MyAmyloidosisTeam

Amyloidosis staging helps doctors understand how far the disease has progressed, estimate prognosis, and guide treatment decisions. Because there are different types of amyloidosis, different staging systems are used depending on the type and which organs are affected.

---

AL Amyloidosis Staging

The most widely used Show Full Answer

Amyloidosis staging helps doctors understand how far the disease has progressed, estimate prognosis, and guide treatment decisions. Because there are different types of amyloidosis, different staging systems are used depending on the type and which organs are affected.

---

AL Amyloidosis Staging

The most widely used system for AL amyloidosis is the Mayo Clinic Staging System, which looks at three biomarkers in the blood:

- NT-proBNP (a marker of heart stress)
- cTnT (cardiac troponin T, a marker of heart damage)
- dFLC (difference in free light chains, a marker of abnormal protein levels)

The stages break down like this:

- Stage 1 — All three biomarkers are normal
- Stage 2 — One biomarker is elevated
- Stage 3 — Two biomarkers are elevated
- Stage 4 — All three biomarkers are elevated

Higher stages are generally linked to more serious heart involvement and shorter survival times. European Modification (AL Amyloidosis)

A variation of the Mayo system, sometimes called the European Modification, uses NT-proBNP and cTnT and adds a Stage 3B for more severe cases:

- Stage 1 — Both biomarkers are normal
- Stage 2 — One biomarker is elevated
- Stage 3 — Both are elevated, but NT-proBNP is below 8,500 nanograms per liter
- Stage 3B — Both are elevated, and NT-proBNP is above 8,500 nanograms per liter

Stage 3B is associated with a more serious outlook. ATTR Amyloidosis Staging

For ATTR amyloidosis (both hereditary and wild-type), doctors use a cardiac staging system based on two markers — NT-proBNP and eGFR (a measure of kidney function):

- Stage 1 — Both markers are in the normal range | Median survival: ~5.8 years
- Stage 2 — One marker is outside the normal range | Median survival: ~3.9 years
- Stage 3 — Both markers are outside the normal range | Median survival: ~2 years

It's worth noting that median survival means half of people in that group lived longer than that timeframe — so these numbers aren't a definitive prediction for any one person. Staging for Nerve (Neuropathy) Involvement

For those with nerve-related symptoms, particularly in hereditary ATTR amyloidosis, doctors may use the Coutinho Staging System:

- Stage 0 — No symptoms
- Stage 1 — Mild symptoms, can walk with some difficulty
- Stage 2 — Moderate symptoms, may need help walking
- Stage 3 — Severe symptoms, requires a wheelchair

A more detailed system, the Polyneuropathy Disability Scoring System, further breaks this down based on mobility aid use.

---

Staging is a tool to help guide care — not a verdict. It's always worth having an open conversation with your care team about what your specific stage means for your treatment plan.

 AI-generated responses are not a substitute for medical advice. These services are not intended to diagnose, treat, or cure any condition or disease. Always ask your doctor about specific health concerns.

AL Amyloidosis Stages: Progression From Stage 1 to 4

Stages of Amyloidosis: Breakdown by Type and More