AL amyloidosis: Full guide

What is AL amyloidosis?

AL amyloidosis is a rare condition that starts in plasma cells. Plasma cells are white blood cells in the bone marrow that make antibodies. In AL amyloidosis, a small group of abnormal plasma cells makes too many light chains. Light chains are small pieces of antibody protein. AL stands for amyloid light chain.

These light chains fold into the wrong shape. They stick together and form fibers called amyloid. Amyloid builds up in organs and tissues, such as the heart, kidneys, liver, nerves, and gut. Over time, it makes the organs stiff and keeps them from working well.

The group of abnormal plasma cells is often small, much smaller than in multiple myeloma. The harm comes mostly from the amyloid, not from the number of cells. Some people have both AL amyloidosis and myeloma.

AL amyloidosis is rare. About 3,000 to 4,000 people are diagnosed in the US each year, most often in their 60s. Symptoms can be vague, like tiredness, swelling, or shortness of breath, so it often takes time to diagnose. Finding it early, before the heart is badly affected, makes a big difference.

Treatment aims at the plasma cells, to stop new light chains from being made. When the light chains drop, organs often slowly get better. Many people now live for many years with good treatment and care at an experienced center.

Types and organs affected

Heart

The heart is affected in most people with AL amyloidosis. The heart walls become thick and stiff. This can cause shortness of breath, swelling in the legs, tiredness, low blood pressure, fainting, and an irregular heartbeat. Heart involvement matters most for the outlook.

Kidneys

Amyloid in the kidneys makes them leak protein into the urine. Urine may look foamy, and fluid can build up in the legs and belly. Over time, kidney function can decline, and some people need dialysis.

Nerves

Amyloid can cause numbness, tingling, or burning in the feet and hands. It can also affect the nerves that control blood pressure and digestion. This can cause dizziness when standing, diarrhea or constipation, and trouble with sexual function.

Liver, gut, and other tissues

The liver may get bigger. The gut can be affected, causing weight loss or poor appetite. Some people get an enlarged tongue, carpal tunnel syndrome, or easy bruising, especially purple bruises around the eyes.

Localized AL amyloidosis

Sometimes amyloid forms in only one place, such as the bladder, skin, lungs, or throat. It does not spread to organs like the heart. It usually needs only local treatment or watching, not chemotherapy.

ATTR amyloidosis (a different type)

Amyloid can also come from a protein made by the liver called transthyretin. This type is not a blood cancer condition and is treated with very different medicines. That is why testing the exact type of amyloid is so important.

AL amyloidosis with myeloma

Some people have both conditions. Treatment then aims at both the amyloid and the myeloma.

Tests you may have

Tissue biopsy

A small piece of tissue is checked under a microscope with a special dye called Congo red, which makes amyloid show up. The sample may come from belly fat (a quick, simple needle test), the bone marrow, or an affected organ like the kidney or heart.

Amyloid typing

A lab test, often mass spectrometry, finds which protein makes up the amyloid. This confirms it is AL and not another type like ATTR. It is a key step before treatment.

Light chain and protein blood tests

Serum free light chains, protein electrophoresis, and immunofixation look for the abnormal light chain and measure it. Urine tests look for light chains too.

Heart blood tests

NT-proBNP and troponin show how much strain and damage the heart has. They are used for staging and to track how the heart responds to treatment.

Echocardiogram

An ultrasound of the heart. It shows if the heart walls are thick and how well the heart relaxes and pumps.

Heart MRI

A detailed scan that can show amyloid in the heart muscle. It helps when the echocardiogram is not clear.

Kidney tests

Blood tests check kidney function, and a 24-hour urine collection measures how much protein leaks out.

Bone marrow biopsy

A small sample from the hip bone counts the plasma cells and checks for gene changes. It also checks for myeloma.

Other tests

Liver blood tests, a heart rhythm test (ECG) or monitor, nerve tests, and blood clotting tests may be done depending on your symptoms.

Stages and heart biomarkers

In AL amyloidosis, the stage is based mostly on the heart, because the heart has the biggest effect on how people do. Doctors use blood tests that show heart strain and damage: NT-proBNP and troponin. Many centers also add the difference between the abnormal and normal light chain levels.

In one common system, each high result counts as one point. More points mean a higher stage. Doctors in Europe also split the most advanced stage into two groups, based on how high the NT-proBNP is. The kidneys have their own staging, based on kidney function and how much protein is in the urine.

Your stage helps your care team choose how strong treatment can be and whether a stem cell transplant is safe. It is not a fixed prediction. Organs can get better with treatment, and people at every stage are treated.

Stage I

Heart tests and light chain levels are not high. The outlook is usually very good with treatment.

Stage II

One of the measures is high. Most people do well with treatment.

Stage III

Two of the measures are high. The heart is more affected, and care is planned carefully.

Stage IV

All three measures are high. The heart is more seriously affected. Treatment is still given, often with lower starting doses and close heart care.

Very advanced heart disease

When NT-proBNP is very high, the risk in the first months is higher. Starting treatment quickly at an experienced center is important. Clinical trials may be an option.

Kidney stages

Based on kidney function and the amount of protein in the urine. This helps predict the chance of needing dialysis.

Lab tests and response to treatment

Doctors track two kinds of response. The first is the blood (hematologic) response, which shows how well the light chains are dropping. The second is the organ response, which shows whether the heart, kidneys, or liver are getting better.

The blood response often comes within weeks to months. Organ response is slower and can take 6 to 12 months or longer, because amyloid already in the organs clears slowly. A deep blood response gives organs the best chance to heal.

dFLC (light chain difference)

The difference between the abnormal light chain and the normal one. This is the main number used to track the plasma cells.

Complete response

The abnormal light chain and protein can no longer be found, and the light chain ratio is normal. This is the goal of treatment.

Very good partial response

The light chain difference falls to a very low level. This is also linked to good organ recovery.

Partial response

The light chain difference falls by more than half. Your care team may change treatment if the response is not deeper.

Heart response

A clear drop in NT-proBNP. This shows the heart is under less strain.

Kidney response

Less protein in the urine, without a big drop in kidney function.

t(11;14)

A common gene change in the plasma cells of AL amyloidosis, found by FISH on the bone marrow. It can affect which medicines work best, such as venetoclax in some cases.

Troponin and NT-proBNP

Heart blood tests used for staging and to follow the heart over time. Kidney problems can raise NT-proBNP, so your care team reads it with your kidney tests.

Main treatments

Dara-CyBorD (Dara-VCd)

The standard first treatment for most people. It combines daratumumab (an antibody shot under the skin), cyclophosphamide (a chemotherapy pill), bortezomib (a shot under the skin), and dexamethasone (a steroid). It is usually given weekly for about 6 months, then daratumumab alone continues for up to 2 years in total.

Autologous stem cell transplant

For some people with good heart and kidney function, high-dose chemotherapy (melphalan) is given, followed by return of their own stem cells. It can lead to deep, lasting responses. Only some people qualify, and it should be done at a center with amyloidosis experience.

Treatment for advanced heart involvement

People with serious heart involvement may start with lower doses and slowly build up. Close heart monitoring is important. A clinical trial may be offered.

Supportive heart care

Water pills (diuretics) and a low-salt diet help control swelling. Some common heart medicines, like certain blood pressure pills, can cause problems in amyloidosis, so they are used carefully. Some people need a pacemaker, a defibrillator, or a blood thinner for an irregular heartbeat.

Supportive kidney care

Controlling swelling, avoiding medicines that strain the kidneys, and sometimes dialysis. A few people later have a kidney transplant.

Help with low blood pressure and nerve symptoms

Compression stockings, standing up slowly, and medicines like midodrine help with dizziness. Nerve pain medicines can ease burning and tingling.

Treatment at relapse

If light chains rise again, there are other options. These include different drug combinations such as daratumumab again, lenalidomide or pomalidomide, other proteasome inhibitors, and venetoclax for people with t(11;14). Newer immune treatments are being studied.

Organ transplant

A heart or kidney transplant is an option for a small number of carefully chosen people, usually along with treatment to control the plasma cells.

Clinical trials

Trials are testing antibodies that aim to clear amyloid that is already in the organs, as well as new immune treatments. None of the amyloid-clearing drugs is approved yet as of 2026. Ask your care team if a trial fits you.

Side effects and what helps

Fluid buildup and swelling

Dexamethasone and the condition itself can cause fluid to build up. Weigh yourself every morning. Call if you gain weight quickly, as your care team may adjust your water pills.

Low blood pressure and dizziness

Treatment can make low blood pressure worse. Stand up slowly, drink fluids as advised, and tell your team if you feel faint.

Numbness or tingling (neuropathy)

Bortezomib can cause or worsen nerve symptoms. Report them early so the dose or schedule can change. Giving it once a week under the skin lowers the risk.

Infections

Treatment lowers your defenses. You will likely take a medicine to prevent shingles. Keep up with vaccines and call for a fever.

Injection reactions

Daratumumab can cause chills, stuffy nose, or cough, mostly with the first dose. Medicines given beforehand help prevent this.

Tiredness

Tiredness is very common from both the condition and treatment. Short walks, rest breaks, and good sleep can help. Ask about physical therapy or cardiac rehab.

Stomach and bowel problems

Diarrhea, constipation, nausea, or poor appetite can happen. A dietitian can help you keep weight and strength up.

Steroid side effects

Dexamethasone can cause trouble sleeping, mood changes, and high blood sugar. Taking it in the morning helps. Doses are often kept low in amyloidosis.

Transplant side effects

High-dose melphalan can cause mouth sores, diarrhea, low blood counts, and infection risk. Recovery takes weeks to months.

Living with AL amyloidosis

AL amyloidosis is a serious condition, but many people do well, especially when it is found early and treated at a center with amyloidosis experience. A team often includes a hematologist, a heart doctor, and a kidney doctor.

Weigh yourself every morning and keep a log, along with your blood pressure if your team asks. Sudden weight gain can mean fluid is building up. Follow a low-salt diet if advised.

Organ recovery is slow. It can take many months to feel better, even when blood tests show the treatment is working. Be patient with yourself, and keep track of small gains, like walking farther or less swelling.

Ask before taking any new medicine, including pain pills like ibuprofen or naproxen, which can strain the kidneys and cause fluid buildup. Keep up with vaccines and call quickly for a fever.

You will have regular blood tests to follow the light chains and heart and kidney markers. These can show a relapse early, often before any symptoms.

Caregivers carry a lot too. Ask about social workers, support groups, and help with travel and costs. The Amyloidosis Foundation and the Amyloidosis Research Consortium offer free information and support.