This is plasmacytoma. Myeloma Diagnosis

Plasmacytoma is a relatively rare but dangerous tumor that can spread throughout the body from one bone. It refers to hematopoietic neoplasms that originate from bone marrow cells and lymph nodes.

Plasmacytoma is formed from plasma cells - cells of the red bone marrow. Plasma cells produce immunoglobulins, that is, antibodies, and are the precursors of lymphocytes. These cells perform a very important function - they protect the body from pathological microbes (viruses, bacteria, etc.). With the development of the oncoprocess, a failure occurs in this complex system, and atypical plasmocytes appear, which reproduce their own kind and form a conglomerate.

Plasmacytoma of bone tissue: symptoms and manifestations

Of course, there is always a risk of developing multiple myeloma. When this will happen, no one knows. Some live for decades. For others, the progression of the disease occurs over a couple of years or even months. After confirmation of multiple myeloma, most patients die within the first 3 years.

Cancer prevention

In recent decades, there has been a jump in tumors of the hematopoietic system. Therefore, scientists are doing their best to find more effective methods of dealing with this pathology, as well as ways to prevent it. So far, there is no effective prevention of plasmacytoma. The most basic thing that everyone can do is to find out her symptoms, and if they are found in themselves, to undergo an examination. Early detection of the disease significantly increases the chances of recovery.

Informative video

Among plasma cell tumors, plasmacytoma is the most common, the disease is also known as myeloma, multiple myeloma. Tumor development is related to the accumulation and proliferation of monoclonal differentiated B cells. Worldwide, plasmacytoma affects approximately four people per hundred thousand of the population. Moreover, in women the disease is diagnosed more often. Basically, cancer of this type is found in patients older than forty, the average age of patients is about seventy years. So far, scientists have not figured out what exactly is the cause that causes plasmacytoma.

This cancer is one of the tumors with low proliferative activity of malignant cells. Presumably, the precursors of cancer cells in this case originate from the lymph nodes, they enter the bone marrow by migration through the blood. In plasmacytoma, tumor proliferation is a consequence of clonal expansion of B cells. Unlike normal cells, these cells have an invariable type of hypermutation. Therefore, they avoided natural selection in the germinal center; programmed cell death did not occur.

It is known that the pathogenesis of plasmacytoma depends on the interaction of neoplastic cells with the stromal microenvironment in the brain, which is of great importance during the process of neoangiogenesis and tumor adhesion.

Symptoms of the disease

Most often with plasmacytoma, there are manifestations characteristic of this disease. So, patients note pain in the bones, and this is especially true of the femurs, pelvis, spine, and humerus. Pathological fractures occur, for example, a compression fracture of the spine may occur, which sometimes causes a decrease in the patient's height. In addition, there are signs of kidney damage, hypercalcemia, normochromic anemia, bacterial infections. Mostly Gram-positive, usually pneumococcal infection. Hemorrhagic manifestations, amyloidosis, hyperviscosity syndrome may occur.

In plasmacytoma, bone pain is explained by increased bone resorption, as osteoclast activation and infiltration by myeloma cells occur. Systemic osteoporosis occurs, its osteolytic foci do not have new marginal bone formation, typical of metastases. Basically, the development of destructive processes was noted in the spine and flat bones, they can be found in the proximal section of the tubular long bones. The lumbar and thoracic sections of the spine are subjected to compression, there may be deformities of the "fish mouth" type.

Spinal cord compression may develop due to penetration of the tumor mass through the intervertebral foramens, or germination occurs directly from the vertebra affected by cancer. With compression, the clinical manifestations are sensory and motor disturbances in the functioning of the intestines and bladder, as well as radicular pain, which becomes more pronounced when sneezing or coughing.

Features of the course of the disease

It happens that with a plasmacytoma, the occurrence of a bacterial infection indicates that the patient has an immunodeficiency. Its basis is a syndrome of insufficiency of opsonization of bacteria and production of antibodies, which is compared with a reduced amount of normal immunoglobulins in the blood. More often than other symptoms, the occurrence of infections of the genitourinary system caused by multifactorial kidney damage was noted. Pneumococcal and other infections are also found, which are caused mainly by bacteria of encapsulated forms. Infectious complications among the causes of death from plasmacytoma are one of the leading places.

With this disease, hemorrhagic phenomena occur, they are explained by the emerging deficiency of the coagulation factor. Hyperviscosity syndrome, which occurs due to an increase in plasma viscosity, is accompanied by a number of neurological disorders, signs of hypervolemia, and ophthalmic symptoms also appear. When diagnosing a plasmacytoma, the main criteria include the detection of multiple myeloma through a biopsy. To determine paraprotein in urine and blood, the electrophoresis method is used, but the immunofixation method has the maximum accuracy, which allows detecting the minimum doses of the paraprotein. This method is of particular value when it is necessary to track the results of therapy, and gives a full response to the treatment.

If the doctor has diagnosed a sluggish plasmacytoma, then immediate therapy is not required in this case. The indication for treatment is the symptomatology of the disease associated with an increased content of paraprotein, as well as the progression of osteolytic lesions. These are bone fractures, spinal cord compression, bone pain, spinal compression, impaired kidney function, hypercalcemia, severe anemic syndrome, and the occurrence of infectious complications. Spinal cord compression requires surgery if possible. For example, kyphoplasty or laminectomy is performed, supplementing these actions with dexamethasone therapy. Local irradiation can be prescribed, and in case of pathological bone fractures, orthopedic fixation is performed.

If multiple plasmacytoma is diagnosed, radiation therapy is used as a palliative treatment of bone local lesions, especially if there is a pronounced pain syndrome. A special indication for radiation treatment is damage to the bones of the facial skull, as well as its base. If high-dose therapy is not planned for a patient suffering from multiple myeloma, then most often the patient receives cytostatic treatment, in which cyclo-nonspecific agents are used that are combined with corticosteroid hormones.

It is important to take into account that acute secondary myeloid leukemia sometimes develops as a long-term effect of the side effect of alkylating drugs, toxic effects accumulate on the bone marrow, and stem cell production is disrupted. In the future, such complications may interfere with autologous transplantation.

If myeloma blood disease progresses, plasma cells are destroyed in the bone marrow, which mutate and become malignant. The disease belongs to paraproteinemic leukemias, has the second name "Cancer of the blood". By nature, it is characterized by a tumor neoplasm, the size of which increases at each stage of the disease. The diagnosis is difficult to treat and can result in unexpected death.

What is multiple myeloma

it an abnormal condition called "Rustitzky-Kahler disease" shortens life expectancy. In the pathological process, cancer cells enter the systemic circulation, contribute to the intensive production of pathological immunoglobulin - paraproteins. These specific proteins, being converted into amyloids, are deposited in tissues and disrupt the functioning of such important organs and structures as the kidneys, joints, and heart. The general condition of the patient depends on the degree of the disease, the number of malignant cells. Diagnosis requires differential diagnosis.

Solitary plasmacytoma

Plasma cell carcinoma of this variety is distinguished by one focus of pathology, which is localized in the bone marrow and lymph node. To make the correct diagnosis of multiple myeloma, a number of laboratory tests are required to exclude the spread of multiple foci. With myeloma bone lesions, the symptoms are similar, the treatment depends on the stage of the pathological process.

With this pathology, several structures of the bone marrow become foci of pathology at once, which are rapidly progressing. The symptoms of multiple myeloma depend on the stage of the lesion, and for clarity, you can see thematic photos below. Blood myeloma affects the tissues of the vertebrae, shoulder blades, ribs, wings of the ilium, bones of the skull, related to the bone marrow. With such malignant tumors, the clinical outcome for the patient is not optimistic.

stages

Progressive Bence-Jones myeloma at all stages of the disease is a significant threat to the patient's life, so timely diagnosis is 50% of successful treatment. Doctors distinguish 3 stages of myeloma pathology, in which the pronounced symptoms of the disease only increase and intensify:

First stage. An excess of calcium prevails in the blood, an insignificant concentration of paraproteins and protein in the urine, the hemoglobin index reaches 100 g/l, there are signs of osteoporosis. The focus of pathology is one, but it is progressing.
Second stage. The lesions become multiple, the concentration of paraproteins and hemoglobin decreases, the mass of cancerous tissues reaches 800 g. Single metastases predominate.
Third stage. Osteoporosis progresses in the bones, there are 3 or more foci in the bone structures, the concentration of protein in the urine and calcium in the blood is increased to a maximum. Hemoglobin pathologically decreases to 85 g / l.

The reasons

Bone myeloma progresses spontaneously, and doctors have not been able to determine the etiology of the pathological process to the end. One thing is known - the risk group includes people after radiation exposure. Statistics report that the number of patients after exposure to such a pathogenic factor has increased significantly. According to the results of long-term therapy, it is far from always possible to stabilize the general condition of a clinical patient.

Multiple myeloma symptoms

With lesions of bone tissue, the patient first develops anemia of unknown etiology, which is not subject to correction even after a therapeutic diet. The characteristic symptoms are pronounced pain in the bones, the occurrence of a pathological fracture is not excluded. Other changes in general well-being with the progression of multiple myeloma are presented below:

frequent bleeding;
impaired blood clotting, thrombosis;
decreased immunity;
myocardial pain;
increased protein in the urine;
temperature instability;
kidney failure syndrome;
increased fatigue;
severe symptoms of osteoporosis;
spinal fractures in complicated clinical presentations.

Diagnostics

Because the At first, the disease proceeds in an asymptomatic form and is not diagnosed in a timely manner., doctors are already detecting complications of multiple myeloma, suspicions of kidney failure. Diagnosis includes not only a visual examination of the patient and palpation of soft, bony structures, it is additionally required to undergo clinical examinations. It:

X-ray of the chest and skeleton to determine the number of tumors in the bone;
bone marrow aspiration biopsy to check for the presence of cancer cells in myeloma pathology;
trepanobiopsy - a study of a compact and spongy substance taken from the bone marrow;
myelogram is necessary for differential diagnosis, as an informative invasive method;
cytogenetic study of plasma cells.

Multiple myeloma treatment

In case of uncomplicated clinical pictures, the following surgical methods are used: transplantation of donor or own stem cells, high-dose chemotherapy using cytostatics, radiation therapy. Hemosorption and plasmaphoresis are appropriate for hyperviscosity syndrome, extensive kidney damage, and renal failure. Drug therapy lasting several months for myeloma pathology includes:

painkillers to eliminate pain in the area of the bones;
antibiotics of the penicillin series for recurrent infectious processes inside and intravenously;
hemostatics to combat heavy bleeding: Vikasol, Etamzilat;
cytostatics to reduce tumor masses: Melphalan, Cyclophosphamide, Chlorbutin;
glucocorticoids in combination with heavy drinking to reduce the concentration of calcium in the blood: Alkeran, Prednisolone, Dexamethasone.
immunostimulants containing interferon, if the disease was accompanied by a decrease in immunity.

If, as a malignant tumor grows, there is increased pressure on neighboring organs with their subsequent dysfunction, doctors decide to urgently eliminate such a pathogenic neoplasm by surgical methods. The clinical outcome and potential complications after the operation can be the most unpredictable.

Nutrition for myeloma

To reduce the recurrence of the disease, the treatment must be timely, while the diet must be included in the complex scheme. Adhere to such a diet is required throughout life, especially with the next exacerbation. Here are valuable dietary recommendations for plasmacytosis:

reduce protein intake to a minimum - no more than 60 g of protein per day is allowed;
exclude from the daily diet foods such as beans, lentils, peas, meat, fish, nuts, eggs;
do not eat foods to which the patient may develop acute allergic reactions;
regularly take natural vitamins, adhere to intensive vitamin therapy.

Description:

Plasmacytoma (in the form of a solitary plasmacytoma or generalized myeloma) is a neoplastic disease of plasma cells caused by bone destruction, anemia, increased immunoglobulin, infections, and kidney disease. Diagnosis requires analysis of protein in the urine (according to Bence-Jones), determination of an increase in the number of plasma cells in a bone marrow biopsy, as well as an x-ray of osteolysis.

Symptoms:

Extramedullary myeloma of the skin (plasmocytoma), in contrast, proceeds without disease-specific lesions of the bone marrow, as well as other organs that are usually involved in the systemic process (spleen, lymph nodes). Skin lesions in extramedullary myelomas occur in 4% of cases. At the same time, observations are known when patients with primary cutaneous myeloma developed systemic myelomatosis after several years. With the development of plasma cell leukemia, the skin can also be involved in the pathological process, in which the lesions can have a specific character. Primary skin plasmacytoma refers to ILD with a relatively favorable clinical course. In the absence of metastasis involving the bone marrow, life expectancy in 40% of patients reaches 10 years. If it metastasizes from the bone marrow to the skin, this is usually considered a poor prognostic sign, the average life expectancy of such patients ranges from 1.5 months to 1 year.

In primary plasmacytoma, solitary or multiple foci appear on the skin in the form of infiltrated plaques, fairly clear, rounded outlines, ranging in size from 1 to 5-6 cm, which gradually increase, become nodular, spherical in shape, and have a dense elastic consistency. The surface of such tumors is usually smooth, shiny, but may macerate and ulcerate. Regional lymph nodes may be involved in the pathological process.

The plasmacytoma consists predominantly of monomorphic, densely adjacent complexes of mature plasma cells. In the cytoplasm, PAS-positive, diastase-resistant inclusions are determined, which are especially noticeable with electron microscopy. Immunoblasts, plasmablasts, lymphocytes are usually absent. Amyloid deposits are sometimes noted among the tumor cells or in the walls of blood vessels. A number of observations describe the presence of pseudoangiomatous structures containing erythrocytes in lacunar formations without endothelial lining. Immunoglobulins are detected in the cytoplasm of plasmacytic cells by direct immunofluorescence.

Phenotypic characteristics of plasmacytoma: CD20-; CD79a±; CD38+; LCA-. Diagnosis of primary plasmacytoma of the skin is quite difficult. Differential diagnosis is carried out with secondary plasma cells, which are observed in patients with multiple myeloma, with other clinical variants of TZLK and IBD, metastases to the skin of malignant neoplasms from internal organs, sarcoidosis, deep mycoses.
lymphoma

Causes of occurrence:

The etiology of plasmacytoma is still unclear. Two most probable points of view are discussed.

1. Plasmacytoma develops as a typical cell proliferation in response to an antigenic stimulus. Logically, this theory can be confirmed by the detection of a specific antigen. Meanwhile, it was possible to prove that M-proteins have antibody activity. The researchers proceeded from the fact that the presence of about 10 species of specificity with antibody activity excludes the randomness of the data obtained. Therefore, it was suggested that the antigenic stimulus can serve as a kind of inductor to start the proliferation process. A similar mechanism operates, for example, in recurrent polyarthritis, when an M-protein with the activity of antibodies to streptolysin is detected, as well as in tetanus infection, when the component reacts with horse macroglobulin.

The following facts speak against the concept based on the existence of antigenic stimulation:

Proliferative processes due to antigenic stimulus occur mainly in the peripheral organs of the lymphatic system: the proliferation of myeloma cells is observed primarily in the bone marrow;

Antigen stimulation typically results in the production of different Ig classes, with each Ig class having antibody activity that is heterogeneous in physicochemical and immunological properties.

Nevertheless, clinical observations and experimental data indicate that a certain role belongs to a specific or non-specific stimulus. So, to some extent, a typical picture of the disease can develop with chronic infections: osteomyelitis, pyelonephritis, tuberculosis, hepatitis, cholecystitis, and also with cholelithiasis. Rather natural than random, the fact of the combination of plasmacytoma or similar phenomena with other tumors should be called. In the analysis of significant clinical material, M-protein was found in 15-19% of neoplasms, primarily in the rectum and sigmoid colon, prostate and mammary glands.

2. Plasmacytoma is considered as a neoplasm. In this case, there must be a specific reason for the transformation of a lymphocyte into a plasma cell, and this in turn means that certain cells are out of control of normal cellular regulation and are involved in the process of abnormal proliferation. Immunological analysis using B-cell markers reveals a situation similar to chronic lymphocytic leukemia. Along with this, among the cells of plasmacytoma, transitional forms were found - from secreting plasma cells (including the production of M-fraction) to proliferation of predominantly B-cell type (with a low level of M-fraction). G neoplastic proliferation is evidenced by the first reports of an antigen specific for myeloma.

Although it follows from experimental data that genetic factors may play a special role, this fact has not yet been proven in relation to human plasmacytoma. It is most likely that this is a neoplasm. There are separate indications of the frequency of familial cases and association with other lympho- or myeloproliferative processes, as well as chromosomal abnormalities.

The involvement of viruses has been shown only in isolated cases. Chronic antigenic stimulation can be essential. Seligmann combined all existing knowledge into the "double stimulus" hypothesis. The first stimulus comes from the antigen and leads to a corresponding monoclinal B-cell proliferation, which, as an exception, may turn out to be a benign monoclonal gammopathy. The second oncogenic or mutagenic stimulus, months or years later, by transformation of a subclone of cells, leads to tumor growth. While endogenous clonal proliferation remains the basis of the pathogenetic theory, data on the activity of the humoral factor in experimental animals became known: the factor increased the number of cells with the plasmacytoma idiotytype and induced the appearance of monoclonal mlg in intact animals. At the same time, antibody production decreased during the primary response. It is believed that this factor could play a similar role in the human body.

Treatment:

For treatment appoint:

The proliferating plasma cell is to a certain extent sensitive to the action. There is a dependence on the degree of cell maturity, in addition, individual differences were revealed. Considering these factors, it is necessary to proceed from the individual dose of radiation therapy. In general, it is 2000-3500 rad.

Among the therapeutic measures, chemotherapy occupies the first place. In the treatment of cytostatics, the most pronounced clinical effect was obtained with the help of sarcolysin and melphalan. In the first 4 days, 0.25 mg / kg of melphalan + 2 mg / kg of prednisolone are prescribed, in the next 4 days - only prednisolone in gradually decreasing doses (1.5-1.0-0.5-0.25 mg / kg) . The second cycle is carried out with a break of about 6 weeks. As needed, use melphalan with a gradual increase in dose by 0.05 mg/kg. Treatment is carried out under the control of a clinical blood test, adjustments are made when the content of leukocytes decreases to 3000/μl.

Less effective is cyclophosphamide. Against the background of this therapy, the course of the disease can vary significantly. When using these two alkylating agents, sometimes after 4 years, an expiration effect occurs, when the tumor shows its properties less and less, which is partly associated with inhibition of the processes of differentiation and proliferation. A clear correlation between the type of plasmacytoma and the effect of cytotoxic therapy has not been established. Remissions occur in 60-70% of cases and can last for several years. Treatment with cytostatics allows you to extend the life of the patient from 17 to 31-50 months. However, it is associated with a real danger of development (2-6%). The fact of the development of resistance to drugs was the reason for the search for optimal combination therapy. As part of this problem, which is relevant for the clinic of other lymphoproliferative diseases, programs have been developed consisting of cyclophosphamide, carmustine, lomustine and prednisolone. Doxorubicin, Vinkristin and other preparations and their complexes are used with known success. Approximately 60% of patients combined therapy leads to a reduction of 75% in the size of the tumor mass with a corresponding clinical improvement. With the help of interferon, temporary remission was achieved in some cases.

A sign of a therapeutic effect is a decrease in the concentration of M-protein. It is also important to determine the content of rumicroglobulin and Bence-Jones protein, as well as peripheral blood B-lymphocytes. The first parameter, due to the short half-life (8-12 hours), changes significantly after a few days, and the paraprotein (half-life 15-20 hours) only after a few weeks. Of particular interest among peripheral blood B cells are monoclonal proliferating cells. Regular monitoring of these indicators is a condition for optimal therapy. The following are suggested as criteria for remission:

Reducing the tumor mass by more than 75%,
- decrease in paraprotein concentration up to 25%,
- decrease in the level of Bence-Jones by more than 90%.

A malignant tumor consisting of plasma cells that grow within the axial skeleton or in soft tissues is called plasmacytoma (hemoblastosis).

More about plasmacytoma

Another treatment for plasmacytoma is bone marrow (or stem cell) transplantation.

Metastases and recurrence

This type of disease rarely metastasizes. They can occur only at an advanced stage, if the neoplasm is large and the tumor cells begin to penetrate into the blood. Metastases spread through the blood vessels and can begin to form in the bones of the skeleton and internal organs. In case of successful treatment of the tumor, metastases are removed surgically. The spread of metastases is well suppressed by chemotherapy.

Relapses of the disease can occur at any time. The probability of their occurrence will be less after radical treatment (total resection of the tumor and polychemotherapy). If a recurrence is detected, then a second operation and second-line therapy are prescribed.

Forecast

If a solitary bone plasmacytoma is diagnosed, the prognosis is quite favorable. After treatment, the patient has a chance for a full life. The prognosis depends on the location and stage of the tumor and on the general health of the person.

With complete removal of the tumor, the patient's survival is 10-20% higher than with incomplete removal.

If the treatment is properly selected and carried out qualitatively, it can last several years. In very rare cases, patients live after diagnosis and treatment for about ten years. On average, if multiple myeloma is confirmed, most patients die within 3 years. After chemotherapy, almost 90% of patients live for more than two years, if treatment is not carried out, their life span will not exceed a few years.

Often the causes of death of the patient are renal failure, sepsis, progression of myeloma, often patients die from strokes and heart attacks.

Disease prevention

There is no effective prevention for plasmacytosis, but its early detection will help to undergo treatment on time, and increase the chances of recovery.