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Why are kidneys eroded by light chains? Revealing the pathogenic mechanism of light chain deposition disease!
Light Chain Deposition Disease (LCD) is a rare blood cell disease characterized by the deposition of immunoglobulin fragments, known as light chains (LCs), in the body. Normally, light chains are cleared by the kidneys, but in LCDD, these deposited light chains can damage organs and lead to multiple diseases. Almost all people with LCDD have effects on their kidneys that may eventually lead to kidney failure.
Symptoms and signs
Optical deposition disease almost always affects the kidneys, which can be identified by microscopic findings of hematuria and proteinuria. As light chains gradually increase, kidney function rapidly declines, often manifesting as acute tubulointerstitial nephritis or rapidly progressive glomerulonephritis.
In addition, patients may develop nephrotic syndrome and hypertension, and although the extent of light chain excretion varies, renal failure occurs with similar frequency. The condition may also affect other organs, including the skin, lungs, gastrointestinal tract, and glands. New research confirms that the liver is the most common non-kidney site of photon deposition disease, but its impact goes beyond that.
Pathogenic mechanism
The development of photocatalytic diseases results from the overproduction and deposition of abnormal immunoglobulins. About 60% of cases are related to plasmacytoma, myeloma and other lymphoproliferative diseases. In many cases, the exact cause remains unknown. Disease manifestations depend largely on the primary light chain structure.
In LCDD, the pathology of light chains is not clearly linked to their structural patterns, but many recurring features are identified. For example, somatic mutations rather than germline mutations are the source of amino acid substitutions, and fused amino acid positions are also commonly found in immunoglobulin binding sites.
Diagnostic methods
Diagnosing LCDD requires multiple laboratory tests, using blood and urine samples to assess kidney and liver function and detect the presence of monoclonal proteins. Imaging tests, namely ultrasound and cardiac ultrasound, also play an important role in this process. The latest diagnostic screening guidelines recommend the use of serum immunofixation testing and immunoglobulin free light chain testing.
Treatment pathways
The therapeutic goal of LCDD is to reduce the production of light chains that cause organ damage. Options include chemotherapy, stem cell transplantation, and immune-modulating drugs. Although there is currently no standard treatment, high-dose melphalan combined with autologous stem cell transplantation has produced good results in some patients.
Prospects and epidemiology
The clinical manifestations of LCDD often depend on the number and type of affected organs, which affects the patient's prognosis and survival time. The median survival time is approximately four years, with mortality rates increasing significantly as the disease progresses. The incidence of LCDD is relatively low and is often misdiagnosed as other protein diseases. According to estimates, the incidence rate in male patients is 2.5 times that of female patients, and the medical community still lacks understanding and diagnosis of this rare disease.
When facing this difficult disease, how can patients and their families find a balance between disease and quality of life?
