Stages of myeloma

The stage of your myeloma tells your doctor about how it is affecting you and how it might develop. Doctors use the international staging system to stage myeloma. There are 3 stages - 1, 2 and 3.

You have tests to look for symptoms of myeloma. Using these results, your doctor classifies your myeloma as either:

• symptomatic - you have symptoms
• smouldering - you don't have symptoms (asymptomatic)

The International Staging System

Doctors use the International Staging System (ISS) to stage myeloma. It looks at the results of blood tests. These measure the amount of these substances in the blood:

• ß2-microglobulin
• albumin
• lactate dehydrogenase (LDH)

You will also have blood tests to look for particular gene changes (mutations). You might hear this call cytogenetic tests. Doctors describe the results as low or high risk cytogenetics. 

 Doctors use this system to help them predict how you might respond to treatment. 

The ISS divides myeloma into 3 stages:

Stage 1 means:

• the level of the protein called beta 2 microglobulin (ß2-microglobulin or ß2-M) is less than 3.5 milligrams per litre (mg/L)​
• the level of albumin in the blood is more than 3.5 grams per decilitre (g/dL)
• normal LDH level
• low risk cytogenetics

Stage 2 means:

• the level of ß2-M is between 3.5 and 5.5 mg/L, with any albumin level OR
• the level of ß2-M is less than 3.5 mg/L and the level of albumin is less than 3.5 g/dL 
• normal LDH
• low risk cytogenetics

Stage 3 means:

• the level of ß2-M is more than 5.5 mg/L 
• high LDH level
• high risk cytogenetics

How doctors classify myeloma

The symptoms and signs (in blood tests and scans) that doctors look for are called CRAB, which stands for:

• calcium (high levels)
• renal (kidney damage)
• anaemia (low levels of red blood cells)
• bone damage 

There are other factors that your doctors use to decide about your treatment. They include:

• the number of plasma cells in the bone marrow biopsy
• levels of light chains in the blood or urine tests
• the number and size of areas of bone damaged by myeloma, showing on scans (such as MRI or CT)

Doctors then describe your myeloma as either:

• symptomatic (with symptoms)
• smouldering (asymptomatic-without symptoms)

Myeloma without symptoms (smouldering myeloma)

Asymptomatic myeloma is also called smouldering or indolent myeloma. This means you don't have symptoms or any tissue or organ damage. 

But you have one or more of these:

• paraprotein in your blood that is more than 30 g/L
• level of abnormal plasma cells in your bone marrow that is between 10% and 60%
• no features of CRAB (including bone lesions on scans that are not causing symptoms)
• low light chain levels in your blood or urine 

You don't normally have treatment for smouldering myeloma. But your doctor will want to monitor you very regularly for any symptoms. Smouldering myeloma may progress to symptomatic myeloma, but your doctor won't be able to say when this will happen. The risk of myeloma progressing is highest in the first 5 years after diagnosis. 

About 50 out of 100 people (50%) with smouldering myeloma develop symptoms and need treatment within the first 5 years. However, after 5 years the risk decreases and some people never develop symptoms or need treatment. 

Myeloma with symptoms (symptomatic myeloma)

If you have symptomatic myeloma you need to have treatment. Symptomatic myeloma means you have:

• abnormal plasma cells in your bone marrow,
• an area of abnormal plasma cells in your bone or soft tissue (plasmacytoma)
• damage to tissues or organs (such as bone problems, high calcium levels, kidney problems or low levels of red blood cells – anaemia)

And any one or more of the following:

• more than 60 out of 100 (60%) abnormal plasma cells in your bone marrow
• a serum free light chain ratio of more than 100
• more than 1 area of damage (involving your bone or bone marrow) on an MRI scan

Treatment

Treatment mainly depends on:

• your symptoms
• the results of your blood tests, bone marrow tests, and scans
• your general health and levels of fitness
• your personal wishes

You might have a combination of:

• chemotherapy such as cyclophosphamide or melphalan
• targeted drugs such as thalidomide, lenalidomide, bortezomib or daratumumab
• steroids such as dexamethasone or prednisalone

Most people have their treatment as an outpatient. Your treatment might be tablets or tablets and an injection. The injection is either just under your skin or into your blood stream.

Some people also need antibiotics to prevent infections or tablets to treat bone or kidney problems.  

If you are fit enough, and your myeloma is under control with the first treatment your doctor might suggest intensive treatment with high dose chemotherapy and stem cell transplant.  You usually have 4 to 6 months of chemotherapy and targeted cancer drugs before you can have a transplant.

Once your myeloma is under control (in remission), you might have a targeted drug. This aims to keep it under control for longer and is called maintenance therapy. 

If you aren’t fit enough for high dose treatment and a transplant you might have either 8 months of your first treatment. Or you may continue with treatment. It depends on the type of treatment you are having and what you want. Your doctor will think about the benefits of the treatment and the possible side effects.

Relapsed myeloma

Myeloma usually responds well to treatment and goes into a complete or partial remission. Remission means there is no sign of active disease in your body, or your bone marrow. Or, the abnormal proteins can stay at a reduced level (called a plateau). Abnormal protein levels staying below half (50%) is called stable disease or partial remission.

You specialist (haematologist) monitors how your myeloma is responding throughout treatment. 

If the first treatment you have doesn’t work, you might need a change to your treatment. This is to get the myeloma to go into remission or to stabilise.

Sometimes, myeloma responds at the start of treatment, then comes back or the protein level rises again. This is called recurrent (relapsed) myeloma. The time between the first treatment and myeloma coming back (relapse) varies from person to person. 

Treatment at relapse

Your treatment depends on your individual situation, such as how long you were in remission for, what treatment you have already had, and your current level of health and fitness.Your doctor may suggest a different type of treatment.

You might have treatment with:

• targeted drugs
• chemotherapy
• steroids

Treatment to control symptoms

Bone pain is a common symptom. A drug treatment called bisphosphonates can help to reduce the bone damage that is causing your pain. These can also lower the amount of calcium in your blood which if it is high can cause symptoms such as sickness and constipation.

You will also have painkillers to take to help control bone pain. This might include paracetamol, co-codamol or stronger pain killers. Let your team know if the pain killers you are taking aren’t working. You may need to try different ones before you find one that works.

You might also be prescribed medicines to stop indigestion, sickness or infections. You my need blood thinners (such as heparin).

You might have one or more of these treatments:

• radiotherapy to an area of myeloma that is causing pain
• surgery to repair a broken bone or lower the risk of bone fractures
• plasma exchange (plasmapheresis) to lower protein levels in the blood and reduce symptoms such as headaches
• blood transfusions to treat tiredness (caused by low red blood cell levels)

This page is due for review. We will update this as soon as possible.