Light Chain Deposition Disease
What is Light Chain Deposition Disease?
Light chain deposition disease (LCDD) is a rare disorder in which abnormal monoclonal immunoglobulin light chains, a protein component of antibody molecules, accumulate in tissues. These proteins, produced by antibody-producing B cells and plasma cells derived from them, deposit primarily along basement membranes (thin, sheet-like structures that underlie epithelial or endothelial tissues), particularly in the kidneys, where they can cause progressive tissue damage and impaired organ function.
The deposited proteins in LCDD are granular deposits, distinguishing the condition from amyloid light chain (AL) amyloidosis, in which misfolded light chains form organized fibrils visualized by staining with Congo red dye and microscopic examination.
Synonyms
- Randall-type monoclonal immunoglobulin deposition disease
- Monoclonal Immunoglobulin Deposition Disease — LCDD is the most common subtype of MIDD
Light chain deposition disease (LCDD) is a rare disorder in which abnormal monoclonal immunoglobulin light chains, a protein component of antibody molecules, accumulate in tissues. These proteins, produced by antibody-producing B cells and plasma cells derived from them, deposit primarily along basement membranes (thin, sheet-like structures that underlie epithelial or endothelial tissues), particularly in the kidneys, where they can cause progressive tissue damage and impaired organ function.
The deposited proteins in LCDD are granular deposits, distinguishing the condition from amyloid light chain (AL) amyloidosis, in which misfolded light chains form organized fibrils visualized by staining with Congo red dye and microscopic examination.
LCDD is considered very rare, and its exact prevalence in the general population is unknown due to underdiagnosis. It is diagnosed most often in middle-aged and older adults with a slight male predominance. About 50-60% of cases are associated with multiple myeloma, a cancer of antibody-producing B cells. Because LCDD is frequently identified through kidney biopsy, its true frequency may be underestimated.
| Name | Abbreviation |
|---|---|
| Randall-type monoclonal immunoglobulin deposition disease | |
| Monoclonal Immunoglobulin Deposition Disease — LCDD is the most common subtype of MIDD | MIDD |
LCDD develops when an abnormal clone of B cells produce an excess of antibody light chain proteins. Light chains come in two types, kappa and lambda. In LCDD, kappa chains are the predominant type. The excess light chains precipitate along tissue basement membranes, triggering an inflammatory response that destroys normal tissue architecture. The condition is not considered to be inherited.
Symptoms depend heavily on the organs affected. Over 90% of LCDD patients have kidney involvement which can manifest through proteinuria (excess protein in urine), edema (fluid buildup particularly in legs and ankles), blood in urine, hypertension, fatigue, and progressive chronic kidney disease. Systemic involvement may occur when deposits form in the liver, heart, lungs and central nervous system, leading to fatigue, liver enlargement, shortness of breath, heart arrhythmias or failure, or neurologic deficits.
| Name | Description |
|---|---|
| Edema | Edema is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body. |
| High blood pressure | High blood pressure that is often hard to control even with medication |
| Nephrotic syndrome | Nephrotic syndrome is a nonspecific disorder in which the kidneys are damaged, causing them to leak large amounts of protein from the blood into the urine. |
Diagnosis requires a combination of tissue biopsy to identify light chain deposits in tissue and hematologic evaluation to assess the underlying clonal growth. Light microscopy can reveal light chain deposits in glomerular and tubular basement membranes of the kidney. Negative Congo red staining differentiates LCDD from amyloidosis. Electron microscopy can show electron-dense granular deposits. Serum free light chain assays and protein electrophoresis tests can further confirm the diagnosis. As appropriate, tissues from other organs besides the kidney can be examined to determine systemic involvement.
Treatment is not directed at the affected organ itself, but rather at eradicating the underlying clonal plasma cells to halt the production of toxic light chains. Chemotherapeutic regimens using a combination of cyclophosphamide, bortezomib and dexamethasone can be a first-line approach. Dialysis may be needed to treat advanced kidney failure. Autologous stem cell transplantation (in which patients are given back their own cells) can follow high-dose chemotherapy and be highly effective for eligible patients. Kidney transplantation may be considered for patients who reach end-stage renal disease (ESRD) and have achieved control of the underlying clonal cell growth.
For LCDD with multiple myeloma, treatment generally follows an appropriate myeloma-directed therapy regimen.
Without treatment, kidney function can decline to end-stage disease within a few months to a few years. The introduction of bortezomib-based therapies and stem cell transplantation has dramatically improved overall survival. The prognosis depends on the severity of the kidney involvement at the time diagnosis, the presence involvement in other organs, and the response to treatment. The presence of multiple myeloma is associated with a poorer outcome.
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Cassano Cassano, R., Bonadio, A. G., Del Giudice, M. L., Giannese, D., Galimberti, S., & Buda, G. (2024). “Light chain deposition disease: pathogenesis, clinical characteristics and treatment strategies.” Annals of Hematology. 104:2083–2093. https://doi.org/10.1007/s00277-024-05911-9.
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Herrera GA, Teng J, Turbat-Herrera EA, Zeng C, Del Pozo-Yauner L. (2020). “Understanding Mesangial Pathobiology in AL-Amyloidosis and Monoclonal Ig Light Chain Deposition Disease.” Kidney Int Rep. 5(11):1870-1893. doi: 10.1016/j.ekir.2020.07.013. PMID: 33163710; PMCID: PMC7609979.
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Masood, A., Ehsan, H., Iqbal, Q., Salman, A., & Hashmi, H. (2022). “Treatment of Light Chain Deposition Disease: A Systematic Review.” Journal of Hematology, 11, 123-130. https://doi.org/10.14740/jh1038.
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Mayo Clinic: Amyloid and light chain deposition disorders: Clinical overview and pulmonary implications.
Last updated 20 Oct 2011, 08:06 PM
20 Oct 2011, 08:06 PM
It's a conjecture, but quite clearly the prednisone helped both maintaining kidney function or more accurately slowing down decline in kidney function and perhaps reducing light chain production. We saw quite clearly that after stopping the low dose prednisone, kidney function deteriorated more quickly. WAe also saw over time a decrease in light chain production. Re. the latter one can't know if it was the course of the disease for her or it was the result of the low dose prednisone. So the doc don't really know why it helped, we know it is very likely that it helped. The program at Boston University is another place to consult in addition to the Mayo. I hope this is helpful Best luck
20 Oct 2011, 04:12 PM
Would like to see more activity here...but I guess the LCDD is pretty rare. My 76-yr-old hubby was dx with LCDD a little over a year ago after a high creatinine reading during a routine physical. The sad thing is that the reading had been elevated the previous year and the doc did not notice nor follow up. A year ago he tried velcade (major neuropathy) and then melphalan. His light chains dropped considerably but are rising again. The Onc's opinion was that his disease will not affect other organs...and since the kidneys are already non-functioning, they discontinued the chemo. As I mentioned a year ago, he's lost his kidney function and is on hemodialysis. We visited Mayo in Rochester and their review was "nothing can be done except dialysis". He is too old for stem cell. And a transplant is out of the question due to lcdd. He has one plus. He is able to still pass urine so it makes the kidney diet a bit easier. Barber, What was the reason (and the benefit) for the low dose prednisone? To help the kidneys continue to function??? Thanks for any response.
20 Oct 2011, 11:50 AM
Sorry to hear Blondie. Very sinilar to what happened to my wife, as I wrote earlier dexa folllowed by low does prednisone was sufficient to help her for the last 10 years. The data indicates that Velcade should be very helpful. Best luck
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I have been recently diagnosed with smoldering myeloma and edema due to capillary leakage syndrome. The build up of fluid started last spring. I have been put on oral steroids which seems to take...
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Just Diagnosed....ANyone out there??
Created by monkeybradjo | Last updated 20 Oct 2011, 08:06 PM
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