What is multiple myeloma?
Multiple myeloma is a cancer of the bone marrow plasma cells, white blood cells that make antibodies. A cancerous or malignant plasma cell is called a myeloma cell. Myeloma is called “multiple” because there are frequently multiple patches or areas in bone where it grows.
How common is myeloma?
There are about 230,000 people are living with myeloma worldwide, and 114,250 new cases are diagnosed yearly. It is the 2nd most common blood cancer in the world. As of 2013 in the U.S., an estimated 95,688 people are living with myeloma. Additionally, an estimated 30,330 new myeloma cases were diagnosed in 2016.
Who is at risk for developing multiple myeloma?
Men are 1.5 times more likely than women to develop multiple myeloma. The disease is most frequently diagnosed in 65-74-year-olds, and it is twice as common in people of African descent.
What causes myeloma?
Exposure to toxic chemicals, atomic radiation, anything that interferes with the immune system, or infection with cancer-causing viruses have all been implicated as causes or triggers of myeloma.
Toxic chemicals that have been identified include:
- dioxins (such as those found in Agent Orange
- agricultural chemicals (such as defoliants and pesticides)
- engine exhausts
- cleansing materials
Several viruses have been identified as potential triggers, including:
- HIV and AIDS
- several herpes viruses
- Simian virus 40 (a contaminant in Sabin polio vaccine preparations used between 1955-1963)
Is myeloma hereditary?
Myeloma can also be hereditary. First-degree relatives of people with myeloma have a higher risk of developing myeloma or its benign (non-cancerous) precursor, monoclonal gammopathy of undetermined significance (MGUS).
If you have a family member (mother, father, brother, sister, son, or daughter) with myeloma or MGUS, mention this to your primary care physician so it is in your permanent medical record. Your doctor can then be more aware of early symptoms and signs, and can order appropriate testing if necessary. If you have myeloma, tell your family members to mention it to their doctors.
What are the symptoms of myeloma?
In 70% of patients, the most common symptoms of myeloma are back or bone pain, fatigue, and recurrent or persistent infections. Consult your physician if you are experiencing any of the following possible symptoms of myeloma
- Persistent or worsening tiredness
- Recurrent unexplained infections (such as pneumonia, sinus infection, or urinary tract infection)
- Back pain or any bone pain that is persistent or recurrent
- Swelling of the extremities
- Shortness of breath
How is myeloma diagnosed?
A myeloma patient most likely will need to see a hematologist/oncologist to receive a correct diagnosis. This doctor will ask about the patient’s symptoms, medical history, family history, and history of toxic chemical or radiation exposures. For an accurate diagnosis of myeloma, the patient may undergo a variety of tests, including blood tests, urine tests, imaging studies, bone marrow aspiration, or a core biopsy.
Learn more about tests used to diagnose myeloma at the 10 Steps to Better Care: Step 2: Tests.
What are the stages of myeloma?
When myeloma is diagnosed, the stage of the disease varies from patient to patient. The most commonly used clinical staging system, the Durie-Salmon Staging System, demonstrates the correlation between the amount of myeloma and the damage it has caused, such as bone disease or anemia.
Staging of myeloma can also be done according to prognosis, or expected survival. The most commonly-used staging system for myeloma that is based on prognostic factors is the International Staging System (ISS).
What are the different types of myeloma, and what do they mean?
Myeloma manifests as different types and subtypes. These types are based on the types of immunoglobulin (protein) produced by the myeloma cell. Normally, the various immunoglobulins have different functions in the body. Each immunoglobulin is composed of two heavy chains and two light chains.
There are five types of heavy protein chains: G, A, D, E, and M. There are two types of light protein chains: kappa (κ) and lambda (λ).
IgG myeloma with κ or λ light chains
- 65% of myeloma patients
- Has usual features of myeloma
IgA myeloma with κ or λ light chains
- Next-most common type
- Sometimes characterized by tumors outside the bone
IgD, IgE, and IgM myeloma
- Rare types
- IgD can be accompanied by plasma cell leukemia and can cause kidney damage.
“Light chain” or “Bence Jones myeloma”
- Present in 10% of myeloma patients
- Most likely to cause kidney damage, and/or lead to deposits of light chains in kidneys and/or on nerves or other organs.
- 1%-2% of myeloma patients
- Patients without detectable monoclonal protein
Visit the 10 Steps to Better Care: Step 1: Diagnosis to learn about how the type of myeloma you have is determined, and how each individual type “behaves.
If I’ve been diagnosed with myeloma, should I get a second opinion?
You should never be afraid to ask for a second opinion. If you feel you would like a second opinion, contact the IMF’s InfoLine for a recommendation to a hematologist/oncologist in your area. The InfoLine may be reached at 1-800-452-CURE (2873) in the US/Canada, or globally at 1-818-487-7455. You may also email them with your inquiry at InfoLine@myeloma.org.
Do I need a myeloma specialist for my care?
There may not be a myeloma specialist in the area for every patient. However, there is almost always a doctor who does have a focus or an area of specialization in myeloma. Ask your attending myeloma clinician how many patients he or she sees with multiple myeloma.
We encourage patients to see a true myeloma specialist at least once during the course of their disease. To identify a local or regional myeloma specialist, contact the IMF InfoLine at 1-800-452-CURE (2873) in the US/Canada, or globally at 1-818-487-7455. You may also email them with your inquiry at InfoLine@myeloma.org.
Finally, you can visit our listing of active myeloma support groups throughout the US. A leader of one of these groups may be a may be able to give you specific feedback on the visits that they have had with myeloma specialists near you.
What diet should I follow if I have myeloma?
As Dr. Brian G.M. Durie simply recommends, “Just eat real food.” For example, avoid processed foods and processed sugar. Our bodies are not used to unnatural ingredients that are mixed into some processed food. Organic food is highly recommended and can be purchased at lower prices these days. Also, practice a mixed diet (similar to a Mediterranean diet) with a focus on fruits and vegetables.
Should I avoid physical activity if I have multiple myeloma?
Multiple myeloma patients are living longer than ever before. Therefore, mobility, fall-risk assessment, and planned activity are important for these patients’ long-term care plans. Given the risks of injury and/or skeletal issues, multiple myeloma patients should have regular assessments evaluating their symptoms, enhancing their day-to-day functions, and intervening with proper assistance for the best overall health possible.
Your attending myeloma clinician may recommend a consult with a physical or occupational therapist to assess your physical activity level and needs for assistance
Common questions to discuss with your healthcare providers when considering your physical activity level include:
- How do you move in and out bed?
- How long can you tolerate exercise, e.g. can you comfortably ride a stationary bike for 10 minutes?
- Are you experiencing any pain in certain parts of your body?
For healthcare providers, an “exercise prescription” should consist of the four components of the FITT principle:
Frequency: the number of sessions per week
Intensity: How hard the person is exercising
Time: the duration of the exercise session
Type: the activity mode.
Before starting any exercise program, healthcare providers should assess a patient’s overall fitness and safety level to engaging in such exercise. Patients should also be educated about when to modify or abstain from their exercise regimens. As always, discuss your planned physical activities with your attending myeloma clinician.
What is the IMF doing for the prevention and cure of multiple myeloma?
The Black Swan Research Initiative (BSRI) is the International Myeloma Foundation’s signature research program aimed at developing a definitive cure for myeloma. Led by a team of global myeloma experts, the BSRI provides coordination and support for more than 40 research projects around the world. Launched in 2012, BSRI research is already resulting in tangible benefits for myeloma patients.
BSRI RESEARCH PROJECT HIGHLIGHTS
MRD testing: Next-Generation-Flow The BSRI team has defined and overcome the first obstacle to finding a cure for myeloma by developing a very sensitive, reliable, standardized, and affordable test to quantify and characterize any myeloma cells that may remain after a patient is in complete response (CR). The new test, known as Next-Generation Flow (NGF), is a flow cytometry technique paired with a specialized software package developed by researchers in Spain to detect even a single myeloma cell surviving among one million bone marrow cells.
Miniscule amounts of myeloma cells that remain after treatment can cause patients to relapse. The BSRI believes that detecting this minimal residual disease (MRD) through ultra-sensitive NGF technology, then eliminating it through the assertive treatment until MRD-negative is achieved and maintained for five years, represents the first step to achieving cure.
In October 2016, 120 researchers from myeloma laboratories around the globe gathered in Salamanca, Spain, for intensive training in NGF. Researchers from 60 labs in 26 countries worldwide attended the training workshop, as well as attendees from 12 sites in the US. The BSRI’s MRD Consortium represents centers in North and South America, Africa, Asia, Australia, and Europe. Three of the labs in the US – Mayo Clinic (Rochester, MN), Roswell Park Cancer Center (Buffalo, NY), and the SWOG Lab at the Levine Cancer Center (Charlotte, NC) – are reference labs to which patient bone marrow samples can be sent for MRD testing from any other hospital, clinic, or doctor’s office.
On March 10, 2017, the journal Leukemia published an article by members of the BSRI team on the design and validation of NGF. With accurate and accessible MRD testing, we have an endpoint to assess deep response.
Cure Trials: iStopMM—Early detection, treatment, prevention Launched in November 2016, Iceland Screens, Treats, Or Prevents Multiple Myeloma (iStopMM®) is the largest population-based screening study for myeloma and its earlier disease precursors that has ever been attempted. The treatment portion of the study is, along with the ASCENT and CESAR clinical trials, one of three BSRI-funded “Cure” trials.
The first step of the project is the screening of approximately 120,000 residents of Iceland who are over 40 years of age for evidence of monoclonal gammopathy of undermined significance (MGUS), smoldering multiple myeloma (SMM), or myeloma. Researchers, under the direction of principal investigator Dr. Sigurdur Kristinsson, calculate that approximately four percent of those screened will have MGUS, which will add up to over 4,000 MGUS patients with full accrual.
With its small and networked population, and centralized healthcare system, Iceland is the ideal place to conduct such a study. Not only will this project allow researchers to observe patterns of occurrence, but they will be able to follow subjects with early disease to see how it progresses. Moreover, monitoring patients with MGUS for many years will demonstrate which prognostic tests are most reliable as indicators of disease progression. We will also learn which patients benefit most from early intervention, as those identified with high-risk smoldering myeloma will be invited to participate in a treatment trial.
Because much of the Icelandic population has already undergone genetic coding, iStopMM researchers will be able to use this genetic data to look for linkages to family gene patterns in those with MGUS. In addition, a questionnaire asks participants about family history and exposure history to see what conditions may lead to the disease.
The chances are excellent that answers will be found to help the BSRI team understand the biology of why people develop MGUS and how it can be prevented.
Cure Trials: ASCENT—Treating high-risk smoldering myeloma with a 4-drug regimen The US-based ASCENT trial (Aggressive Smoldering Cure evaluating Novel Therapies) is headed by Dr. Shaji Kumar of the Mayo Clinic (Rochester, MN) and uses a combination of carfilzomib, lenalidomide, dexamethasone, daratumumab with or without stem cell transplant in an attempt to cure patients with high-risk smoldering multiple myeloma (HR-SMM).
The trial is, along with the CESAR and iStopMM clinical trials, one of three BSRI-funded “Cure” trials.
All enrolled patients will receive a reduced dose of carfilzomib, lenalidomide, and daratumumab for one year of maintenance. At this point, it is anticipated that approximately 80% of patients will achieve MRD negativity (versus 62% already achieved with KRd in the CESAR trial), said Dr. Brian G.M. Durie. MRD testing will be done at diagnosis and during therapy, allowing intensive tracking of the disease in patients. The number of cycles of therapy will be determined by the individual patient’s response.
The ASCENT trial will open for accrual in March 2018 at 12 IMF Consortium sites. Further information may be found here.
Cure Trials: CESAR—Treating high-risk smoldering myeloma with a 3-drug regimen Interim data presented at the 2017 Annual Meeting of the American Society of Hematology from the single-arm, phase II CESAR trial demonstrated a high rate of very deep responses in patients with high-risk smoldering myeloma. At the time of ASH, 62% of patients had achieved an MRD negative status. Patients were given induction therapy with carfilzomib, lenalidomide, and dexamethasone followed by high-dose therapy and autologous stem cell transplantation (HDT-ASCT), consolidation therapy and then maintenance therapy with lenalidomide and dexamethasone.
The CESAR trial, based in Spain and led by Dr. María-Victoria Mateos of University Hospital of Salamanca (Spain), is, along with the ASCENT and iStopMM clinical trials, one of three BSRI-funded “Cure” trials. Although longer follow-up is required, this curative strategy for high-risk SMM is encouraging.
Results of the CESAR trial were published in the journal Blood.
Combating resistant disease The Black Swan Research Initiative team has taken the first steps to achieve cure for a subset of patients—those who rapidly achieve MRD negativity with currently available therapies. The next challenge is to eliminate residual disease in patients who remain persistently MRD positive, and prevent the of development recurrent resistant disease. Major BSRI efforts are underway to study recurrent and resistant disease, and develop appropriate treatment protocols.
Multiple BSRI-sponsored clinical trials are being set up in the US, Europe, and the Asia-Pacific region to study and treat patients with MRD-positive disease. These studies will use new therapies to attempt to eliminate resistant MRD myeloma cells.
In addition, there are more than 40 BSRI projects under way around the globe to study various aspects of residual disease.
An ongoing study of long survivors will help better characterize and understand the nature of their disease, treatments, and response to treatment.
Learn more about the IMF’s Black Swan Research Initiative.