Light Chain Deposition Disease: Causes, Symptoms, Treatment, Survival Rate
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. As this condition can severely affect the kidneys, it is important to know about the causes, symptoms and treatment of light chain deposition disease.
What Is Light-Chain Deposition Disease?
In order to understand light-chain deposition disease, it is important to first understand what light chain is. For this we should first understand that our immune system produces antibodies which are used to fight infection and foreign bodies like bacteria and viruses. These antibodies are called immunoglobulins. The foreign bodies like bacteria and virus are called antigens. These saviors or immunoglobulins have a shape of the letter “Y” and are made up of two heavy chains and two light chains. These chains attach to the left and right of this “Y.” Together, these two pairs of chains make such a shape that is perfect to catch an antigen or the foreign body which is the bacteria or the virus.
Now coming to light-chain deposition disease, these light chains are produced by plasma cells which are a type of white blood cells produced by the bone marrow. Light-chain deposition disease is a pathological condition in which the plasma cells uncontrollably start producing abnormally excessive light chains. These excess light chains circulate through the blood and get deposited in various vital organs of the body like the kidneys, liver, or heart but mostly these chains get deposited in the kidneys causing severe symptoms. This condition is very detrimental for the kidneys and may cause severe damage to the kidneys up to the extent of renal failure.
What Are The Symptoms Of Light Chain Deposition Disease?
If light chains are deposited in the kidney, then it may result in scarring of the kidneys - a medical condition called glomerulosclerosis. The symptoms of light chain deposition disease include nephrotic syndrome and proteinuria, and high levels of blood lipids along with edema. In case of light-chain deposition disease in the heart, the symptoms often include cardiomegaly, congestive heart failure and irregular heartbeat. Light-chain deposition disease in the liver is usually asymptomatic although in some cases liver failure has been reported due to this condition. Symptoms of light-chain deposition disease involving the nervous system may result in neuropathy with pain, alteration of sensation, or inability to control muscles. If light-chain deposition disease involves the skin then the affected patient may develop numerous skin lesions or rashes.
How Is Light Chain Deposition Disease Diagnosed?
Light-chain deposition disease is often diagnosed when the individual is being tested for conditions like proteinuria or nephrotic syndrome. When testing for these conditions, a renal biopsy is done which clearly shows light chain deposits. The diagnosis of light chain deposition disease can be made by blood and urine examination as the light chains can also be found in the blood and urine of the patient.
What is the Treatment of Light Chain Deposition Disease?
The main aim of treatment of light-chain deposition disease is to reduce plasma cells which are responsible for producing these light chains. This task is quite complicated and sometimes chemotherapy is done to reduce the number of plasma cells. A stem cell transplant is also proved effective in reducing plasma cells and thus in the treatment of light-chain deposition disease. This is done by taking the patient’s own stem cells and then preserving them from bone marrow and blood. High dose of chemotherapy is given and then the stem cells are replaced back into the body through infusion. These cells then reach the bone marrow and start producing normal plasma cells and hence treating light-chain deposition disease.
Kidney transplant may be considered as a treatment for light-chain deposition disease in some patients with kidney failure when frequent dialysis and other forms of treatment fails. Hence, stem cell transplant is mainly considered for treatment of light-chain deposition disease.
What Is The Survival Rate For Light Chain Deposition Disease?
On an average, it takes about three years for an individual with light-chain deposition disease to progress to kidney failure. After five years, around 40% of people with light-chain deposition disease survive without any kidney problems due to light-chain deposition disease. Much depends on the severity of the condition, health status of the patient and the most appropriate treatment possible in a particular case.