What is Light-Chain Deposition Disease?

Light-chain deposition disease (LCDD) is a rare blood disorder. It happens when pieces of antibodies called immunoglobulin light chains build up in organs such as the kidneys, liver, heart, or lungs. Immunoglobulin light chains are abnormal proteins produced by plasma cells. LCDD belongs to a group of disorders called monoclonal immunoglobulin deposition diseases.

Who is affected by LCDD?

The condition is most often seen in men. The median age at diagnosis is 58 years. Because it is rare, the true number of people affected each year is unknown. 

LCDD can be found in any organ. Kidneys are almost always affected. People may experience blood in the urine (hematuria), protein in the urine (proteinuria), or swelling in the lower extremities (edema). This causes kidney damage.The accumulation of light chains can also affect the liver, heart, and lungs, although these are less common. 

Is LCDD cancer?

LCDD is not cancer. It is a blood disorder. However, about 50% to 60% of people with LCDD also have multiple myeloma. Around 17% have monoclonal gammopathy of undetermined significance (MGUS). 

What are the signs and symptoms of LCDD?

LCDD symptoms vary. This is because it depends on which organ is primarily affected. 

Symptoms of LCDD that affect the whole body include: 

• Weakness
• Fatigue
• Weight loss
• Bone pain
• Numbness or tingling in arms and legs
• Skin lesions

Symptoms of LCDD that affects the kidney include: 

• Scarring of the blood vessels in the kidney, called glomerulosclerosis
• Changes to the urine, such as protein in the urine (proteinuria) 
• Swelling
• Fluid buildup (edema)
• Frequent urination
• Thirst

Symptoms of LCDD that affects the heart: 

• Heart enlargement
• Congestive heart failure
• Irregular heartbeat

When LCDD affects the liver, there may be no symptoms other than liver failure.

How is LCDD diagnosed?

Diagnosing LCDD begins with a full medical history and examination. You will also have blood and urine tests and imaging. A biopsy of the kidney or other affected organ(s) can confirm the condition. Your doctor will confirm that it is LCDD and not another condition that can have similar symptoms, such as amyloidosis or cryoglobulinemia. 

How is LCDD treated?

Because LCDD is uncommon, there are no formal treatment guidelines. The main goal is to reduce the production of harmful light chains and protect organ function. Options include:

• Autologous stem cell transplantation (ASCT): During an ASCT, healthy blood stem cells are removed from your body. After a high-dose of chemotherapy, the stem cells are placed back in the body. Studies show improvements in kidney function after ASCT.
• Bortezomib (Velcade, Takeda): Bortezomib blocks protein breakdown in cells, improving kidney outcomes and reducing the amount of protein in the urine.
• Immunomodulatory drugs (thalidomide, lenalidomide, pomalidomide): These therapies modify immune activity and have shown benefit in case reports. These drugs can have severe side effects.
• Daratumumab (Darzalex, Janssen): Daratumumab is a targeted therapy that targets CD38 on plasma cells. In a small study, people who had LCDD that affected the kidney improved their health after treatment.
• Dialysis or kidney transplantation: For people with kidney failure, treatment is needed. Dialysis removes waste from the bloodstream that would normally be removed by the kidneys. If light-chain production is under control, a kidney transplant may be recommended for end-stage kidney disease. Recurrence in the transplanted kidney is common, so careful monitoring is required.

What is the outlook for people with LCDD?

Median survival for people with LCDD may vary depending on age, whether multiple myeloma is also present, and if the disease has spread to organs outside the kidney. Even with dialysis or kidney failure, survival is mostly determined by the underlying plasma cell disorder.

Although LCDD is a rare condition,  it should be monitored, especially in people diagnosed with multiple myeloma. Keep track of your health with HealthTree Cure Hub, where you can have all your records in one place and connect with your genetic twin to see what has worked for them that might work for you. Start exploring all HealthTree functions by clicking the button below.  

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Source: 

• Light-Chain Deposition Disease