Myeloma

Typical myeloma is the most common type of myeloma. It is often referred to as just myeloma. It is classified by the paraprotein it produces. They are made up of one type of heavy chain and one type of light chain.

There are 5 types of heavy chain immunoglobulins (Ig):

• IgG
• IgA
• IgD
• IgE
• IgM.

There are 2 types of light chain immunoglobulins:

• kappa
• lamba.

In typical myeloma, the heavy chain is bound to the light chain to form the paraprotein. It is often present in the blood but not the urine. Blood tests are a good way to monitor this type of myeloma.

When the myeloma is active, there will be a rise in paraprotein levels. When the myeloma responds to treatment, there will be a decrease in paraprotein levels. It is normal for paraprotein to fluctuate a little bit. A small rise does not always mean that more or different treatment is needed.

Light chain myeloma occurs in about 15% of people with myeloma. It is also known as Bence Jones myeloma.

In light chain myeloma, myeloma cells only produce the light chain part of immunoglobulin. Not the heavy chain part. This is mostly detected in the urine.

Small increases in the number of light chains in the blood can be detected through a blood test. This is called a serum free light chain test. This test can be used to see how you are responding to treatment. It is also used to look at the kappa and lamba light chain ratio.

Non-secretory myeloma occurs in about 3% of people with myeloma. This type of myeloma produces no measurable paraprotein, so it cannot be detected in blood or urine tests, making it difficult to diagnose and monitor. Therefore, bone marrow biopsies and PET scans are used to diagnose and monitor this type of myeloma.

MGUS is a condition that affects plasma cells, which are part of the immune system. People with MGUS make an abnormal protein called an M-protein instead of normal antibodies. This protein can build up in the blood and urine and may reduce the body’s ability to fight infections.

MGUS does not usually need treatment, but regular monitoring is recommended to check for any changes.

About 20% of people with MGUS develop myeloma.

Smouldering myeloma is an early form of myeloma that may develop into active myeloma over time. It usually does not cause noticeable symptoms and is often found through a blood test.

Around 10% of people with smouldering myeloma may develop active myeloma within 5 years of being diagnosed

Solitary plasmacytoma is a condition that occurs when plasma cells become abnormal, multiply uncontrollably and form a tumour that affects the bones. Unlike some other conditions, it usually appears in only one specific area of the body.

Around 50% of people with solitary plasmacytoma may later develop myeloma.

Extramedullary plasmacytoma occurs when plasma cells become abnormal, multiply uncontrollably and form a tumour in soft tissues outside of the bones. It most commonly affects the tissues of the upper respiratory tract, such as the nose, throat and nearby areas.

Approximately 15% of people with extramedullary plasmacytoma may later develop myeloma.

A bone marrow biopsy is the best test to diagnose myeloma.

A bone marrow biopsy uses a needle to take a sample of bone marrow, usually from the back of the hip. A small amount of liquid bone marrow is removed and sent to the lab for testing. Often, a tiny piece of the bone itself is also taken to be examined.

Blood tests can detect abnormal proteins made by myeloma cells and show how the disease is affecting the body. Different types of blood tests include:

• full blood count (FBC)
• biochemistry tests including liver function, electrolytes and kidney function
• lactate dehydrogenase (LDH)
• cytogenetics
• beta-2 microglobulin
• serum free light chain assay.

Other tests you may have include:

• medical history and physical exam
• urine tests
• genetic tests
• computed tomography (CT) scan
• positron emission tomography (PET) scan
• magnetic resonance imaging (MRI).

The SLiM-CRAB criteria are used by your treatment team to help determine whether you have active myeloma that may need treatment. The criteria looks at different signs of the disease, including changes in plasma cells, blood tests, MRI results, kidney function, red blood cell levels and bone health.

The criteria are:

• S – 60% or more abnormal plasma cells in the bone marrow
• Li – high levels of light chains in the blood
• M – MRI with more than one bone abnormality
• C – high levels of calcium in the blood
• R – kidney damage
• A – low red blood cell count
• B – bone lesions or fractures.

Treatments for myeloma include:

Active monitoring (also known as watch and wait) involves regular blood tests and general health checks. No intervention is needed unless you develop signs and symptoms to suggest the myeloma is progressing.

People with smouldering myeloma and MGUS are generally actively monitored.

Supportive care controls symptoms of myeloma and side effects of treatment. It aims to improve your quality of life but does not intend to treat the disease. It can help with symptoms such as tiredness, breathlessness, bruising or bleeding and high blood calcium levels.

Supportive care may include:

• blood transfusions
• bisphosphonates – medications to strengthen bones
• supportive medications.

You can read more on our supportive care webpage.

Most myeloma treatment plans include steroids. They can be used on their own, but they are usually given with other treatments such as chemotherapy, targeted therapy or immunotherapy.

Steroids can:

• actively kill myeloma cells
• help other myeloma treatments be more effective
• act as an anti-inflammatory for people with myeloma bone disease
• act as an anti-nausea medication
• manage allergic type reactions to other myeloma medications.

Dexamethasone and prednisolone are 2 commonly used steroids. You can read more about them and their side effects on our corticosteroids webpage.

Targeted therapy is a cancer treatment that finds and blocks parts of cancer cells that help them grow and spread.

Proteasome inhibitors are an example of targeted therapy used to treat myeloma. They work by blocking the proteasomes inside myeloma cells. Proteasomes are structures that help cells break down and recycle proteins. When proteasomes are blocked, proteins build up inside the myeloma cells, causing stress and eventually destroying the cells.

Bortezomib, ixazomib and carfilzomib are examples of proteasome inhibitors.

You can read more about proteasome inhibitors and their side effects on our immunotherapy and targeted therapy webpage.

Immunotherapy is a type of biological therapy. It uses the immune system to recognise cancer cells and destroy them. Your treatment team will give this to you via a drip into a vein (intravenous) or a needle under the skin (subcutaneous).

Immunotherapy works by:

• stimulating the immune system to find and attack cancer cells
• removing barriers that are stopping the immune system killing cancer cells.

Daratumumab is a type of immunotherapy called a monoclonal antibody. It is used to treat myeloma. It damages cancer cells and causes them to die by attaching to a protein called CD38 on the blood cancer cell. It is given as an injection under the skin (subcutaneous), usually in an outpatient unit. 

Immune system modulators are also a type of immunotherapy used to treat myeloma. They stimulate the immune system to attack and destroy cancer cells. They can also stop their blood supply and directly destroy cancer cells.

Thalidomide, lenalidomide and pomalidomide are examples of immune system modulators used to treat myeloma.

You can read more about immunotherapy and their side effects on our immunotherapy and targeted therapy webpage.

Chemotherapy (often called chemo) is used to treat myeloma. Chemotherapy destroys cancer cells and stops them from growing. It also damages healthy cells and causes side effects. There is information on chemotherapy side effects and how to manage them on our chemotherapy webpage.

The type of chemotherapy recommended will depend on your stage of myeloma, the treatment goal, your age and medical history. Chemotherapy can be given in combination with targeted therapy or immunotherapy.

Treatment plans for myeloma are shortened to letters like CyBorD. The letters represent the name of the medicines in that plan. Your haematologist will provide you with information on your plan, including when and how often you will have it. 

There are several treatment plans for myeloma. They can be found on the Cancer Institute NSW website.

Radiation therapy, also called radiotherapy, is a type of treatment that:

• uses high energy x-rays to kill cancer cells
• is a local therapy because it only destroys cancer cells in the area it treats.

Radiation therapy is used in myeloma to:

• relieve and reduce bone pain
• shrink the amount of myeloma cells in the bones to slow down bone damage
• target specific growths, lumps or areas that need treatment without affecting the whole body
• treat in combination with other treatment
• prevent complications like further bone damage.

You can read more on our radiation therapy webpage.

A bone marrow or blood stem cell transplant is a treatment that restores stem cells after high dose chemotherapy. Your treatment team might suggest a transplant if your myeloma is aggressive, difficult to treat or has come back after a period of remission. There are 2 types of transplants:

• An autologous stem cell transplant  is when your own stem cells are collected and then given back to you after high dose chemotherapy.
• An allogeneic stem cell transplant is when you receive stem cells from a donor, often a blood relative.

It is more common to receive an autologous transplant for myeloma. There are some situations where an allogeneic transplant may be recommended.

CAR T-cell therapy is a type of treatment that uses your own immune cells to fight cancer. Your T cells are collected from your blood and changed in a laboratory so they can find and attack your blood cancer cells. After you have chemotherapy to prepare your body, the CAR T-cells are returned to you through a drip into a vein.

Your treatment team may recommend CAR T-cell therapy if your myeloma has relapsed or is refractory. Not everyone is eligible for CAR T-cell therapy. Your treatment team will discuss whether this treatment is suitable for you.

You can read more on our CAR T-cell therapy webpage.

Clinical trials may be available for some types of myeloma. They often compare new or combination therapies with current treatments, including side effects.

Many are randomised, meaning some patients receive the new treatment, and others receive standard care. Trials help improve future treatments and may offer access to costly new therapies not on the Pharmaceutical Benefits Scheme (PBS) in Australia.

If you are considering a clinical trial, your doctor will explain:

• the possible risks and benefits
• how it compares to standard treatment
• what is involved.

You will need to give informed consent before taking part. Read more on our clinical trials webpage.