.
Light chain deposition disease neuropathy resembling. Neurofilament light chain in blood and csf as marker of. Aug 02, 2016 technically, light chain deposition disease lcdd is not considered a cancer. Light chain deposition disease accessed 17 january 2018. Normally, they are present in equal amounts in the blood, giving a ratio of 1 to 1.
Lcs are normally cleared by the kidneys, but in lcdd, these light chain deposits damage organs and cause disease. The clinical syndromes at presentation include nephroticrange proteinuria with or without renal dysfunction, hepatomegaly. 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. These three subtypes are distinguished depending on the components of the deposits. 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. In large part this is related to the effects of light chains in other organs and not necessarily due to kidney disease. Amyloidosis information a general overview for patients. 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. Al amyloidosis amyloid light chain cleveland clinic. Alpers, md2 clinical and pathologic features light chain deposition disease lcdd is the most common of the nonal amyloid monoclonal immunoglobulin deposition diseases midd. 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. To the best of our knowledge, lcdd has not been previously reported from saudi arabia. Light chain deposition disease lcdd is a rare illness with, as yet, no clear evidencebased guidelines for its treatment.
Patient survival from the time that symptoms begin has ranged from 1 month to 10 years. Lcdd is a clonal plasma cell proliferative disorder in which fragments of light chains. Pulmonary manifestations of light chain deposition disease are rare when considering the full disease spectrum of light chain deposition disease pathology. The kidneys are almost always affected and this often leads to kidney failure. 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. Renal involvement is the most common clinical manifestation. Persons with light chain disease may develop lytic bone lesions, hypercalcemia, impaired kidney function, and amyloidosis. 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. A 59yearold male was diagnosed with nephrotic syndrome secondary to lightchain deposition disease. The kidneys are almost always affected while heart, liver and other tissues are occasionally involved.
Light chain disease definition of light chain disease by. 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. Differentiating immunoglobulin lightchain al from transthyretinrelated ttr cardiac amyloidoses is imperative given the implications for prognosis, therapy, and genetic counseling. Patients with lcdd are middleaged or older adults, and men are affected. 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. Light chain deposition disease lcdd is characterized by the deposition of monotypic immunoglobulin light chains in the kidney, resulting in renal dysfunction. Pdf a case of light chain deposition disease lcdd in a.
Natural history and outcome of light chain deposition disease. The deposits are derived from monoclonal light chains only in lcdd, monoclonal light and heavy chains. 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. Lightchain deposition disease lcdd is the most common form of ig deposition disease and has been considered as a contraindication to transplant. Systemic light chain deposition disease presenting as multiple pulmonary nodules. 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. Among the systemic diseases associated with immunopro liferative syndromes is a recently identified lightchain depo sition disease lcdd li, 21.
Measure of disease and treatment response in light chain multiple myeloma patients using urine and serum assays. Heavy chain disease an overview sciencedirect topics. Diagnosis and management of castleman disease jacob d. 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. Castleman disease is an uncommon lymphoproliferative disorder characterized as either unicentric. A condition in which plasma cells secrete immunoglobulin light chains of only one type, kappa or lambda.
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. Maia,1,2,4,10 oliver preische,1,5 juliane schelle,1,2,3 anja apel,1,6 stephan a. A typical antibody is composed of two immunoglobulin ig heavy chains and two ig light chains. 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. See also gammopathy, heavy chain disease, multiple myeloma. Multiple myeloma early detection, diagnosis, and staging. Amyloidosis is generally a disease of middleaged people and older, although the disease has been seen in individuals in their thirties.
In 2015, an estimated 28,850 new cases of multiple myeloma were. Renal failure, bone disease, and systemic light chain al amyloidosis appear to be more frequent in patients with light chain multiple myeloma lcmm. A 65yearold man with igg lambda multiple myeloma developed severe polyneuropathy with prominent thermalpain sensory impairment and autonomic failure. Monoclonal immunoglobulin deposition disease is a group of multisystem disorders characterized by deposition of monoclonal immunoglobulin light or heavy chains in various organs. Monoclonal immunoglobulin deposition disease light chain deposition disease monoclonal. 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. 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. Diagnosis and monitoring a case of lightchain deposition. 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. View the article pdf and any associated supplements and figures for a period of 48 hours. Hcd, mediterranean lymphoma is considered a variant of malttype lymphoma. Technically, light chain deposition disease lcdd is not considered a cancer.
However, when there is light chain restriction to a single light chain isotype e. Light andor heavy chain deposition disease, high dose therapy, autologous stem cell transplantation. 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. Light chains are used to make antibodies that the body needs to fight infection. 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. Light chain cast nephropathy myeloma kidney is a disease resulting from acute tubular injury fig. 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.
The light chains are secreted by a plasma clone and deposit in the alveolar walls, small airways, and vessels. Ninetyseven patients with light chain disease lcd were studied. Lightchain al amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. Natural history and outcome of light chain deposition disease ncbi. Light chain deposition disease genetic and rare diseases. Although the clinical presentation suggested amyloid neuropathy, nerve biopsy showed the immunohistochemical and ultrastructural features typical of light chain deposition disease lcdd. A case of atypical light chain deposition diseasediagnosis and. Light chain deposition disease is a rather uncommon monoclonal gammopathy with predominantly renal manifestations with presence of monoclonal light chains in serum and urine. Pulmonary manifestations of 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. Patients commonly have an underlying plasma cell dyscrasia, and produce excess levels of monoclonal light chains. Pdf on aug 1, 2001, p m ronco and others published light chain deposition disease.
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