Multiple myeloma (MM) is a tumor that is characterized by the proliferation of abnormal plasma cells inside the bone marrow, which is the factory of blood cells and found inside the bones of the spine vertebral, the skull, the pelvis, the rib cage and the areas around the shoulders and hips. We all have plasma cells, a type of white blood cells in a proportion no greater than 5%, which are responsible for producing immunoglobulins, proteins capable of attacking germs before an infection. Under normal conditions, mature plasma cells manufacture a single type of immunoglobulin throughout their existence and do not reproduce.
In patients with myeloma, the plasma cells become abnormal and also proliferate in an uncontrolled manner, partially occupying the bone marrow.
MM is the second hematological disease in order of frequency after non-Hodgkin’s lymphomas and there are between 4 and 5 new cases per 100,000 inhabitants per year, which means that in Spain approximately 2,000 new cases are diagnosed per year.
The profile of the typical patient with multiple myeloma, as indicated by Dr. María Casanova, hematologis of HC Marbella “is a person over 70 years of age who usually goes to the doctor due to the appearance of bone pain, secondary to the skeletal involvement characteristic of this disease. or by increased fatigue as a result of anemia associated with myeloma. Only 5-10% of patients are under 40 years old “.
There is a certain familial predisposition for multiple myeloma: between 5% and 7% approximately of the diagnoses of myeloma occur in a family member who has a close relative who has been previously diagnosed with some disease of the plasma cells, although it is not always a myeloma.
As we mentioned earlier, normal plasma cells produce immunoglobulins, the proteins that defend us from external attacks. Myeloma cells do not produce normal immunoglobulins, so there is an alteration in the immune system that results in an increased tendency to develop infections.
Clinically active MM interferes with the normal functioning of the bone marrow causing anemia (lowering of red blood cells) and an increased risk of infections, since it interferes with the normal functioning of the immune system. It also interferes with the mechanisms of bone renewal causing its decalcification, which usually causes intense bone pain, increases the risk of spontaneous fractures and is associated with an increase in blood calcium (hypercalcemia) that can also cause serious alterations.
In the same way as normal plasma cells, MM cells produce an immunoglobulin, which we call the monoclonal band or monoclonal component. This immunoglobulin can accumulate in the kidney causing renal failure.
When multiple myeloma causes any of these alterations: anemia, hypercalcemia, bone lesions or renal failure we say that it is injuring its “target organs” and it is necessary to start treatment as soon as possible. On the other hand, when myeloma is asymptomatic, it does not require immediate treatment (since myeloma can remain asymptomatic for years or for a lifetime).
Anyway, there are certain markers (determinable in blood tests, in bone marrow studies or by imaging studies) that predict the onset of symptoms soon and therefore are also considered criteria for initiating treatment in advance.
MM is still considered an incurable disease. Most patients will relapse after initial treatment of the disease and will need to be treated again, probably several times. That does not mean that the forecast is very bad, in fact it is extremely variable. A small proportion of patients (up to 10%) will have an excellent response to first line treatment and may never relapse, we call these patients “functionally cured” since we never have the assurance that the MM will not reappear, although they have spent 10 or 15 years free of disease.
At the other extreme there is 10-15% of patients who may be resistant to initial treatment or respond initially but relapse very soon. The vital prognosis in these cases is very bad, around two years. Between these two extremes, the majority of patients will respond adequately to the initial treatment, their symptoms will disappear or improve a lot after the first weeks of treatment and they will not have a vital risk in the short or medium term. Even so, the long-term prognosis is more uncertain, since it will depend on the frequency and aggressiveness of the relapses.
There are several useful drugs for the treatment of MM but it is important to individualize the treatment well, depending on the age and characteristics of the patient and the disease.
The standard treatment options include the following:
Targeted therapy. Treatment with targeted drugs focuses on the specific abnormalities present within the cancer cells that allow them to survive. These drugs block the action of a substance in myeloma cells that breaks down proteins. This action causes the myeloma cells to die.
Other targeted therapy treatments include drugs with monoclonal antibodies that adhere to specific proteins present in the myeloma cells and cause their death.
Immunotherapy or biological therapy. Biologic therapy medications use the body’s immune system to fight myeloma cells. These medications improve the cells of the immune system that identify and attack cancer cells.
Chemotherapy. Chemotherapy drugs kill fast-growing cells, including myeloma cells. They are used at high doses before a bone marrow transplant.
Corticosteroids. Corticosteroids regulate the immune system to control inflammation in the body. They are also active against myeloma cells.
Bone marrow transplant. There are two types of bone marrow transplant and it is important to know them, the autologous transplant and the allogeneic transplant.
– In the autologous transplant, the patient’s own stem cells are used, that is, the cells of the patient are extracted or collected and frozen after receiving several cycles of induction of treatment and after confirming that the patient is in a good situation. to his hematologic disease. The patient subsequently receives intensive chemotherapy treatment. The chemotherapy treatment is administered to eliminate residual tumor cells but also eliminates the healthy medullary cells and while the bone marrow recovers from this intensification treatment the collected stem cells are administered to avoid complications due to the descent of all the medullary cells. This is the type of transplant most used in patients with multiple mieloma.
– In the allogeneic transplant: the stem cells come from a donor whose tissue resembles that of the donor. The donor can be family (brother / sister) or non-family member. It is a procedure in which the diseased bone marrow is replaced by healthy bone marrow In the autologous transplant, the patient’s own stem cells are used, that is, the cells of the patient are extracted or collected and frozen after receiving several cycles of induction of treatment and after confirming that the patient is in a good situation. to his hematologic disease. The patient subsequently receives intensive chemotherapy treatment. The chemotherapy treatment is administered to eliminate residual tumor cells but also eliminates the healthy medullary cells and while the bone marrow recovers from this intensification treatment the collected stem cells are administered to avoid complications due to the descent of all the medullary cells. This is the type of transplant most used in patients with multiple mieloma.
In the allogeneic transplant the stem cells come from a donor whose tissue resembles that of the donor. The donor can be family (brother / sister) or non-family member. It is a procedure in which the diseased bone marrow is replaced by healthy bone marrow
Radiotherapy. In this treatment, energy beams, such as X-rays and protons, are used to damage the myeloma cells and stop their growth. Radiation therapy can be used to rapidly reduce myeloma cells in a specific area, for example, when an accumulation of abnormal plasma cells forms a tumor (plasmacytoma) that causes pain or destroys a bone.
Sources:
Dr. Casanova Espinosa
Haematology Specialist.
AECC (Asociación Española Contra el Cáncer) / Cancerdelasangre.com / Fundación Josep Carreras / National Cancer Institute / American Cancer Society.
September 11, 2018
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