THE DIAGNOSIS IS MYELOMA. NOW WHAT?

What is myeloma? Myeloma is a relatively uncommon cancer of the plasma cells in the bone marrow-- the soft issue inside the bones where new blood cells are made. For reasons not completely understood, plasma cells grow out of control and eventually crowd out healthy cells, causing anemia and poor clotting. These abnormal cells (known as myelomas) collect in the bone marrow and slowly destroy the bone. They also trigger the release of bone-weakening chemicals that lead to bone pain and fractures. Over time, they group together and form tumors called plasmacytomas. Eventually, multiple soft spots or holes, called lytic lesions, form in the bones; thus the name, "multiple" myeloma. Some oncologists call it a plasma cancer. Others classify it as bone cancer. We refer to it as bone marrow cancer. The differentiations matter a great deal to scientists, exploring treatments and cures. Probably less to patients. The disease travels away from its first site in the blood. From that point on, the fight is against an elusive foe, apt to be discovered in other bones and in some organs. What causes this disease to develop? There are environmental factors that make the chance of getting myeloma statistically more likely. But the candid answer is that, though we have some interesting theories, no one knows how it chooses its victims or why.  An often harmless condition called monoclonal gammopathy of unclear significance always occurs first. About three percent of the time, MGUS, as it is universally known, becomes myeloma. Nobody knows the cause of MGUS or how to cure it, either. Nor does anybody know which MGUS patients will contract myeloma, or why the other 97 percent don't. Whatever ethnicity or age--or length of time with MGUS--death usually followed the onset of myeloma within two or three years, until about ten years ago. Then new drugs and stem cell transplants began to extend lives dramatically. Is myeloma related to any other cancer? Leukemia is the family name of several hundred cancers of the blood. Lymphoma is the name of one large group of them. Myeloma is thought to be a single disease, or a small family of diseases, also in the Leukemia family. Are there different types of myeloma? There is only one myeloma, but it takes different forms. And there is another disease that patients always get first. MGUS, or monoclonal gammopathy of unknown significance, is a condition which occurs when people have a low level of M protein -- meaning there are small quantities of abnormal plasma cells -- but the cells do not form tumors or cause other myeloma symptoms. All patients who have myeloma had MGUS first, but only about three percent of MGUS patients develop myeloma. It's a good idea to re-examine patients with MGUS every six months. Smoldering multiple myeloma (SMM) or asymptomatic myeloma occurs when patients have slightly higher levels of M protein and more plasma cells in the bone marrow than people with MGUS, but still not enough damage to the body to cause symptoms. This form of myeloma may worsen very slowly, enabling patients to live symptom-free for many years. Patients with SMM will go on to develop myeloma eventually and are monitored closely. SMM is usually not treated, but vaccinations against infection and bone-strengthening medicines, called bisphosphonates, may be given. Zometa is one of the most frequently used drugs in this circumstance. Dental surgeons and osteoporosis specialists are often consulted, due to potential side effects of these drugs. Symptomatic or active myeloma is characterized by the presence of M proteins in the blood or urine and an increased number of plasma cells in the bone marrow. These abnormal plasma--or myeloma cells--cause one or more forms of damage, summarized by the acronym, "CRAB": high levels of calcium in the blood (hypercalcemia), kidney (renal), problems, anemia (low red blood count), or bone damage (soft spots known as lytic lesions appear in the bones weakening them, causing pain or fractures). Another sign of active myeloma is growth of a tumor, (plasmacytoma) in the bone or soft tissue. In active myeloma, the manner in which the cancerous plasma cells make different antibodies, also known as immunoglobulins, varies. Some patients produce only partial, rather than complete immunoglobulins. This is known as light chain myeloma, or Bence Jones myeloma. Since these light chain proteins are smaller and do not show up in the blood, urine tests are used to diagnose and monitor the disease. When free light chain proteins are present in the urine, they can accumulate in the kidney, causing damage. In less than two percent of myeloma patients, the immunoglobulin does not show up in the blood or the urine. This is referred to as non-secretory myeloma and is harder to diagnose; however, a new blood test called 'serum free light chain test' can help detect small amounts of free light chain proteins in the blood. Patients with this type of myeloma have a lower risk of kidney damage. What are “stages” and how does my stage influence the prognosis? Staging is a measurement that describes the degree to which cancer has advanced from its point of origin. Albeit all individuals are different, this information can help select a treatment method. In myeloma, staging is a three-step process. Two systems are currently used to determine myeloma stages: the Durie-Salmon system and the International Staging System (ISS). Under Durie-Salmon, the stage of the disease is decided by four factors: • Levels of M protein, or monoclonal immunoglobulin, a marker found in blood and urine that indicates how many multiple myeloma plasma cells are present and producing that abnormal protein • Blood calcium level: high blood calcium levels indicate advanced bone damage • Number of bone lesions, or myelomas, seen on x-rays • Hemoglobin levels: low levels indicate that myeloma cells are crowding out normal marrow cells. Normal cells produce red blood cells. Low numbers of them lead to anemia and other problems. Each stage is sub classified as A or B, with A designating normal kidney function and B designating some kidney problems. If your oncologist uses Durie-Salmon, and if this explanation of it confuses you, try diagramming the D-S system on paper: Three stages, determined by four criteria, with each stage further classified "A" or "B", depending on how the kidneys are functioning. The International Staging System (ISS) is a newer, more commonly used system that divides disease progression into three stages, based only on the levels of two compounds found in the blood, beta-2 microglobulin and albumin. Stage I. The beta-2 microglobulin level is less than 3.5 mg/L and the albumin level is 3.5 g/dL or higher. The blood calcium levels and red blood cell counts are usually normal and common symptoms of myeloma may not be present. Stage II. This is an inexact nether land between stage I and III. Either the beta-2 microglobulin level is between 3.5 and 5.5, with any albumin level, or the albumin is below 3.5 while the beta-2 microglobulin is less than 3.5. In Stage II, a moderate number of myeloma cells are detected throughout the body. Stage III. The beta-2 microglobulin level is greater than 5.5. A large number of cancer cells have spread throughout the body; blood calcium levels are elevated; red blood cell levels are decreased; growth of bone tumors can be detected and high levels of M-protein can be detected in blood or urine. Most patients have stage III multiple myeloma at diagnosis. Why does myeloma need to be treated? The treatment process for myeloma is very complex. Some patients with early stage myeloma are not treated. At least three factors cause this decision: The disease does not appear to be progressing. Secondly, there is no effective way to eradicate the condition. And on the other side of the coin, attempts to do so are hard on the patient. Certain drugs and procedures have been shown to be effective at specific points in the progression of the disease. So, generally, as the disease progress to one of those points, the drug or procedure that is currently thought to be most successful is instituted. There are also times later in the fight when cessation of treatment has been shown to be the best strategy. The patient's body gets a chance to rest and recover at a time when the cancer's progress has slowed or stopped. What treatment options are available? There is no "standard" therapy and there are many options. The best treatment is dictated by the stage of the disease, age, overall health, medical history and tolerance to specific medications, procedures, or therapies. Treatment for the disease typically includes chemotherapy, with or without steroids, stem cell transplant, radiation therapy and surgery. Supportive therapies are often added. They include medicines to reduce the calcium in your blood, control pain, prevent bone loss, stimulate blood cell production, and to fight infection. Orthopedic surgical procedures are performed to repair bone damage and hemodialysis is used to filter the blood if kidneys are not working well. What are the goals of initial treatment? The goals of first treatment are to eliminate myeloma cells, control tumor growth and to alleviate the symptoms of myeloma, thereby preserving the patient's quality of life. Although there is not yet a definite cure for multiple myeloma, one or more treatments often can keep the disease under control, sometimes for years.