AL amyloidosis, which is also called amyloid light-chain amyloidosis or primary amyloidosis, can affect many different organs. It most commonly leads to problems with the heart and kidneys but can also affect the nervous system, digestive system, and skin.

Some people with AL amyloidosis may only have one affected organ, but most people experience problems with multiple organs, especially as the disease progresses. People who have amyloid deposits in more than one organ are more likely to have a worse prognosis.

Read on to learn what causes this organ damage, how doctors diagnose it, and what it can mean for your heart, kidneys, nervous system, digestive system, and skin.

🗳️ If you’re living with AL amyloidosis, which of your organs is affected?

Heart

Kidneys

Other organ or tissue

Multiple organs

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What Causes Organ Damage in AL Amyloidosis?

Amyloidosis is caused by abnormal proteins that attach together, forming clumps. These clumps can be deposited in different organs and tissues. As amyloid deposits build up in an organ, they can prevent it from working properly, leading to damage and eventually organ failure.

Experts believe there are a couple of reasons amyloid deposits cause damage:

• The buildup of amyloid proteins can change the structure of an organ, causing its tissue to become disorganized and misshapen.
• Abnormal light-chain proteins may be toxic, damaging nearby cells.

There are different types of amyloidosis. Some types are localized, meaning amyloid deposits are only found in one location in the body. Other types are systemic and cause amyloid proteins to build up in many areas.

AL amyloidosis is the most common type of systemic amyloidosis. In this form, plasma cells — a type of white blood cell — make misfolded light-chain proteins.

Light chains are part of antibodies — proteins that latch onto specific germs or other invaders. This can stop the germs directly, or mark them so the rest of your immune system can clear them out. Although plasma cells are usually found in the bone marrow tissue located inside certain bones, the amyloid light-chain proteins they produce can spread throughout the body.

How Doctors Determine Which Organs Are Affected

The symptoms you have may provide a clue as to which organs are affected. Diagnosis often starts with less invasive tissue sampling, such as a biopsy of the fat pad in your abdomen or a bone marrow biopsy. If these tests come back negative, or if your doctor needs more specific information, a biopsy of the affected organ itself may be needed, such as a renal (kidney) biopsy.

In the lab, specialists stain the tissue sample with a dye called Congo red. Under a certain light, amyloid deposits show a distinctive apple-green glow, called birefringence, that confirms their presence. Once amyloid is confirmed, the protein is studied to determine which form of amyloidosis is present, since this guides your treatment plan.

Organs Commonly Affected in AL Amyloidosis

Knowing which organs may be damaged may help you and your healthcare team understand how to treat your condition and what your prognosis might be.

Heart

As many as 3 out of 4 people with AL amyloidosis develop amyloid proteins in the muscle tissue of their hearts. This is sometimes referred to as cardiac amyloidosis. When amyloid fibrils collect in the heart, the walls of this organ become thicker, which can lead to heart failure. Common symptoms that may indicate heart damage include:

• Tiredness
• Chest pains
• Arrhythmia (irregular heart rhythms)
• Shortness of breath

The main factor that determines a person’s AL amyloidosis prognosis is whether the disease affects the heart. Past studies have found that those who have heart failure due to AL amyloidosis live for an average of six months after diagnosis, if the disease is left untreated.

For most people with newly diagnosed AL amyloidosis, first-line treatment now centers on daratumumab-based therapy — typically daratumumab combined with bortezomib, cyclophosphamide, and dexamethasone. This treatment targets the abnormal plasma cells causing the damage, which can reduce light-chain levels and ease cardiac symptoms over time.

Heart transplantation is an option for carefully selected people with severe heart involvement. Studies show that 65 percent of people with AL amyloidosis live for at least five years after receiving a heart transplant.

Not everyone is healthy enough to undergo a heart transplant, but other treatments can also help. Pacemakers can help with certain slow heart rhythms and electrical signaling problems caused by amyloid damage.

Kidneys

AL amyloidosis affects the kidneys in about 65 percent of cases. It often causes a condition called nephrotic syndrome. Nephrotic syndrome can lead to signs like high levels of protein in the urine, low levels of albumin protein in the blood, or high cholesterol levels. Eventually, nephrotic syndrome can lead to kidney failure.

If you have amyloid deposits in the kidneys, you may experience symptoms like:

• Changes in how often you urinate
• Waking up at night to urinate
• Bubbles in the urine
• Swelling in the legs or abdomen

Treatments that target plasma cells can also help improve kidney function by reducing the production of amyloid-forming light chains. For most people with newly diagnosed AL amyloidosis, this starts with daratumumab-based therapy.

For some people who are healthy enough to tolerate it, treatment also includes high-dose chemotherapy followed by an autologous stem cell transplant. In this procedure, doctors collect a person’s own healthy stem cells before the high-dose chemotherapy and return them afterward to help the bone marrow recover.

Other treatments, such as dialysis and kidney transplant, may also be used to help people with AL amyloidosis who experience kidney failure.

Nervous System

In a small study of 43 people with AL amyloidosis, 35 percent had nervous system involvement. Amyloid deposits can damage nerves in the arms, hands, feet, or legs. AL amyloidosis can also affect the nerves that control body functions like blood pressure or movement of food through the intestines.

People with nerve system damage may experience:

• Peripheral neuropathy — Damage to the nerves in the limbs, which can lead to numbness, pain, or tingling
• Autonomic neuropathy — Damage to the nerves that control body functions, which can cause symptoms like dizziness, nausea, bladder dysfunction, and diarrhea
• Carpal tunnel syndrome — Pressure on the nerves in the wrist, leading to numbness, weakness, or tingling in the wrist or hand

These symptoms might go away if you are treated for AL amyloidosis. However, many people continue to have nerve-related symptoms even after their treatment is finished.

Nervous system symptoms can also be caused by chemotherapy drugs used to treat AL amyloidosis. These treatment-related symptoms often improve within a few months of stopping the medication, but in some people they can last for years, and they don’t always go away completely.

Digestive System

Experts estimate that roughly 10 percent to 15 percent of people with AL amyloidosis have symptomatic involvement of the gastrointestinal (digestive) system, though estimates vary depending on how doctors define and confirm GI involvement.

Amyloidosis can affect the tongue, esophagus, stomach, small intestines, large intestines, liver, or some combination of these. Deposits can form in the muscle, mucous membrane, nerves, or blood vessels of these organs, leading to a wide variety of symptoms.

These symptoms can include:

• Swallowing problems
• Acid reflux
• Nausea or vomiting
• Bloating
• Diarrhea
• Constipation
• Blood in the stool
• Weight loss

Treatment for GI involvement focuses on controlling the AL amyloidosis itself by reducing the abnormal light chains that cause it — for most people, this now means daratumumab-based therapy that treats the whole body.

Skin

Amyloid deposits can be found in the skin of about 30 percent to 40 percent of people with AL amyloidosis. Skin-related symptoms can include:

• Purpura (small red, blue, or purple spots)
• Waxy bumps or areas of thickened skin near the eyelids, neck, or groin
• Blisters filled with blood
• Hair loss
• Brittle nails

Skin treatments may include surgery to remove the bumps. However, skin symptoms usually have less impact on overall health than symptoms from other organs.