Multiple myeloma, also called as myeloma, plasma cell myeloma or Kahler’s disease, is a malignant degeneration of the plasma cells (also known as plasma B cells, plasmocytes). In other words, you have multiple myeloma when there is an inappropriate and excessive production of plasma cells in your body.
Multiple myeloma is a cancer that develops from white blood cells, a group of blood cells formed in the bone marrow. These cells, forming part of the immune system, produce antibodies (immunoglobulins) to combat infections and pathogenic attacks capable of causing diseases. Myeloma occurs when plasma cells multiply and invade the bone marrow at higher than normal level. These turbulent cells (myeloma cells) take the place of healthy blood cells to form, in the skeleton, multiple osteolytic tumors.
Without an early and effective treatment, myeloma cells will eventually attack your bones, causing pain and increasing your risk of fractures. As the cancer progresses, myeloma cells can also spread into your kidneys and nervous system, leading to fatal problems.
Multiple myeloma represents approximately 10% of hematologic malignancies and 1% of all cancers; it is responsible for 2% of all cancer deaths. Each year, multiple myeloma affects about 5 out of 100 000 individuals aged 50 or less; and 20 out of 100 000 individuals aged 80 and over. In the United States alone, nearly 45 000 people are living with myeloma, with an estimate of 20 000 new cases per year. According to the American Cancer Institute (ACI), it is estimated that 11,680 men and 8,900 women (20,580 men and women) were diagnosed with myeloma in 2009; 10,580 people died of the disease.
The incidence of multiple myeloma, however, varies with age. From 2002-2006, the median age at diagnosis for myeloma was 70 years of age. The percentages and ages of people diagnosed were approximately:
- 0% under age 20;
- 5% between 20 and 34;
- 3% between 35 and 44;
- 9% between 45 and 54;
- 7% between 55 and 64;
- 5% between 65 and 74;
- 6% between 75 and 84;
- 5% 85+ years of age.
Multiple Myeloma Causes
In your bone marrow, specifically in the lymphoid tissue, live a variety of lymphoid cells. Their main role is to secrete immunoglobulins (antibodies) to fight infection and other pathogenic conditions. This work must be done constantly to keep your body healthy. Multiple myeloma occurs when, due to certain factors, plasma cells multiply excessively in several regions of the bone marrow. Instead of producing normal antibodies (immunoglobulin) to protect your organism, myeloma cells produce an abnormal immunoglobulin called monoclonal protein (or M protein).
In addition, this disorder can result in formation of cells called osteoclasts. Osteoclasts are cells responsible for bone resorption. They act by lowering the pH in contact with the bone matrix and secreting proteolytic enzymes, leading to deterioration in the bone structure and increased risk of fractures and pain.
Multiple Myeloma Risk Factors
The causes of this abnormal change leading to myeloma are not well known. Medical Researches, nevertheless, have detected several factors that may contribute to the development of these abnormalities in the plasma cells.
- Age– myeloma is especially diagnosed in people over 50 years;
- Sex– myeloma is more common among men than women;
- Race– multiple myeloma is about twice higher among black Americans of African descent than racial groups;
- Radiation exposure– you are at risk of myeloma if you are constantly exposed to high doses of radiation;
- Family history of multiple myeloma– if you have a close relative suffering from myeloma, your risk is higher;
- History of a monoclonal gammopathy of undetermined significance (MGUS)– according to the staff of Mayo Clinic, about 1% of MGUS patients in the United States develop multiple myeloma;
- Obesity– excess fat in your body is a causative factor for all cancers including multiple myeloma.
Some researchers believe that exposure to certain chemicals used in agriculture and the oil industries can be possible causes of multiple myeloma. Although not yet confirmed, as far as you can, avoid exposure to these substances.
Multiple myeloma can remain asymptomatic for many years; the disease continues its physiological damage without causing noticeable symptoms. In most cases, signs and symptoms begin to emerge after a long period after the tumor is advanced.
Many patients find out they have multiple myeloma during a routine blood test or a diagnostic for another medical condition. As myeloma affects the bones of the spine, the first symptom is often a pain in the back, ribs, neck or pelvis.
The most common multiple myeloma symptoms include:
- weakness and fatigue
- easy bruising
- weight loss
- blurred vision
- unexplained bone fractures
- leg weakness or numbness
- excessive thirst and urination
- loss of appetite
- nausea and vomiting
- loss of sensation
- headache and dizziness
- purple patches under the skin
- unusual nose or gums bleeding
- confusion, disorientation, difficulty concentrating
- frequent infections, mostly pneumonia, sinusitis, bladder or kidney infections, skin infections
- Muscle weakness associated with decreased muscle reflexes.
Myeloma is a serious disease that can result in many complications; the most common include:
Anemia – uncontrolled growth of myeloma cells may cause decreased production of blood platelets and inhibition of normal red blood cells.
Infection – normally, the role of anti-bodies (immunoglobulins) is to fight infection; however, presence of antibodies in very large amounts (myeloma cells) inhibits the synthesis of other normal immunoglobulins, which no longer allow the body to cope with infectious agents. Although the list of infection that can be due to a weakened immune system is enormous, the most common infections that are related to myeloma are pneumonias and urinary tract infection (UTI).
Neurological disorders – multiple myeloma can also lead to neurological disorders such as confusion and fatigue, severe headache, visual changes and retinopathy, and other problems of the peripheral nervous system. In severe cases, you may also experience:
- loss of bowel control
- bladder problems
- carpal tunnel syndrome
- radicular pain
Kidney disorders – myeloma complications can lead to kidney problems including kidney failure. Kidney problems can be due:
- recurrent pyelonephritis
- high level of uric acid in the blood (hyperuricemia)
- elevated calcium level in the blood (hypercalcemia)
- abnormal accumulation of fibrous protein in your blood
- Tubular damage from excretion of monoclonal globulin protein.
Bone disorders – problems such as bone pain, fractures, vertebral collapse are commonly associated with myeloma. Without appropriate treatment, paralysis in the legs can occur. In addition, dissolving of bone can also cause release of high levels of calcium in the blood, leading to hypercalcemia and its symptoms:
- loss of appetite
- nausea and vomiting
- excessive thirst
- frequent urination
- abdominal pain
- muscle weakness
- muscle and joint aches.
Many patients find out they have multiple myeloma during a routine blood test or a diagnostic for a medical condition that has nothing to do with myeloma. However, if you go to hospital because you experience symptoms related to multiple myeloma, your doctor will do test specific to myeloma to confirm the disease.
In general, before moving to sophisticated diagnostic techniques, your doctor will question you about your health, your medical history, and perhaps that of your family. After which, he will examine your body in search of signs that indicate multiple myeloma. These procedures, however, are not sufficient to declare accurately the disease; other special tests need to be performed to confirm a myeloma diagnosis.
Blood and urine tests – during these tests, a specialist will collect urine and blood samples to determine the quantity and quality of different substances in your blood cells. These tests also help your physician to evaluate the functioning of certain organs such as your kidneys.
Your physician will recommend a blood test called serum protein electrophoresis (SPEP) to detect presence of abnormal proteins such as M protein, an antibody found in unusually large amounts in the blood or urine of patients with multiple myeloma; or Bence Jones protein, light chains of immunoblobulin found in the urine, of which increased levels may indicate multiple myeloma. In addition, the blood test helps your doctor to know whether the concentrations of calcium in the blood are higher than normal.
Imaging techniques – some imaging exams such as x-rays of your skeleton, magnetic resonance imaging (MRI), computerized tomography (CT) scan or positron emission tomography (PET) scan can be performed to help your doctor do a thorough analysis of your organs, tissues and skeleton.
Biopsy – a biopsy is a key exam in the diagnosis of multiple myeloma. It allows your doctor to establish with certainty the presence of the cancer. It involves removing cells or tissue of bone marrow for laboratory analysis. If cancer cells are found, your physician will perform other tests to determine their rate of spread (staging).
Once it is confirmed that you have multiple myeloma, further exams are needed to determine the stage of the cancer. This is an important step; it helps your doctor to choose the most appropriate treatment to fight the disease. In general, the stage of cancer depends on the tumor size and degree of spread.
There are several methods to describe the multiple myeloma stages, but the classification system mostly used is the Durie–Salmon. Durie-Salmon Myeloma Staging System is based on 3 stages: stage I, stage II and stage III.
Stage I – the cancer is considered as a “stage 1 myeloma” when the following criteria are met:
- low number of cancer cells
- slightly low hemoglobin level
- blood calcium levels can be normal
- X-ray has revealed only 1 area of bone damage
- Blood and urine test has revealed small amount of monoclonal immunoglobulin.
Stage II – at stage 2, the cancer can present the same characteristics as stage 1, the only difference is that the number of myeloma cells has increased.
Stage III – in this last stage, the cancer has become more aggressive, and your chance to survive has decreased considerably. The cancer is considered as a “stage 3 myeloma” when the following conditions are present:
- large amount of myeloma cells
- hemoglobin level is below 8.5 g/dL
- blood calcium level is above 12 mg/dL
- three or more areas of bone are affected
- Large amount of monoclonal immunoglobulin in blood or urine.
There is no cure for multiple myeloma. There are, however, therapeutic approaches to improve your life. Even if you do not experience symptoms, it is important that you regularly visit your doctor in order to monitor the disease, and prevent complications.
If you have a symptomatic myeloma associated with high levels of M protein and calcium in your blood or your urine, and/or impaired renal function, your doctor will recommend treatments to control the disease. In general, your doctor will prescribe one or a combination of these therapies:
- stem cell transplant
Chemotherapy is the most common cancer treatment. It consists of using strong chemical substances to damage myeloma cells, preventing them from reproducing. Chemotherapy drugs work by attacking cells that multiply rapidly, the main characteristic of cancer cells. Unfortunately, these chemicals also damage normal cells that multiply rapidly and lead to side effects:
- hair loss
- fever or chills
- black, tarry stools
- joint pain
- lower back or side pain
- nausea and vomiting
- chest pain or tightness
- allergic reactions (rash, hives, etc.)
- difficulty breathing
- swelling of the mouth, face, lips, or tongue
- irregular or absent menstrual periods
- blood in urine or stools.
Chemotherapy drugs can be administered orally or by intravenous injection. In myeloma treatment, chemotherapy drugs are recommended to be taken for several months. If the level of M proteins stabilizes, your doctor may decide to stop the chemotherapy. The drugs most often used in the treatment of multiple myeloma include:
- Melphalan (Alkeran)
- Cyclophosphamide (Cytoxan)
- Vincristine (Oncovin)
- Doxorubicin (Adriamycin)
- Liposomal doxorubicin (Doxil)
In myeloma treatment, this method always involves a combination of high-dose chemotherapy with an infusion of healthy stem cells to replace damaged bone marrow. A stem cell transplant is recommended to help your body make enough healthy white blood cells, red blood cells or platelets, and reduce your risk of infections, anemia and bleeding. These stem cells can be originated from your body (autologous stem cell transplant) or that of a donor (allogeneic stem cell transplant).
Radiation Therapy (Radiotherapy)
Radiation therapy consists of using X-rays of high energy to destroy myeloma cells. Unlike chemotherapy, radiation therapy is local-regional therapy; it treats a specific part of your body, the region where the tumor has developed. Usually, the therapy must be performed once a day, four or five days a week for several weeks.
Immunotherapy (Biological Therapy)
Immunotherapy is a therapeutic method involves the use of medical substances in order to stimulate the immune system to attack the myeloma cells. Cancer immunotherapy includes many medications; however, in the treatment of multiple myeloma, the drug most often used is interferon. It works by slowing the growth of myeloma cells, and help your body produces enough healthy stem cells. Depending on the reaction of your body to the drug, it can prolong your life.
Other medications – Other drugs commonly used in the treatment of multiple myeloma include Bortezomib (Velcade), Thalidomide (Thalomid) and Lenalidomide (Revlimid).
Myeloma Survival Rates
Multiple myeloma prognosis depends on the stage of the disease when it was diagnosed.
Approximately, the median survival is:
- 62 months for stage 1
- 45 months for stage 2
- 29 months for stages 3
In addition, the prognosis differs from one race to another. According to the National Cancer Institute (NCI), the overall 5-year relative survival rate for 1999-2005 from 17 SEER geographic areas was 37.1%. Five-year relative survival rates by race and sex were:
- 6% for white men;
- 9% for white women;
- 0% for black men;
- 2% for black women.
The best way to prevent the occurrence of any cancer is to live a healthy lifestyle. Myeloma is not attributable to a unique cause; several factors can cause the formation of cancerous cells in your bone marrow. Some risk factors such as sex and age are impossible to change; others, however, can be prevented. If you want to prevent myeloma and all other cancers, it is necessary to live a healthy lifestyle and eat a healthy diet. Below, are some tips that can help you:
- do not consume alcohol regularly and/or in large quantity; doing so helps you prevent many types of cancer including multiple myeloma.
- avoid tobacco smoke, including second hand smoke
- maintain a healthy weight;
- reduce your exposure to toxic chemicals
- reduce your exposure to ultraviolet (UV) radiation
- exercise regularly
- practice safe sex
- keep your immune system healthy
- tell your doctor or dentist of any change that indicates cancer
- adopt a healthy diet: a diet containing 5 to 10 servings of fruits and vegetables a day may help prevent occurrence of many cancers. Increase the following foods in your diet:
- a) avocados
- b) blueberries
- c) black Raspberries
- d) curcumin
- e) flax
- f) garlic
- g) papaya
- h) pomegranate juice
- i) tomatoes
- j) soy products
- k) grapefruits, oranges and other citrus fruits
- l) cruciferous vegetables (broccoli, cauliflower, kale, Brussels sprouts, and cabbage)