Trombotsitopatiya
Contents:
Description:
The term "trombotsitopatiya" is used for the general designation of all disturbances of a hemostasis caused by qualitative inferiority or dysfunction of thrombocytes.
Microphotos of thrombocytes: — at the scanning submicroscopy (the thrombocyte with various wiped
Trombotsitopatiya's symptoms:
The hemorrhagic syndrome which is clinically proceeding as thrombocytopenic (skin hemorrhages in the form of ecchymomas, are more often - on extremities, a trunk, in places of injections, a petechia standing, bleeding of mucous membranes of an oral cavity, a nose, genital tract). Emergence of a hemorrhage against the background of normal or slightly reduced maintenance of thrombocytes has to suggest the doctor an idea of qualitative inferiority of thrombocytes:
* at 72% of patients with recurrent nasal bleeding of not clear genesis dezagregatsionny trombotsitopatiya and at 12% - an angiohemophilia or Villebranda-Yurgens's syndrome come to light;
* at 12% of patients with dysfunctional juvenile uterine bleedings and at 6% of patients of childbearing age the research of system of a hemostasis revealed a hereditary trombotsitopatiya.
The hereditary trombotsitopatiya with obligatory verification like pathology is a peculiar lifelong characteristic of the patient and has to be considered at treatment of other diseases and at family consultations of both the patient, and his relatives.
Treat inborn trombotsitopatiya: Bernard-Sulye's syndrome (autosomal and recessive inheritance), Villebrand's syndrome of platelet type (autosomal and dominant inheritance), Glantsman's thrombasthenia (autosomal and recessive inheritance), a syndrome of "gray thrombocytes", the isolated deficit of 8 granules (autosomal and dominant inheritance), Herzhmansky-Pudlaka's syndrome (autosomal and recessive inheritance), a syndrome of Chediaka-Higasi, Viskotta-Aldrich's syndrome (defect is associated with X-chromosome), a TAR syndrome.
Trombotsitopatiya's reasons:
In implementation of functions of initially platelet and vascular hemostasis (formation of a platelet stopper) distinguish 7 main phases of platelet reactions and interactions: adhesion, rasplastyvaniye, reaction of release and aggregation, retraction of a blood clot, exhibiting of a platelet factor 3 and activation of cascades of coagulant system. Reaction of adhesion happens parallel to activation of thrombocytes with the participation of glycoproteins of a membrane of the thrombocytes which are receptors of collagen and Villebrand's factor. Thrombocytes are activated under action:
* the collagen which is a part of a subendothelial vystilka of vessels;
* ADF, released from the damaged cells,
* the thrombin which is formed in the site of damage to result of initiation of the cascade of coagulation;
* adrenaline;
* factor of activation of thrombocytes;
* cell-bound immune complexes.
At activation there are a change of a form of thrombocytes, formation of outgrowths of a membrane, a rasplastyvaniye of adgezirovanny thrombocytes and emission from thrombocytes of contents of the granules, ADF and A2 thromboxane strengthening activation and involvement in process of a thrombogenesis of the circulating thrombocytes and also modification of a complex of the membrane glycoproteins gaining ability to fibrin binding. The activated thrombocytes take part in formation of a fibrinous clot, accelerating operation of the coagulative cascade. The catalyst of reaction of coagulation is the platelet factor 3 appearing on a surface of the activated thrombocytes. Disturbance of one or several of the described reactions can lead to disturbances of functional activity of thrombocytes and development of a hemorrhagic syndrome - the main clinical sign of trombotsitopatiya. Similar disturbances can be hereditary or arise under the influence of external factors (reception of medicines, change of proteinaceous composition of plasma).
Acquired trombotsitopatiya develop generally owing to:
* disturbances of adhesive and aggregation function of thrombocytes and availability of a factor 3 (at uraemia, cirrhosis, tumors and parasitic diseases);
* consumption and structural damage of thrombocytes at the diseases which are followed by the IDCS;
* blockade of thrombocytes proteins (paraproteinemic hemoblastosis), ADF-aggregation disturbance (scurvy, B12-scarce anemia).
Trombotsitopatiya's treatment:
Hemotransfusion and its components (plasma, thrombocytes) at the majority of a trombotsitopatiya is useless and can aggravate dysfunctions of thrombocytes. Transfusion of donor plasma and erythrocytes is recommended only at massive blood loss for compensation of volume and correction of anemia. Transfusion of donor thrombocytes is necessary at bleeding against the background of operative measures at the states which are characterized by a combination of a trombotsitopatiya and thrombocytopenia.
At massive uterine and nasal bleeding intravenous administration to 100 ml of 5-6% of solution of e-aminocapronic acid is necessary.
Planned therapy. Patients with a trombotsitopatiya are subject to constant dispensary observation. The full laboratory inspection defining a form of trombotsitopatiya and its communication with diseases and various influences has to be carried out. Family inspection in case of identification hereditary trombotsitopatiya at one of family members is desirable.
