Frequently asked questions (FAQ)
What is multiple myeloma?
Multiple myeloma is a mclassant blood disease that affects plasma cells. These cells are a specialized form of blood cells that produce immunoglobulin (protein) to help fight infection.
Multiple myeloma develops when these plasma cells grow out of control. The pathological proliferation of plasma cells infiltrates in the bone marrow and in some cases in other organs. Population of myeloma cells can affect the bone tissue causing pain and in some cases spontaneous fracture called pathologic fracture.
Cancerous plasma cells crowd out normal plasma cells and thereby impair their functions. As the number of myeloma cells increases, fewer red blood cells, white blood cells, and platelets (playing an important role in blood clotting) are made. Additionally, as the number of the normal plasma cells helping the organism to fight infections is decreased, the resistence to infections is lowered. The immune deficiency in people with multiple myeloma increases their risk of infections.
Who can develop the multiple myeloma disease?
Multiple myeloma is often found in elderly people, most often in people older than 60 years of age. Rarely, the disease strikes people younger than 40 years (less than 2 % of all myeloma patients). Multiple myeloma occurs more frequently in man than in woman. It represents approximately 1% of all cancers and, in mclassant blood diseases, it represents 10% of all cases. The incidence of myeloma is 2 - 4 case per 100 000 inhabitants.
Can be multiple myeloma hereditary?
Myeloma is not hereditary and not a clear outcome of an increased incidence in close relatives of a myeloma patient was proven. There are, however, very rare families with several cases of multiple myeloma. The significance of this rare phenomenon remains unclear.
What are the causes and risk factors for multiple myeloma?
The exact cause of multiple myeloma is not known. The research have been evaluating the combination of different influences, factors that are still not precisely defined. Generally, individuals who develop multiple myeloma have no clear risk factors. Myeloma may be the result of several factors acting together. These factors are called risk factors.
As most frequent risk factors are mentioned: some of genetic factors, exposure to radiation and lifelong accumulation of some of the chemical insults.
What are the symptoms of multiple myeloma?
Myeloma symptoms depend on the stage of the disease.
The main symptoms include bone pain, anemia and symptoms related to renal failure. Bone pain is due to the dissolution of bone tissue by myeloma cells resulting in lytic lesions. In some cases, a spontaneous fracture may therby occur. Anemia (low level of red blood cells) is associated with impaired functions of normal blood stem cells. Insufficient production of red cells causes fatigue and shortness of breath, especially when practicing sports. In some patients, the pathologic protein called light chain protein is present in urine. It may provoke kidney damage; if the damage is severe renal failure may develop. The clinic signs of renal failure include loss of appetite, malaise and decreased urine output. Some patients suffer from frequent infections due to low levels of immunoglobulin.
However, these mentioned symptoms are not necessarily proof of multiple myeloma. The symptoms could be an indication of another illness and that is why an immediate medical care is needed.
Can be multiple myeloma diagnosed at an early stage of the disease?
Multiple myeloma, diagnosed at an early stage, is less dramatic because the disease can be more effectively treated. Nonetheless, there is no a standard plan of MM detection and people suspecting some risk of developing the disease should discuss the problem they are worrying about with a doctor.
How is multiple myeloma diagnosed?
A physical exam and complete medical history are usually the first steps in diagnosing multiple myeloma, the doctors ask about the family medical history, medication history and exposure to risk factors, for example radiations. An overall physical examination assesses the other possible signs of the illness.
Other tests that are commonly used to diagnose multiple myeloma include:
- Skeleton X-rays - x-ray survey of the overall skeleton is performed in symptomatic and asymptomatic patient to detect the extent of the disease. This test shows the areas that are affected and may develop a pathologic fracture.
- Paraprotein test - In multiple myeloma patients, plasma cells produce excessive amount of antibodies called immunoglobulin or paraprotein. The levels of paraprotein help the doctor identify the stage of disease. The protein may be present in urine as well, therefore, the quantitation of the paraprotein in serum and in urine is another important test in multiple myeloma.
- Bone marrow aspiration and biopsy - are ways detecting cancerous cells in the bone marrow. In this test, a sample of bone marrow is removed from iliac or thoracic area with a long, thin needle and studied for the presence of abnormal plasma cells.
- Lytic lesion biopsy - if the detection of myeloma disease is not possible by the puncture biopsy method and a lytic lesion is suspected to result from cancerous stem cell activity, it is advisable to perform a histology of an aspirate taken from the lytic lesion by an orthopaedist. Histology is the most sensitive single test for setting the diagnosis.
What are the examinations undergone by myeloma patients and what kind of preparation do they require?
Basic testing (performed in all patients) - x-ray survey, densitometry
Complement testing (not necessarily performed) - CT (Computer Tomography), NMR (Nuclear Magnetic Resonance)
New applications - MIBI (radionuclide scintigraphy), PET (Positron Emission Tomography)
||Lytic lesion, osteoporosis and pathologic fracture detection
||Assessment of the density of bone tissues and osteoporosis detection Follow-up of bisphosponate treatment
||Non-standard forms of MM, spinal cord or nerve roots compression by metastatic cancer are suspected
||Spinal cord or nerve roots compression suspected; bone pain and unclear finding on x-ray survey
||Detection and follow-up of myeloma lytic lesions, not revealed on x-ray films
||Detection of myeloma lytic lesions not revealed on other imaging tests, detection of another tumour
|When is it performed
||Entrance testing, before transplantation, check-up once per year or as needed
||Entrance testing, check-up once a year
||Within 2 days
||It is still not clearly defined
||6 h before exam. empty stomach, 1 h before exam Dithiaden 1 tbl., before CT administration of contrast agent
||During exam, 50 g chocolate should be consumed
||no exercising 48 h before exam., no sugar, rice, potatoes, pasta, cheese 24 h before exam. only water 6 h before exam.
|| 45 min
|| 15 min
|| 45 min
||Within 2 weeks, in acute cases immediate
||Within 1 month, in acute cases immediate
||Within 1 day
||Within1 - 3 days, in acute cases immediate
||Within 1-3 days, in acute cases immediate
||Within 1 week
||Within 2 days
||Comparison with previous films is important
||Comparison with previous measures is important
||In iodine allergy, it is recommended to be performed in hospital
||Metallic implanted device except titanium is contraindication, pacemaker
||Comparison with previous findings is important
||Comparison with previous films is important
Has the patient right to a medical assessment by another doctor?
Many patients with cancer demand a diagnostic assessment by another doctor in such a situation. There are many reasons for providing it, especially if the patient is not satisfied with medical decisions, is the tumour is classified as a rare disease and if there are different treatment options.
How to get another diagnostic assessment?
There are many ways how to get another assessment.
Every general practitioner should be able to refer to a relevant specialist, such as to a surgeon, clinical oncologist, radiologist-oncologist. These specialists may work in a team or in the scope of oncology centres or programs.
The patient can get contacts to specialists in local health care institution, neighbouring hospital, medicine schools or from other people who are affected with the same type of tumour.
- Doctor directory is classified according to the positions and the specialisations and provide information about the doctor´s background. Further information is available in most libraries, on hospital and healthcare websites similar to these internet pages.
Are all patients with multiple myeloma treated identically?
Deciding which therapy to pursue depends upon patient´s overall health and the stage of the disease.
Some of the patients are diagnosed accidentally at the time they have no symptoms. These patients are called asymptomatic patients. Instituting a therapy appears to be justified only when progression of the disease is evident in laboratory, x-ray or other tests. The clinical trials has not shown therapy benefit for asymptomatic patients.
Examinations of paraprotein levels with a median follow-up of 2-6 months are required in asymptomatic patients. Additionally, the progression of the disease has to be monitored with regular x-ray survey and bone marrow biopsies. This medical approach is described as watch-and-wait. As soon as the radiography displays bone affection, the therapy should be instituted immediately.
The treatment of multiple myeloma consists of several modalities often referred to as "complex" therapy. This means the combination of cytostatics (antitumour affects), radiotherapy (radiation therapy), intensive support care (bisphosponates - preserving bone mass; analgesics) and orthopaedic treatment (conservative and surgery). The treatment is predominantly ambulatory, unless the type of therapy demands the hospitalization or complications emerge.
1. Anti-cancer treatment (chemotherapy) - targeted at myeloma cells
2. Supportive care - manages the symptoms but it doesn´t have an effect on myeloma cells.
The goal of anti-cancer treatment is to eliminate or minimize all clinically or laboratory detectable symptoms.
The anti-cancer treatment involves a standard chemotherapy or high-dose chemotherapy with stem cell transplant. The treatment option depends on the patient´s age and overall health condition. Drugs are delivered through intravenous infusion or orally as a pill. Cytostatics may form different combination chemotherapy regimens repeated evenly (mostly with 1 month interval). The overall duration of induction therapy depends on patient´s response. Predominantly, it is administered in 6-8 cycles (6-8 months). During remission phase, the therapy using cytostatics may be replaced by maintenance therapy. The maintenance therapy consists mostly of different immunomodulatory agents or its combinations. When relapse of the disease occurs, a cytotoxic chemotherapy is instituted.
High-dose chemotherapy with stem cell transplant procedure:
- Induction chemotherapy (4 cycles) - the goal is to minimize the number of myeloma cells or to damage or kill cancer cells to the point that the progress of the disease is slowed.
- Stimulatory treatment with the harvest of the stem cell - the goal is to collect stem cells (following a chemotherapy and growth factors administration) by means of an intravenous cathether to obtain a sufficient amount of stem cells for the transplantation that will have a subsequent use in lowering the number of myeloma cells.
- High-dose chemotherapy with autologous stem cell transplant - with this type of transplant, the patient's stem cells are obtained prior to high-dose chemotherapy, during the harvest. The purpose is to assure the blood formation with a minimized number of myeloma cells. The transplant is performed by means of intravenous injection or infusion.
- Maintenance therapy - the goal is to prolong the phase without symptoms of the disease.
This procedure is the most commonly used pattern. It can be, nevertheless, modified according to the response to the treatment and to the actual condition of the patient.
High-dose therapy in conjunction with allogenic stem cell transplant is rarely performed for treatment of myeloma. The stem cells used for the transplant are from a donor whose blood cells closely match the patient´s one. Nevertheless, this type of transplant remains very risky.
Radiotherapy (radiation treatment) is an integral part of myeloma treatment process. Radiotherapy kills off the myeloma cells in the bone and shrinks the cancer. This helps to reduce bone pain. The treatment duration is usually 5 days a week, during one or more weeks.
The goal of supportive care is to improve comfort and quality of life of the patient. Supportive care has no effect on myeloma cells but helps to minimize troublesome symptoms provoked by anti-cancer treatment. These medical approaches may be resumed in two procedures:
Long-term specialized bone treatment by means of bisphosponates. These drugs bind to a bone areas destroyed by myeloma cells, slow down the dissolution of bone mass and reduce pain. After an acute phase of the disease, administrating of vitamin D and calcium together with bisphosponates is recommended to boost its effect.
- Analgesics are administrated to myeloma patients in order to reduce or eliminate pain. The dosage should be set to manage the pain during whole day preventing it to emerge. Analgetics should be reduced or stopped as soon as the pain decreases or disappears.
- Orthopaedic treatment in multiple myeloma involves braces, supporting skeleton and helping to relieve pain, or orthopaedic surgery, if necessary.
- Infection treatment. In case of infection (demonstrated for instance by high body temperature), the patient should always consult a doctor who may prescribe antibiotics. Severe forms of infections may be treated by drugs stimulating the immune system.
- Kidney damage resulting from multiple myeloma may require dialysis. Commonly, this complication is reversible.
- Plasmapheresis. If the level of abnoramal immunoglobulin is too high in the blood, the doctor may suggest a treatment called plasmapheresis. Too much protein in the blood can make it too thick, which may cause damage to certain organs. Plasmapheresis will take out the excess of protein thanks to a machine called separator.
- Anemia treatment. Chemotherapy treatment is often associated with reduced production of red cells. If the anemia is severe (red cell count is really low), the patient may be given drugs stimulating their production.
- Physiotherapy should be undertaken in every treatment strategy if the patient´s condition allows exercising. It helps muscle and bone mass to remain strong and make a general benefit for organism. It must be always performed with care to respect the damaged area of the skeleton.
What is "medical response"?
Medical response is an evaluated (measurable) outcome of the treatment. It is evaluated in regular intervals with common examinations (usually the same as in diagnosing - serum and urine analysis to detect the level of paraprotein, bone marrow analysis, x-ray survey) that permit to check up the symptoms and compare them with the previous results. According to the overall outcome, the medical response may be classified in several categories with precisely defined criteria.
Complete response - CR requires that the patient complies with all three criteria
- Absence of monoclonal immunoglobulin (paraprotein) in serum and in urine. This absence must be confirmed by two methods, protein electrophoresis and immunofixation electrophoresis. This outcome evaluated in serum and urine by immunofixation must be maintained for a minimum of 6 weeks.
- If we want to confirm the complete remission, we have to perform sternal pucture. The result of the examination mustn´t exceed the 5% level and the same criterion is valid for bone marrow biopsy. If monoclonal immunoglobulin is absent for more than 6 weeks, it is not necessary to verify the bone marrow finding by a repeated puncture.
- In patient with non-secretory myeloma (without paraprotein in serum and urine), it is necessary to confirm the above-defined reduction of plasmocytomas by means of another sternal puncture carried out 6 weeks later.
- Bone lytic lesions must not grow as well as their number must not increase. This criterion is confirmed by x-ray survey. Nonetheless, a compression fracture resulting from a previous lytic lesion doesn´t exclude a complete remission.
- Plasmocytomas of soft tissues of the body must disappear.
In patients that comply only with some of the critaria of the complete remission and that meet the bellow-defined conditions, the response is said to be partial.
Partial response - PR requires that the patient complies with all three criteria
- Reduction of monoclonal immunoglobulin level in blood by at least 50 % for a minimum period of 6 weeks.
- Reduction of light-chain protein (a form of paraprotein) concentration in a 24-hours urine test by a minimum of 90%.
- In patients with non-secretory myeloma, the decrease of stem cell concentration in a cytology or histology evaluation of bone marrow sample by a minimum of 50% for a minimum of 6 weeks is required.
- Reduction of plasmocytoma dimensions of soft tissues by a minimum of 50% in clinical or x-ray examination.
- During the examination period, the number of lytic lesions or their extent mustn´t increase, which is checked up on x-ray. Nonetheless, a compression fracture resulting from a previous lyic lesion doesn´t exclude a partial remission..
In patients that comply only with some of the critaria of partial remission and that meet the bellow-defined conditions, the response is said to be minimal.
Minimal response - MR requires that the patient complies with all three criteria
- Reduction of monoclonal immunoglobulin concentration in serum by a minimum of 25 to 49 % for a minimum of 6 weeks.
- Reduction of monoclonal immunoglobulin (light-chain protein) concentration in a 24-hours urine test by a minimum of 50-89 % with the concentration still exceeding 200 mg/24 h for a minimum of 6 weeks.
- In patient with non-secretory myeloma, the abnormal plasma cell concentration in bone marrow must be reduced by 25 - 49% in a cytology or histology evaluation of bone marrow sample for a minimum of 6 weeks. To evaluate this, a repeated sternal puncture is necessary.
- Reduction of plasmocytoma dimensions of soft tissues by a minimum of 25-49% in clinical or x-ray examination.
- The number and extent of lytic lesions mustn´t grow, which is checked up on x-ray.
Patients that have complied with some of the criteria of partial response and meet the criteria of minimal response are classified as patients with minimal medical response.
No change - NC: the disease complies with none of the defined criteria but doesn´t progress.
Plateau: the term plateau is used for the phase of the disease where the concentrations of immunoglobulin remain stable or vary not more than by 25% for a minimum of 3 months.
Medical response to transplantation
- Medical response to transplantation is evaluated by comparison of the patient´s condition before and after transplantation
- If the transplantation is a part of a larger treatment plan, we compare the values before the treatment plan and after its ending.
Relapse (occurs when a person is affected again by symptoms of a disease that affected them in the past)
Relapse after complete response requires that the patient complies with at least one criterion
- Reappearance of monoclonal immunoglobulin in urine or in serum and its detection by immunofixation method or electrophoresis that is repeated at least once for confirmation.
- The number of plasma cell in bone marrow is equal or superior to 5 % in cytology evaluation of the aspirate or in histology evaluation of trepanobiopsy.
- New lytic lesions develop or the existing lytic lesion grow. A compression fracture doesn´t necessarily reveal progression of the disease.
- Hypercalcemia (calcium concentration in serum exceeding 2,8 mmol/l) is associated with relapse of the disease, unless there are other causes different from myeloma.
Progressive disease: this term is used to determine the condition where the complete response was not achieved. At least one criterion is than met:
- Monoclonal immunoglobulin concentration in serum increased by a minimum of 25 %, simultaneously, its absolute value increased by 5 g/l. The finding must be confirmed by a repeated examination.
- The monoclonal immunoglobulin concentration at the time of medical response is considered to be the value of reference.
- Concentration of light-chains in urine increased by a minimum of 25% per 24h, simultaneously, its absolute value increased by 200mg per 24h. Excretion at the time of diagnosis is considered to be the value of reference. The increase of excretion must be confirmed at least by one subsequent examination.
- The plasma cell count of the sample taken from bone marrow increased by 25 % which means it increased at least by 10% in absolute value. The sternal puncture is carried out only when there is a clinical indication.
- Significant growth of lytic lesions or of soft tissue plasmocytoma.
- Appearance of new lytic lesions or other lesions in soft tissues.
- Patient developed hypercalcemia exceeding 2,8 mmol/l.
What are "clinical trials"?
Clinical trials are research studies involving new anti-cancer drugs. Physicians investigate clinical outcomes to assess the efficacy of a new treatment and its side effects. If a clinical trial results appear promising, they are compared with the most widely used treatment to state whether the new treatment is more efficient or has less side effects. Patients that participate in clinical trials may benefit from state-of-the-art treatment methods before they are authorized by the relevant national institutions. Furthermore, participation in clinical trial can help understand mclassant diseases and, thereby, help yourself and future patients.
What is new in myeloma?
Oncology research gives people hope. Physicians and researchers worldwide are now given further information about the causes of multiple myeloma and they investigate the ways how to prevent this disease. At the same time, the trials evaluating detection alternatives are carried out.
Ongoing trials are testing high-dose chemotherapy with stem cell transplant targeted at gain in life expectancy. This treatment option is considered to be the most effective but the most demanding.
The growing interest in so-called immunomodulatory drugs having the ability to influence immune response in organism and inhibit angiogenesis (an important process for cancer cell growth) has now opened the field for research. These drugs are a form of biological treatment and their mechanism of action is different from classical chemotherapy. Its precise indication in the treatment of multiple myeloma is being evaluated. This class of novel drugs involves thalidomide, bortezomib (Velcade), Revlimid and Actimid.
Are there any support groups for patients with multiple myeloma?
Information about newly formed Patient support group are available on www.mnohocetnymyelom.cz or you can contact us on these e-mail addresses: firstname.lastname@example.org, email@example.com, firstname.lastname@example.org.