Plasmacytoma
Description:
The plasmacytoma (in the form of a single plasmacytoma or a generalized myeloma) is the neoplastic disease of cells of plasma caused by destruction of bones, anemia, increase in immunoglobulin, infections and diseases of kidneys. The analysis of a protein in urine (according to Bensu-Jones), definition of increase in quantity of cells of plasma is necessary for diagnosis at a marrow biopsy, and also ossifluence X-ray.
Plasmacytoma symptoms:
The extramedullary myeloma of skin (plasmacytoma) unlike a multiple myeloma proceeds without damage of marrow, specific to this disease, and also other bodies which are usually involved in system process (a spleen, lymph nodes). Damage of skin at extramedullary myelomas meets in 4% of cases. At the same time observations when at patients with primary skin myeloma after several years the system myelomatosis developed are known. At development of plazmokletochny leukemia skin can be also involved in pathological process, in it the centers of defeat can have specific character. Primary plasmacytoma of skin belongs to VZLK with rather favorable clinical current. In the absence of innidiation with involvement of marrow and a hypercalcemia life expectancy at 40% of patients reaches 10 years. If the multiple myeloma metastasizes from marrow in skin, it usually is considered as a bad predictive sign, average life expectancy of such patients fluctuates from 1,5 months to 1 year.
At primary plasmacytoma on skin there are solitary or multiple centers in the form of infiltrirovanny plaques, enough accurate, roundish outlines, from 1 to 5-6 cm in size which gradually increase, become knotty, spherical in a form, have I plotnoelastiches-forge a consistence. The surface of such tumors usually smooth, brilliant, but can be macerated and ulcerate. Regional lymph nodes can be involved in pathological process.
The plasmacytoma consists preferential of complexes of mature plasmocytes monomorphic, densely adjacent to each other. In cytoplasm CHIC-positive, diastazorezistentny inclusions which are especially noticeable at a submicroscopy are defined. Immunoblasts, plasmablasts, lymphocytes, as a rule, are absent. Sometimes among tumor cells or in walls of vessels amyloid deposits are noted. In series of observations existence of the psevdoangaomatozny structures containing erythrocytes in lakunoobrazny educations without endothelial vystilka is described. By method of a direct immunofluorescence in cytoplasm of cells of a plazmotsitarny row immunoglobulins come to light.
Phenotypical characteristic of a plasmacytoma: CD20-; CD79a±; CD38+; LCA-. Diagnosis of primary plasmacytoma of skin is quite difficult. The differential diagnosis is carried out with secondary plazmatsptoma which are observed at patients with a multiple myeloma, with other clinical options of TZLK and VZLK, metastasises in skin of malignant new growths from internals, a sarcoidosis, deep mycoses.
lymphoma
Plasmacytoma reasons:
The etiology of a plasmacytoma is still not clear. Two most probable points of view are discussed.
1. The plasmacytoma develops as typical cellular proliferation in response to an antigenic incentive. Identification of a specific antigen can confirm logically this theory. Meanwhile it was succeeded to prove that M-proteins have activity of antibodies. Researchers recognized the fact that existence near 10e types of spetsifichnost with activity of antibodies excludes accident of the obtained data. Therefore it was suggested that the antigenic incentive can serve as a peculiar inductor for proliferation process start. The similar mechanism operates for example at recurrent polyarthritis when reveal M-protein with activity of antibodies to streptolysin, and also at a tetanic infection when the component reacts with macroglobulin of a horse.
Against the concept based on existence of an antigen challenge tell the following facts:
- the proliferative processes caused by an antigenic incentive happen preferential in peripheral bodies of lymphatic system: proliferation of cells of a myeloma is observed first of all in marrow;
- the antigen challenge, as a rule, leads to products of the different classes Ig, at the same time each class Ig has activity of antibodies which differ in heterogeneity on physical and chemical and immunological properties.
Nevertheless clinical observations and eksperimentalnye.danny demonstrate to what a certain role to a prinadla - zhit to a specific or nonspecific incentive. So, to some extent the typical picture of a disease can develop at persistent infections: osteomyelitis, pyelonephritis, tuberculosis, hepatitis, cholecystitis, and also at cholelithiasis. Rather natural, than accidental, it is necessary to call the fact of a combination of a plasmacytoma or the similar phenomena with other tumors. In the analysis of the considerable clinical material M-protein it is found in 15-19% of new growths, first of all in direct and sigmoid guts, prostatic and mammary glands.
2. The plasmacytoma is considered as a new growth. In this case there has to be a specific reason of transformation of a lymphocyte into a plasmocyte, and it in turn means that certain cells get out of the control of normal cellular regulation and are involved in process of abnormal proliferation. Immunoassay by means of V-cellular markers reveals the situation similar to a chronic lymphoid leukosis. Along with it among cells of a plasmacytoma transitional forms - from the cosecreting plasmocytes (including products of M-fraction) before proliferation of preferential V-cellular type are found (with the low level of M-fraction). G of neoplastic proliferation testify the first messages on the antigen specific to a myeloma.
Though follows from experimental data that genetic factors can play a special role, concerning a plasmacytoma of the person this fact is not proved yet. It is the most probable that it is process of a new growth. There are separate instructions on the frequency of family cases and communication with other lympho-or myeloproliferative processes, and also anomalies of chromosomes.
Participation of viruses was shown only in isolated cases. The chronic antigen challenge can have essential value. Seligmann combined all existing knowledge in a hypothesis of "a double incentive". The first incentive proceeds from antigen and leads to the corresponding uniclinal V-cellular proliferation which by way of exception can be a high-quality monoclonal gammapathy. The second oncogenous or mutagen incentive in months or years by transformation of a subclone of cells leads to tumoral growth. While endogenous clonal proliferation remains a basis of the pathogenetic theory, data on activity of a humoral factor at experimental animals became known: the factor raised number of cells with an idiotype of a plasmacytoma and induced emergence of monoclonal mlg in intact animals. At the same time products of antibodies at primary answer decreased. Believe that the specified factor could play a similar role and in a human body.
Treatment of the Plasmacytoma:
The proliferating plasmocyte in a certain measure is sensitive to action of radiation therapy. There is a dependence on degree of a maturity of cells, besides, - individual distinctions are revealed. Considering these factors, it is necessary to proceed from an individual dose of radiation therapy. In general it makes 2000-3500 is glad.
Among medical actions first place is won by chemotherapy. At treatment by cytostatics the most expressed clinical effect was gained by means of sarcolysine and a melfalan. In the first 4 days appoint 0,25 mg/kg of a melfalan of +2 mg/kg of Prednisolonum, in the next 4 days - only Prednisolonum in gradually decreasing doses (1,5-1,0-0,5-0,25 mg/kg). The second cycle is carried out with a break approximately in 6 weeks. As required use мельфалан with gradual increase in a dose on 0,05 mg/kg. Treatment is carried out under control of clinical blood test, introduce amendments at reduction of maintenance of leukocytes to 3000/mkl.
Cyclophosphamide is less effective. Against the background of this therapy disease can vary considerably. When using of two specified alkylating means sometimes 4 years later there is an effect of the expiration when the tumor shows the properties less that partly connect with oppression of processes of a differentiation and proliferation. To accurate correlation between type of a plasmacytoma and action of cytotoxic therapy it is not established. Remissions occur in 60-70% of cases and several years can proceed. Treatment by cytostatics allows to prolong the term of life of the patient from 17 to 31-50 months. However it is accompanied by real danger of development of a leukosis (2-6%). The fact of development of resistance to drugs served as the reason of search of an optimum combination therapy. Within this problem urgent for clinic of other limfoproliferativny diseases the programs consisting of cyclophosphamide, a karmustin, a lomustin and Prednisolonum were developed. With the known success use Doxorubicin, Vinkristin and other drugs and their complexes. Approximately at 60% of patients the combination therapy leads to decrease by 75% of the sizes of tumoral weight with the corresponding clinical improvement. By means of interferon temporary remission was in some cases reached.
Decrease in concentration of the M-protein is a sign of therapeutic effect. Important also determination of content of a rumikroglobulin and Bens-Jones's protein, and also V-peripheral blood lymphocytes. The first parameter in connection with short half-life (8-12 hour) considerably changes in several days, and a paraprotein (half-life 15 - 20 hour) only in several weeks. Among V-cells of peripheral blood monoclonal proliferating cells are of special interest. Regular control of these indicators serves as a condition of optimum therapy. As criteria of remission offer the following:
- reduction of tumoral weight more than for 75%,
- decrease in concentration of a paraprotein to 25%,
- decrease in level of a proteinuria of Bens-Jones more than for 90%.
