This is plasmacytoma. Diagnosis of multiple myeloma

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

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

Plasmacytoma 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. Once multiple myeloma is confirmed, most patients die within the first 3 years.

Prevention of cancer

In recent decades, there has been an increase in tumors of the hematopoietic system. Therefore, scientists are trying their best to find more effective methods combating this pathology, as well as ways to prevent it. So far, there is no effective prevention of plasmacytoma. The most important thing that anyone can do is to find out its symptoms, and if they find them in themselves, undergo an examination. Early detection illness significantly increases the chances of recovery.

Informative video

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

This cancer is one of the tumors that have low proliferative activity of malignant cells. Presumably the predecessors cancer cells In this case, they originate from the lymph nodes and enter the bone marrow by migrating 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 escaped 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 has great importance during the process of neoangiogenesis and tumor adhesion.

Symptoms of the disease

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

In plasmacytoma, bone pain is explained by increased bone resorption as osteoclasts are activated and infiltrated by myeloma cells. Arises systemic osteoporosis, its osteolytic lesions 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 part of the tubular bones. long bones. The lumbar and thoracic parts of the spine are subjected to compression, and there may be fish-mouth deformities.

Compression spinal cord may develop due to penetration of the tumor mass through the intervertebral foramina, or germination occurs directly from the vertebra affected by cancer. During compression clinical manifestations are sensory and motor disorders 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 plasmacytoma the appearance bacterial infection indicates that the patient has an immunodeficiency. Its basis is the syndrome of insufficiency of bacterial opsonization and antibody production, which is compared with reduced amount normal immunoglobulins in blood. Infections occur more often than other symptoms genitourinary system caused by multifactorial kidney damage. Pneumococcal and other infections are also detected, which are caused mainly by bacteria of encapsulated forms. Infectious complications Among the causes of death from plasmacytoma, one of the leading places stands out.

With this disease, hemorrhagic phenomena occur; they are explained by the resulting deficiency of coagulation factor. Hyperviscose syndrome, which occurs due to an increase in plasma viscosity, is accompanied by a number of neurological disorders, signs of hypervolemia also appear ophthalmological symptoms. When diagnosing plasmacytoma, the main criteria include 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 highest accuracy, making it possible to detect minimum doses paraprotein. This method is of particular value when it is necessary to monitor the results of therapy and provides a complete response to the treatment.

If the doctor has diagnosed indolent plasmacytoma, then immediate therapy is not required in this case. The indication for treatment is the symptoms of the disease associated with increased content paraprotein, as well as progression of osteolytic lesions. These are bone fractures, spinal cord compression, bone pain, spinal compression, impaired kidney function, hypercalcemia, severe anemic syndrome, as well as the occurrence of complications infectious nature. In cases of spinal cord compression, it is necessary, if possible, surgical intervention. For example, kyphoplasty or laminectomy is performed, supplementing these actions with dexamethasone therapy. Local irradiation may be prescribed, and for pathological bone fractures, orthopedic fixation is performed.

If multiple plasmacytoma is diagnosed, radiation therapy is used as palliative treatment for bone lesions. local lesions, especially if there is a pronounced pain syndrome. Special indication for radiation treatment is a bone lesion facial skull, as well as its basics. If high-dose therapy is not planned for a patient suffering from multiple myeloma, then most often the patient receives cytostatic treatment, which uses cyclic nonspecific drugs that are combined with corticosteroid hormones.

It is important to take into account that in the form long-term consequences side effects alkylating drugs sometimes develops acute secondary myeloid leukemia, accumulates in the bone marrow toxic effects, the production of stem cells is disrupted. In the future, such complications may prevent autologous transplantation.

If myeloma of the blood progresses, plasma cells are destroyed in the bone marrow, which mutate and become malignant. The disease belongs to paraproteinemic leukemia and is also called “blood cancer”. 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 may end unexpectedly fatal.

What is myeloma

This An abnormal condition called Rustitsky-Kahler disease shortens life expectancy. During a pathological process, cancer cells enter systemic blood flow, contribute to the intensive production of pathological immunoglobulin - paraproteins. These specific proteins, converted into amyloids, are deposited in tissues and disrupt the functioning of such important organs and structures such as kidneys, joints, heart. The general condition of the patient depends on the degree of the disease and the number of malignant cells. Differential diagnosis is required to make a diagnosis.

Solitary plasmacytoma

Plasma cell cancer of this type is distinguished by one focus of pathology, which is localized in the bone marrow and lymph node. For staging correct diagnosis multiple myeloma requires a series of laboratory research, to exclude the spread of multiple foci. For myeloma bone lesions the symptoms are similar, treatment depends on the stage of the pathological process.

With this pathology, several bone marrow structures become foci of pathology that rapidly progress. 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 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, therefore timely diagnosis– this is 50% successful treatment. Doctors distinguish 3 stages of myeloma pathology, in which severe symptoms diseases are only growing and intensifying:

1. First stage. Excess calcium predominates in the blood, an insignificant concentration of paraproteins and protein in the urine, hemoglobin reaches 100 g/l, and there are signs of osteoporosis. There is only one source of pathology, but it is progressing.
2. Second stage. The lesions become multiple, the concentration of paraproteins and hemoglobin decreases, the mass of cancer tissue reaches 800 g. Single metastases predominate.
3. Third stage. Osteoporosis progresses in the bones, 3 or more lesions are observed in bone structures ah, the concentration of protein in the urine and calcium in the blood is increased to the maximum. Hemoglobin pathologically decreases to 85 g/l.

Causes

Bone myeloma progresses spontaneously, but doctors have not been able to fully determine the etiology of the pathological process. One thing is known - people who have been exposed to radiation are at risk. Statistics report that the number of patients after exposure to such a pathogenic factor has increased significantly. According to the results long-term therapy It is not always possible to stabilize the general condition of a clinical patient.

Symptoms of multiple myeloma

When bone tissue is damaged, the patient first develops anemia of unknown etiology, which cannot be corrected even after therapeutic diet. Characteristic symptoms severe pain in the bones becomes evident, the occurrence of pathological fracture. Other changes in general health with the progression of multiple myeloma are presented below:

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

Diagnostics

Because the At first, the disease is asymptomatic and is not diagnosed in a timely manner, doctors are already identifying complications of myeloma, suspicions of renal failure. Diagnostics includes not only visual inspection patient and palpation of soft, bone structures, additionally required to undergo clinical examinations. This:

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

Treatment of multiple myeloma

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

• painkillers to relieve pain attack in the area of ​​bones;
• antibiotics penicillin series for recurrent infectious processes orally and intravenously;
• hemostatics to combat intense bleeding: Vikasol, Etamzilat;
• cytostatics to reduce tumor masses: Melphalan, Cyclophosphamide, Chlorobutin;
• glucocorticoids in combination with drinking plenty of fluids to reduce the concentration of calcium in the blood: Alkeran, Prednisolone, Dexamethasone.
• immunostimulants containing interferon, if the disease is accompanied by a decrease in immunity.

If as you grow malignant tumor observed high blood pressure on neighboring organs with their subsequent dysfunction, doctors make a decision on urgently eliminate surgical methods such a pathogenic neoplasm. Clinical outcome And potential complications after the operation may turn out to be the most unpredictable.

Nutrition for myeloma

To reduce relapses of the disease, treatment must be timely, and a diet must be included in the complex regimen. It is necessary to adhere to such a diet throughout life, especially during the next exacerbation. Here are the valuable ones recommendations from specialists on diet for plasmacytosis:

• reduce the consumption of protein foods to a minimum - no more than 60 g of protein per day is allowed;
• exclude from daily ration foods such as beans, lentils, peas, meat, fish, nuts, eggs;
• do not consume foods that may cause acute illness in the patient allergic reactions;
• take regularly natural vitamins, adhere to intensive vitamin therapy.

Description:

Plasmacytoma (in the form of solitary plasmacytoma or generalized myeloma) is a neoplastic disease of plasma cells caused by bone destruction, anemia, increased immunoglobulin, infections, and kidney disease. To make a diagnosis, a protein analysis in the urine (Bence-Jones method), determination of an increase in the number of plasma cells during a bone marrow biopsy, and an x-ray of osteolysis are necessary.

Symptoms:

Extramedullary myeloma of the skin (plasmocytoma), unlike of this disease lesions of the bone marrow, as well as other organs that are usually involved in the systemic process (spleen, The lymph nodes). Skin lesions in extramedullary myelomas occur in 4% of cases. At the same time, there are known observations in which patients with primary cutaneous myeloma developed systemic myelomatosis after several years. When plasma cell leukemia develops, the skin may also become involved. pathological process, in this case the lesions may have a specific character. Primary plasmacytoma of the skin belongs to ILDL with 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, it is usually considered a poor prognostic sign, average duration The lifespan of such patients ranges from 1.5 months to 1 year.

With primary plasmacytoma, solitary or multiple foci appear on the skin in the form of infiltrated plaques, quite 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 and shiny, but can macerate and ulcerate. Regional lymph nodes may be involved in the pathological process.

Plasmacytoma consists predominantly of monomorphic, tightly adjacent complexes of mature plasma cells. In the cytoplasm, PAS-positive, diastase-resistant inclusions are detected, which are especially noticeable under electron microscopy. Immunoblasts, plasmablasts, and lymphocytes are usually absent. Sometimes amyloid deposits are observed among tumor cells or in the walls of blood vessels. A number of observations describe the presence of pseudoangomatous structures containing erythrocytes in lacunae-like formations without endothelial lining. Using direct immunofluorescence, immunoglobulins are detected in the cytoplasm of plasmacytic cells.

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

Causes:

The etiology of plasmacytoma is still unclear. The 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 identification 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 types of specificities with antibody activity excludes the randomness of the data obtained. Therefore, it was suggested that an antigenic stimulus could serve as a kind of inducer to trigger 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 caused by an antigenic stimulus occur predominantly in peripheral organs lymphatic system: proliferation of myeloma cells is observed primarily in the bone marrow;

Antigenic stimulation usually leads to the production different classes Ig, while each class of Ig has antibody activity that differs in heterogeneity in physicochemical and immunological properties.

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

2. Plasmacytoma is considered a neoplasm. In this case, there must be a specific reason for the transformation of the lymphocyte into a plasma cell, and this in turn means that certain cells escape the 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, plasmacytoma cells were found transitional forms- from secreting plasma cells (including the production of the M-fraction) to proliferation predominantly B cell type(With low level M-fractions). G neoplastic proliferation is evidenced by the first reports of a myeloma-specific antigen.

Although it follows from experimental data that special role can play genetic factors, this fact has not yet been proven regarding human plasmacytoma. Most likely, this is a process of neoplasm. There are some indications of the frequency of familial cases of the disease and the relationship 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 may be significant. Seligmann combined all existing knowledge into the "dual incentive" hypothesis. The first stimulus comes from an antigen and leads to a corresponding monoclonal B-cell proliferation, which, exceptionally, may turn out to be a benign monoclonal gammopathy. A second oncogenic or mutagenic stimulus months or years later by transforming a subclone of cells leads to tumor growth. While endogenous clonal proliferation remains the basis of the pathogenetic theory, data on the activity of humoral factor in experimental animals: the factor increased the number of cells with the plasmacytoma idiotype and induced the appearance of monoclonal mlg in intact animals. At the same time, antibody production during the primary response decreased. It is believed that this factor could play a similar role in the human body.

Treatment:

For treatment the following is prescribed:

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 have been identified. Taking these factors into account, an individual dose should be taken into account. radiation therapy. In general, it is 2000-3500 rad.

Among therapeutic measures Chemotherapy ranks first. When treated with cytostatics, the most pronounced clinical effect was obtained using sarcolysin and melphalan. In the first 4 days, 0.25 mg/kg melphalan + 2 mg/kg prednisolone is 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 approximately 6 weeks. As necessary, use melphalan with a gradual increase in dose by 0.05 mg/kg. Treatment is carried out under control clinical analysis blood, adjustments are made when the leukocyte count decreases to 3000/μl.

Cyclophosphamide is less effective. During this therapy, the course of the disease can vary significantly. When using the two indicated alkylating agents, sometimes after 4 years an expiration effect occurs when the tumor exhibits 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 can extend the patient's life from 17 to 31-50 months. However, it is associated with a real risk of development (2-6%). The development of drug resistance led to 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. WITH famous success use Doxorubicin, Vinkristin and other drugs and their complexes. In approximately 60% of patients combination therapy leads to a 75% reduction in tumor mass size with corresponding clinical improvement. With the help of interferon, temporary remission was achieved in some cases.

Sign therapeutic effect serves to reduce the concentration of M-protein. It is also important to determine the content of rumicroglobulin and Bence Jones protein, as well as B-lymphocytes peripheral blood. The first parameter, due to its short half-life (8-12 hours), changes significantly after just 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:

Reduction of tumor mass by more than 75%,
- decrease in paraprotein concentration up to 25%,
- reduction in the Bence-Jones level 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 option for plasmacytoma is bone marrow (or stem cell) transplantation.

Metastasis and relapse

This type of disease rarely metastasizes. They can occur only at an advanced stage, if the neoplasm big size And tumor cells begin to penetrate the blood. Metastases spread throughout blood vessels and can begin to form in the bones of the skeleton and internal organs. If the tumor is successfully treated, the metastases are removed surgically. The spread of metastases is well suppressed by chemotherapy.

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

Forecast

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

At complete removal patient survival rate is 10-20% higher than with incomplete tumors.

If the treatment is correctly selected and carried out efficiently, it can last several years. Very in 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 more than two years; if treatment is not carried out, their life span will not exceed several years.

Often the reasons fatal outcome the patient becomes renal failure, sepsis, progression of myeloma, patients often die from strokes and heart attacks.

Disease prevention

There is no effective prevention for plasmacytosis, but early detection will help you receive treatment on time and increases the chances of recovery.