Multiple myeloma

Description:

Multiple myeloma — the malignant tumor of system of V-lymphocytes arising at the level пре - In - cellular stages of monoclonal development and keeping differentiation potency to a final stage — a plasmocyte. Definition of a multiple myeloma as plazmokletochny tumor with a paraproteinemia not absolutely precisely as the option of a disease at which pathological cells synthesize immunoglobulins is allocated, but do not cosecrete them in blood (the so-called not cosecreting myeloma).
There are data on considerable fluctuations of the standardized indicators of incidence of a multiple myeloma in various countries. In our country according to selective researches of a multiple myeloma meets in 5,3% of cases of all diseases of hemoblastoses. The annual average standardized incidence indicators in various territories fluctuated from 0,3 to 1,1.

The multiple myeloma is observed equally often at men and women. More often aged people get sick 50 years are more senior.

Symptoms of the Multiple myeloma:

The developed clinical picture of a disease is usually preceded by the asymptomatic period of various duration.
In a clinical picture takes place:
1. Damage of a skeleton. Radiological the miyelomatozny tumors of various sizes in edges, a breast, a backbone, a clavicle, a skull, extremities which are shown the pains in the beginning having flying character are found, then they become more intensive and long. Quite often there are pathological changes with deformation of a thorax, a compression of bodies of lumbar and chest vertebrae.
2. Defeats of a nervous system. They are connected in most cases with tumoral damage of bones of a calvaria and a backbone therefore can develop a paraplegia, a syndrome of a prelum of roots of spinal nerves, a hemiplegia or a hemiparesis. In some cases diencephalic and pituitary and mental disorders, giperkaltsiyemichesky encephalopathy are observed.
3. Renal pathology. The myelome nephropathy arising at 60 — 90% of patients is characterized by a proteinuria, a renal failure, an angioretinopatiya, hypostases and a hamaturia. Owing to destruction of bones the hypercalcemia and a giperkaltsiurikemiya is observed. At 1/3 patients the azotemia is a cause of death.
4. Hyper viscous syndrome. It is caused by high content in serum of proteins, meets at a myeloma of IgG and IgA. Changes of an eyeground and possible development of heavy disturbances of the central nervous system are connected with the increased viscosity of blood.
5. Hemorrhagic syndrome. Disturbances of platelet, plasma and vascular factors of a hemostasis. Paraproteins settle on membranes of thrombocytes. They form complexes with V, VII, VIII factors of coagulation, a prothrombin, fibrinogen.
6. Disturbances of a hemogenesis. More often normokhromny anemia develops. At bleedings it has iron deficiency character, in cases of deficit of B12 vitamin and folic acid in marrow lines of a megaloblastichesky hemopoiesis come to light. Miyelomonokletochny infiltration is found in 86 — 90% of patients at researches of a marrowy hemopoiesis.
7. Immunity depression. Decrease in content in blood serum of level of normal immunoglobulins (frequent less than 20%), disturbance the antiteloobrazovaiiya, reduction of quantity of granulocytes and their functional deficiency cause predisposition of patients to infections.

Reasons of the Multiple myeloma:

The origin of paraproteinemic hemoblastoses from one cell is confirmed by uniformity of paraproteins on a class, type of L-chains, hallo - and to an idiotype, compliance of amount of the cosecreted immunoglobulins to the mass of a tumor.

Results of immunomorphological researches convince that tumoral process at a multiple myeloma and Valdenstrem's macroglobulinemia arises at the level of V-kletok-predshestvennits and does not depend on an antigenic incentive.

At a terminal stage of a course of these diseases along with the general manifestations characteristic of hemoblastoses, find changes of products of monoclonal Ig.

Treatment of the Multiple myeloma:

At patients with a multiple myeloma somatic and hematologic compensated (stages of IA and NA) abstain from cytostatic therapy under control of a monthly research of a gemogramma and level of pathological immunoglobulin (Pig). At a part of patients within several years the slow form of a disease can come to light. At emergence of signs of progressing of process (increase of tumoral weight, existence of a pain syndrome, anemia, increase of number of Pig) resort to cytostatic therapy. The alkylating drugs were widely used: sarcolysine, мельфалан (left-handed isomer of sarcolysine), Cyclophosphanum (эндоксан), derivative nitrozomochevina.