• Early diagnosis and treatment improves the prognosis for amyloid light-chain (AL) amyloidosis, which progresses rapidly without treatment.
• Treatment goals for AL amyloidosis are to reduce the production of abnormal light-chain proteins, preserve organ function, and improve quality of life.
• Treatment options for AL amyloidosis include chemotherapy, steroids, immunotherapy medications, and stem cell transplants.
• Organ transplant surgeries, including kidney transplant or heart transplant, may become necessary if advanced AL amyloidosis affects these organs.

In amyloid light-chain amyloidosis, plasma cells produce an abnormal immune protein which accumulates in tissues and organs as a substance called amyloid. The goal of treatment for AL amyloidosis is to reduce the production of amyloid proteins, preserve organ function, and improve quality of life. Since AL amyloidosis progresses rapidly without treatment, doctors recommend the most rapid and effective treatments that can be tolerated.

Before recommending a treatment plan, your doctor will assess your overall health, any other health conditions you have, and whether amyloidosis may be impacting your heart and kidneys. These details will help your doctor determine whether your case of amyloidosis is high or low risk and assess your eligibility for various treatments.

Treatment options for AL amyloidosis, also known as primary amyloidosis or immunoglobulin light-chain amyloidosis, can include traditional chemotherapy, stem cell transplants, and recent advances in immunotherapy. If amyloidosis severely damages the heart or kidneys, organ transplantation may be recommended.

The Importance of Early Diagnosis and Treatment

The diagnostic process for amyloidosis can take some time, but a proper diagnosis is vital before treatment can begin. Early detection and diagnosis shortens the time window to the establishment of a treatment regimen. There is currently no cure for AL amyloidosis, but establishing a treatment protocol can improve quality of life and slow disease progression.

Read more about causes, risk factors, and prevalence of AL amyloidosis.

Medications To Treat AL Amyloidosis

Medication is a treatment option for AL amyloidosis, including chemotherapy, immunotherapy, and steroids. Many people who have an AL amyloidosis diagnosis take a combination of these medications, such as one or two chemotherapy drugs plus a steroid. Medications can stop or slow the progression of AL amyloidosis, however they cannot remove the amyloid deposits already in the body. Therefore, the goal of medication for AL amyloidosis is to prevent further protein deposits.

Chemotherapy

Chemotherapy drugs destroy abnormal cells. Sometimes a doctor may recommend combining a chemotherapy drug with stem cell transplantation. The chemotherapy drugs used to treat AL amyloidosis include:

• Melphalan, branded as Alkeran or Evomela
• Cytoxan (cyclophosphamide)
• Revlimid (lenalidomide)
• Pomalyst (pomalidomide)

Corticosteroids

Corticosteroid medications, also known as steroids, are used to enhance the effects of chemotherapy drugs. Steroids used as part of amyloidosis treatment include dexamethasone and prednisone.

Proteasome Inhibitors

Some doctors may also suggest the use of proteasome inhibitors. Proteasomes are organelles (tiny organ-like structures) within cells that are responsible for cleaning up damaged and abnormal proteins. Proteasome inhibitors are drugs that modulate the action of proteasomes and encourage the death of the plasma cells that produce amyloid proteins.

Proteasome inhibitors sometimes used to treat AL amyloidosis include:

• Velcade (bortezomib)
• Kyprolis (carfilzomib)
• Ninlaro (ixazomib)

Immunotherapy

Immunotherapy is a treatment strategy that uses parts of your immune system to fight cancer and other diseases. Monoclonal antibodies, also referred to as biologics, are one type of immunotherapy. Monoclonal antibodies are immune proteins that have been bioengineered to recognize and neutralize a specific molecule.

The most common immunotherapy drug used for AL amyloidosis is Darzalex (daratumumab). Recently, the U.S. Food and Drug Administration (FDA) approved Darzalex Faspro (daratumumab/hyaluronidase-fihj) in combination with other medications for the treatment of AL amyloidosis. Darzalex Faspro is the first FDA-approved treatment specifically indicated for newly diagnosed individuals with AL amyloidosis.

Clinical Trials

New treatments for amyloidosis are being studied in clinical trials. One potential advancement in the treatment of AL amyloidosis involves monoclonal antibody CAEL-101, which may effectively remove amyloid deposits already deposited in organs. For those who are eligible to participate, clinical trials for amyloidosis can provide access to new therapies that are not yet widely available.

Surgical Procedures for AL Amyloidosis

Surgical procedures for AL amyloidosis are used to manage symptoms and prolong life. Stem cell transplants, formerly known as bone marrow transplants, may help prevent the production of amyloid proteins. Organ transplants can replace a diseased and damaged organ with a healthy one from a donor.

For people with AL amyloidosis, both kidney transplantation and heart transplantation have had good clinical outcomes. Stem cell transplants are also considered highly effective treatments for AL amyloidosis in those who are eligible to receive them. However, transplants are not appropriate for all individuals, particularly if the amyloid deposits are widespread and deposited in several organs.

Lifestyle Changes and AL Amyloidosis

Lifestyle changes can potentially help with some of the symptoms of AL amyloidosis. Generally speaking, getting enough exercise and eating a healthy diet are helpful in maintaining overall wellness.

If you are experiencing poor appetite and unintentional weight loss with AL amyloidosis, you may find that going on a short walk can stimulate appetite. Regular exercise can also help fight fatigue and pain. However, keep in mind that lifestyle changes are not a substitute for sticking with the treatment plan tailored for your condition in close partnership with your doctor.

Read more about symptoms of AL amyloidosis.

Challenges in AL Amyloidosis Treatment

AL amyloidosis often develops alongside other diseases, such as multiple myeloma. The presence of multiple chronic conditions requires concurrent and overlapping treatment. It is also vital to stick with prescribed therapy for the entire course of treatment, even if you start to feel better. Stopping treatment early can mean losing the potential benefits of therapy.

Talk With Others Who Understand

MyAmyloidosisTeam is the social network for people with amyloidosis and their loved ones. On MyAmyloidosisTeam, members come together to ask questions, give advice, and share their stories with others who understand life with amyloidosis.

Are you living with AL amyloidosis? Share your experience in the comments below, or start a conversation by posting on your Activities page.