Multiple myeloma is a treatable but incurable blood cancer that typically occurs in the bone marrow. It is a relatively uncommon cancer, affecting approximately 30,000 new people each year. 1 Difficult to diagnose until it is in the advanced stages, it is mainly treated with chemotherapy and stem cell therapies. But the survival rate is increasing, especially as advances in treatment are being discovered. Here are the ten things you need to know about the disease.
Please note: nothing can replace the care of your clinician or doctor. Please do not make changes to your treatment or schedules without first consulting your healthcare providers. This article is not intended to diagnose or treat illness.
1. What is multiple myeloma?
Multiple myeloma is a type of cancer that typically occurs within a bone due to the presence of malignant plasma cells. Under normal circumstances, plasma cells develop from B cells—a type of cell that the immune system uses to fight disease or infection. When B cells react to an infection or disease, they change into plasma cells, which are responsible for creating antibodies to help fight germs. These plasma cells are found mainly in bone marrow.
Sometimes, after plasma cells develop, they can begin to grow out of control and create a tumour called a plasmacytoma. These tumours generally develop within a bone but can occasionally be found in other body tissues. When a person develops more than one of these tumours, they have multiple myeloma.
2. Risk factors and causes
Unlike many other cancers, there are very few known risk factors associated with getting multiple myeloma. These factors are listed below.
Age: The majority of diagnoses are in people who are more than 45 years old (96 percent), and more than 63 percent of diagnoses are in people older than 65. Less than one percent of cases are in people younger than 35.2
Race: For reasons unknown, it is more than twice as common in African-Americans than in white Americans.
Gender: Men are at a slightly higher risk than women.
Family history: A person with a parent or sibling who has the disease is four times more likely to get the disease, too.
Obesity: Being overweight or obese increases the risk.
Having other plasma cell diseases: A person with solitary plasmacytoma (a single tumour), or someone diagnosed with monoclonal gammopathy of undetermined significance, which is a plasma cell disorder that does not normally cause problems, is more likely to later develop multiple myeloma.
Radiation: People exposed to are at a higher risk.
Workers exposed to ionizing radiation have been shown to have an increased risk of the disease as well, according to a study conducted at US Department of Energy facilities.
Workplace exposure: Some studies have shown that workers in occupations such as agriculture, leather, petroleum and cosmetology, and workers exposed to chemicals such as asbestos, benzene, and pesticides are at an increased risk.
Researchers do not have a clear understanding of what causes multiple myeloma, though they have made progress into better understanding how specific DNA changes can cause plasma cells to mutate. Studies show that abnormalities in genes called oncogenes, which promote cell division, develop early in the growth of plasma cell tumours. Studies also show that myeloma cells have abnormalities in their chromosomes; specifically, research has revealed that pieces of chromosome 13 are missing.
Research also shows that in approximately half of people diagnosed with multiple myeloma, a translocation has occurred. This is when “a part of one chromosome has switched with a part of another chromosome in the myeloma cell.” 4 Scientists have also discovered that people with plasma cell tumours have abnormalities in other bone marrow cells, which might cause too much plasma cell growth.
The early stages of multiple myeloma may not have any symptoms, and even when symptoms are present, they may be similar to those that occur with other conditions. Below are some of the common symptoms of the disease:
*Bone pain and/or bone fractures
*Increased risk of infections
*Loss of appetite/weight loss
*Restlessness that is later followed by significant fatigue and weakness
*Problems with kidney function
4. Positive results from targeted therapies
There are many drugs available to treat multiple myeloma, with chemotherapy and autologous stem cell transplants (when stem cells are collected from the patient) the most common, but several of the most recent and exciting treatments to become available are two medications called daratumumab and ixazomib, and a form of treatment known as immunotherapy.
Darzalex (Daratumumab): In November 2015, the FDA granted “accelerated approval” for daratumumab injections in the treatment of multiple myeloma. The drug may only be used by individuals who have already undergone at least three other types of therapy.
Darzalex is part of a category of drugs called monoclonal antibodies. It works by binding to a protein called CD38, which is typically found on the surface of myeloma cells. Once it is attached to the cell, the drug attacks the cell while simultaneously signaling to the immune system to fight against the cells.
Almost one-third of clinical trial participants (with a median of five previous therapies) responded positively to the drug. Daratumumab can be purchased via our products page.
Ixazomib: Recently approved by the FDA, this completely oral treatment is used in combination with standard myeloma drugs to treat people who have already undergone at least one previous therapy. Clinical study results showed that the drug taken in combination with lenalidomide and dexamethasone increased “progression-free survival (PFS) in patients with relapsed/refractory multiple myeloma.” You find more information about Ixazomib on our products overview.5
Immunotherapy: Immunotherapy is when a person’s immune system is used to treat an infection or disease. In a recent study, scientists discovered that 70 percent of people with multiple myeloma who were treated with immunotherapy had a “significant clinical response” to the disease. 6 In the study, 14 of the 20 participants with an advanced form of multiple myeloma had a “near-complete or complete response three months after treatment; median progression-free survival was 91.1 months, while the overall survival lasted 32.1 months.” 7 Further, no significant side effects were reported. This is an important advance, given that current treatments such as chemotherapy and autologous stem cell transplants have low long-term responses and an average survival rate between three and five years. 8 *Update January 2018 – Empliciti (elotuzumab), is a type of immunotherapy that was approved in the US, Europe, and Australia, but has still not been approved in many other parts of the world and is therefore not directly available there. For more information head to our medicines overview page.
Despite advancements in the treatment of multiple myeloma, not all medicines are available in the same countries at the same time. This can be due to delays in initial approval by one regulatory body and approvals within a certain country — if the manufacturer has filed for approval in that country. There is no global, harmonised approval system and it’s up to manufacturers to decide where to go to market first (also known as applying for market authorisation). Regulatory bodies also differ in speed, which can cause delays.
Almost any country in the world allows individuals to import elsewhere approved medicines for personal use, which may give multiple myeloma patients access to new-to-market medicines. If you are seeking a medicine not yet available in your country, head to our home page to find out how our team can help.
5. Tom Brokaw is living with the disease and has written a book about it
NBC News anchor Tom Brokaw was diagnosed with multiple myeloma in August 2013 following a bout of severe back pain. Though he originally wanted to keep the diagnosis private, he eventually announced his fight against the disease. His memoir, A Lucky Life Interrupted, was published last year and details his journey following his diagnosis. In it, Brokaw discusses the challenges he faced from the disease: weight loss, the inability to sometimes walk without help, the side effects from his medications, and the moment when he learned that the disease was affecting 60 percent of his blood. After 16 months of treatment, his cancer went into remission.
6. How it’s diagnosed
Several different diagnostic tests must be used to confirm a multiple myeloma diagnosis because it is challenging to diagnose based on a single laboratory result. A physical evaluation will be done alongside a review of the individual’s history, symptoms, blood and urine tests, and a bone marrow biopsy. Other tests might include an MRI, CT scan, PET scan and X-rays.
In order to definitively diagnose multiple myeloma, a person must meet at least one major and one minor or three minor criteria. Those criteria are:
*Plasmacytoma (based on a biopsy)
*The existence of 30 percent plasma cells in a bone marrow sample
*Increased levels of M protein in either blood or urine
*10 percent to 30 percent plasma cells in a bone marrow sample
*A minor elevation in M protein levels in blood or urine
*Low levels of antibodies (that are not produced by cancer cells) in the blood.
7. Stages and classifications
The criteria as discussed above helps doctors determine not only whether a person has the disease, but also under which classification the disease falls.
Those classifications are:
*Monoclonal gammopathy of undetermined significance (MGUS)
*Asymptomatic myeloma, which is then divided into two subcategories:
Once the classification is known, a doctor will then determine which stage of the disease exists, which will help establish the prognosis and treatment options.
The most common way to diagnose the stage of the disease is through the International Staging System (ISS), which is based on two different blood test results: the beta 2-microglobulin (β2-M) and the albumin. There are three stages of classification under the ISS:
Stage I: β2-M less than 3.5 mg/L and albumin greater than or equal to 3.5 gm/dL
Stage II: Either β2-M greater than 3.5 mg/L but not greater than 5.5 mg/dL and/or albumin less than 3.5 g/dL
Stage III: β2-M greater than 5.5 mg/L
The Durie-Salmon Staging System is an older system of diagnosis. This uses four measurements to determine which stage of the disease exists: 1) the amount of hemoglobin in the blood; 2) the amount of calcium in the blood; 3) the production rate of M protein; and 4) the number of bone lesions. The disease’s stage is then further subdivided based on kidney function.
The three stages of the disease as determined by the Durie-Salmon Staging System are: Stages I, II and III. Each of these stages is then subdivided into either Stage A or Stage B based on whether kidney function is affected. (Stage B means there is significant kidney damage.)
Stage I: Though a person with Stage I often shows no symptoms of the disease because there are fewer cancer cells present in the body, other signs will be present, such as: amount of red blood cells within or a little below the normal range, a normal amount of calcium in the blood, low levels of M protein in the urine or blood.
Stage II: More cancer cells are present in the body than in Stage I. An individual who does not fit into either Stage I or Stage III is said to have Stage II.
Stage III: There are many cancer cells present. Other characteristics of this stage include; hypercalcemia, high levels of M protein, anemia, and significant bone damage.
Note: In any stage, if kidney function is affected, the prognosis will be worse.
8. It’s treatable, not curable
The most common multiple myeloma treatment has typically been chemotherapy followed by stem cell transplants. Because the disease is not curable, this method of treatment aimed to create longer and longer stretches of time during which it did not progress. Now, however, significant advances in research have dramatically changed not only the prognosis but the treatment that is offered. In fact, treatments have advanced so much that there is an increasing discussion among the scientific community as to whether a stem cell transplant should be done after diagnosis or if it is better to wait until a relapse.
Scientists are currently experimenting with different combinations of medications to increase the survival rate. For example, efforts are being made to combine certain drugs that not only have diminished side effects but that also “lengthen stretches of progression-free survival (PFS). 9 Other drugs are being studied to see how they can work with the body’s immune system to fight the disease.
“It’s a massive convergence of our understanding of biology, the technology becoming available to understand myeloma cells and how they respond, the genetic subtypes of myeloma, the ability to engage both the patient community and researcher, to transfer data and information,” says Walter Capone, president and CEO of the Multiple Myeloma Research Foundation, in an article with Cure. 10
9. There’s an international support network
The International Myeloma Foundation—while not an official sponsor of the more than 150 multiple myeloma support groups around the world—conducts yearly conferences for support group leaders. Information on support groups according to an individual’s geographical location can be found on the IMF website.
10. The survival rate continues to increase
According to Cancer Research UK (which used data from 2010-2011), 78 percent of men diagnosed with the disease survive for at least one year and 50 percent survive for five years or longer. For women, that number is 75 percent for one year and 44 percent for at least five years or longer. 11
In the United States, researchers reported that a “newly diagnosed myeloma patient 15 years ago, for example, was about one-third as likely as someone without myeloma to live another five years.” 12 Those same researchers found that “By the end of the 2000s, in contrast, that same myeloma patient would be 45 percent as likely as someone without myeloma to live another five years.” 13 According to the American Cancer Society, the median survival rate for Stage I is 62 months; Stage II: 44 months; and Stage III, 29 months. 14
With advances in treatment, as well as ongoing clinical studies, those prognoses continue to increase. In fact, the prognosis today of someone diagnosed with the disease is nearly triple what it once was. 15