Thanks to a host of effective new therapies, patients are enjoying years in remission from this malignant blood cancer. If there's one disease that most shows the effects of recent improvements in cancer treatment, it's multiple myeloma. Advances in biotechnology have enabled scientists to identify several genetic and chromosomal abnormalities underlying the disease, while fostering the development of more effective and less toxic drugs. Although there's still no cure for myeloma, various treatments are bringing about lengthy remissions, and people who were once given two to three years to live are successfully managing their condition for five to seven years, with some living a decade or longer. What is multiple myeloma? The word myeloma derives from the Greek roots myelos (bone marrow) and oma (tumor); it's "multiple" because the tumors occur at many places in the bone marrow. The cause of the disease is unknown. It originates in plasma cells — white blood cells in the marrow called B lymphocytes, which differentiate in response to the presence of viruses and bacteria (pathogens) in the body. Normally, plasma cells make a different antibody to fight each type of invading pathogen. Thus, whenever that pathogen enters the body, the plasma cell becomes activated, reproducing in large numbers and generating enough antibodies to rid the body of the germ in question. Myeloma cells are abnormal plasma cells that multiply for no apparent reason, producing substances that suppress normal marrow function and crowding out the normal blood components in the marrow — oxygen-carrying red blood cells, infection-fighting white cells, and platelets, which aid clotting. Consequently, people with myeloma may have anemia, lowered resistance to disease, and abnormal bruising and bleeding. Instead of functioning antibodies, myeloma cells generate excessive amounts of an abnormal protein called M protein, which can damage the kidneys as well as suppress normal immune function. As myeloma cells accumulate in the marrow, they excrete enzymes that increase the rate of bone breakdown (resorption) and slow the rate of bone formation. As a result, calcium stored in bones is released into the blood, where high calcium levels may cause weakness, confusion, and further kidney damage. The disease can also metastasize beyond the marrow and bone — that is, it can spread from the bone marrow to other parts of the body, including the liver, spleen, and soft tissues. Myeloma can thus wreak havoc with the bones and kidneys, and in advanced cases, the liver, spleen, and lungs, as both M protein and plasma cells build up in these various organs. Risk factors for myeloma There's little evidence so far that we can do anything to prevent myeloma. The most common risk factors are outside our control. They include the following: Age. Myeloma is rare in people under age 35 and most common in people ages 65 and older. Ethnicity. The disease is almost twice as common in African Americans as it is in Caucasians. It's generally less common in Asians. Gender. Slightly over 55% of people with myeloma are male. Genetics. Having a close relative with myeloma may increase risk, but familial myeloma is uncommon. Monoclonal gammopathy of unknown significance (MGUS). This benign condition affecting plasma cells is often an incidental finding during a routine blood test. Its incidence increases with age. MGUS requires no treatment, but anyone with the condition should be carefully monitored over time, as up to about 25% of people who have it will ultimately develop myeloma. Diagnosing and staging multiple myeloma Early multiple myeloma doesn't always produce symptoms, but your clinician's suspicions may be aroused by abnormal results on a routine blood test or an unexpected broken bone. More often, the disease is signaled by symptoms like fatigue, weakness, back pain, frequent urination, constipation, and susceptibility to infection. In either case, the following laboratory and imaging tests can rule out or confirm the presence of multiple myeloma and determine the stage of the disease: Blood tests that identify the number of red cells, white cells, and platelets; the presence of M protein; and levels of antibodies, the proteins beta-2 microglobulin and albumin, calcium, and creatinine (an indicator of kidney damage). Urine testing that measures levels of another abnormal protein, called Bence Jones protein. X-rays to check for damaged or broken bones. A bone marrow biopsy to detect myeloma cells. Studies have shown that there are several different kinds of myeloma cells with different genetic and chromosomal abnormalities, causing different forms of the disease that respond to different treatments. Tests for these abnormalities are used to identify the 25% to 30% of people with myeloma who are at especially high risk and likely to need early aggressive treatment. Myeloma that's discovered before symptoms occur — with levels of beta-2 microglobulin only slightly higher and albumin levels slightly lower than normal — is usually described as inactive or smoldering or stage I disease. Studies haven't shown that drug treatment at this stage extends survival time, so the standard practice is "watchful waiting" — monitoring patients closely with physical exams and lab tests to determine whether the disease is advancing. What's a stem-cell transplant? Although not curative, stem-cell transplantation is a mainstay of myeloma treatment, especially for younger (ages 65 to 70), healthier patients. Stem cells have the capacity to regenerate healthy red cells, white cells, and platelets, so they can be used to help people withstand chemotherapy at doses high enough to kill myeloma cells — a strategy that also destroys most of the healthy blood components. After therapy to reduce the initial myeloma, you're treated with drugs to stimulate the production of your own blood stem cells. These stem cells live in small numbers in all of us, and they develop into healthy red cells, white cells, and platelets. A few days later, you undergo leukapheresis, a process in which your blood is routed from a vein through a machine that removes the stem cells and returns the blood to another vein. The process, sometimes referred to as stem cell harvest, may take several hours and may need to be repeated a few times. Usually, enough stem cells for two transplants are harvested and frozen. Then you undergo high-dose chemotherapy, which kills all of your blood cells — the cancerous myeloma cells and the healthy cells — as well as producing its well-known side effects: hair loss, nausea, diarrhea, and fatigue. Then the stored healthy stem cells are infused back into your blood. If the treatment works, the stem cells go on to form a healthy blood system, free of myeloma cells. During this time, you're anemic and vulnerable to infection, because you have low numbers of infection-fighting white blood cells. So while your blood cells are growing back — a process that usually takes about two weeks — you'll receive transfusions, growth factor, and antibiotics to help prevent anemia and infection.  Thanks to a host of effective new therapies, patients are enjoying years in remission from this malignant blood cancer.  If there's one disease that most shows the effects of recent improvements in cancer treatment, it's multiple myeloma. Advances in biotechnology have enabled scientists to identify several genetic and chromosomal abnormalities underlying the disease, while fostering the development of more effective and less toxic drugs. Although there's still no cure for myeloma, various treatments are bringing about lengthy remissions, and people who were once given two to three years to live are successfully managing their condition for five to seven years, with some living a decade or longer.  The word myeloma derives from the Greek roots myelos (bone marrow) and oma (tumor); it's "multiple" because the tumors occur at many places in the bone marrow. The cause of the disease is unknown. It originates in plasma cells — white blood cells in the marrow called B lymphocytes, which differentiate in response to the presence of viruses and bacteria (pathogens) in the body. Normally, plasma cells make a different antibody to fight each type of invading pathogen. Thus, whenever that pathogen enters the body, the plasma cell becomes activated, reproducing in large numbers and generating enough antibodies to rid the body of the germ in question.  Myeloma cells are abnormal plasma cells that multiply for no apparent reason, producing substances that suppress normal marrow function and crowding out the normal blood components in the marrow — oxygen-carrying red blood cells, infection-fighting white cells, and platelets, which aid clotting. Consequently, people with myeloma may have anemia, lowered resistance to disease, and abnormal bruising and bleeding. Instead of functioning antibodies, myeloma cells generate excessive amounts of an abnormal protein called M protein, which can damage the kidneys as well as suppress normal immune function.  As myeloma cells accumulate in the marrow, they excrete enzymes that increase the rate of bone breakdown (resorption) and slow the rate of bone formation. As a result, calcium stored in bones is released into the blood, where high calcium levels may cause weakness, confusion, and further kidney damage.  The disease can also metastasize beyond the marrow and bone — that is, it can spread from the bone marrow to other parts of the body, including the liver, spleen, and soft tissues. Myeloma can thus wreak havoc with the bones and kidneys, and in advanced cases, the liver, spleen, and lungs, as both M protein and plasma cells build up in these various organs.  There's little evidence so far that we can do anything to prevent myeloma. The most common risk factors are outside our control. They include the following:  Age. Myeloma is rare in people under age 35 and most common in people ages 65 and older.  Ethnicity. The disease is almost twice as common in African Americans as it is in Caucasians. It's generally less common in Asians.  Gender. Slightly over 55% of people with myeloma are male.  Genetics. Having a close relative with myeloma may increase risk, but familial myeloma is uncommon.  Monoclonal gammopathy of unknown significance (MGUS). This benign condition affecting plasma cells is often an incidental finding during a routine blood test. Its incidence increases with age. MGUS requires no treatment, but anyone with the condition should be carefully monitored over time, as up to about 25% of people who have it will ultimately develop myeloma.  Early multiple myeloma doesn't always produce symptoms, but your clinician's suspicions may be aroused by abnormal results on a routine blood test or an unexpected broken bone. More often, the disease is signaled by symptoms like fatigue, weakness, back pain, frequent urination, constipation, and susceptibility to infection. In either case, the following laboratory and imaging tests can rule out or confirm the presence of multiple myeloma and determine the stage of the disease:  Blood tests that identify the number of red cells, white cells, and platelets; the presence of M protein; and levels of antibodies, the proteins beta-2 microglobulin and albumin, calcium, and creatinine (an indicator of kidney damage).  Urine testing that measures levels of another abnormal protein, called Bence Jones protein.  X-rays to check for damaged or broken bones.  A bone marrow biopsy to detect myeloma cells.  Studies have shown that there are several different kinds of myeloma cells with different genetic and chromosomal abnormalities, causing different forms of the disease that respond to different treatments. Tests for these abnormalities are used to identify the 25% to 30% of people with myeloma who are at especially high risk and likely to need early aggressive treatment.  Myeloma that's discovered before symptoms occur — with levels of beta-2 microglobulin only slightly higher and albumin levels slightly lower than normal — is usually described as inactive or smoldering or stage I disease. Studies haven't shown that drug treatment at this stage extends survival time, so the standard practice is "watchful waiting" — monitoring patients closely with physical exams and lab tests to determine whether the disease is advancing.  Although not curative, stem-cell transplantation is a mainstay of myeloma treatment, especially for younger (ages 65 to 70), healthier patients. Stem cells have the capacity to regenerate healthy red cells, white cells, and platelets, so they can be used to help people withstand chemotherapy at doses high enough to kill myeloma cells — a strategy that also destroys most of the healthy blood components.  After therapy to reduce the initial myeloma, you're treated with drugs to stimulate the production of your own blood stem cells. These stem cells live in small numbers in all of us, and they develop into healthy red cells, white cells, and platelets. A few days later, you undergo leukapheresis, a process in which your blood is routed from a vein through a machine that removes the stem cells and returns the blood to another vein. The process, sometimes referred to as stem cell harvest, may take several hours and may need to be repeated a few times. Usually, enough stem cells for two transplants are harvested and frozen.  Then you undergo high-dose chemotherapy, which kills all of your blood cells — the cancerous myeloma cells and the healthy cells — as well as producing its well-known side effects: hair loss, nausea, diarrhea, and fatigue. Then the stored healthy stem cells are infused back into your blood. If the treatment works, the stem cells go on to form a healthy blood system, free of myeloma cells. During this time, you're anemic and vulnerable to infection, because you have low numbers of infection-fighting white blood cells. So while your blood cells are growing back — a process that usually takes about two weeks — you'll receive transfusions, growth factor, and antibiotics to help prevent anemia and infection.
The cause of the blood disease multiple myeloma is unknown, but advances in treatment, including new medications and stem-cell transplants, are giving patients longer periods of remission.