Trombocytopathies

According to current classifications, one of which is given below, thrombocytopathy divided into hereditary (Congenital) and acquired (symptomatic), with both the first, and when Second prevails petechial-spotted type of bleeding, inferiority is revealed microcirculatory hemostasis.

Overwhelming Most thrombocytopathy characterized by various disturbances aggregation of platelets (when deployed forms - all or most aggregating agents, with partial - to separate them from), that may be associated with a deficit of membrane glycoproteins, is a receptor for aggregating agents (Glanzmann thrombasthenia, trombotsitodistrofiya, or illness Bernard - Soulier, Wiskott-Aldrich syndrome, and others.), and violation release of dense granules or absence (storage pool disease), COX deficiency, thromboxane and other enzymes, or blocking them from outside (acetylsalicylic acid and other drugs), violation of calcium transport and function of cells of the contractile apparatus. Less common isolated factor deficiency 3 platelets - clotting lipid matrix.

These major violations can be combined with a constant or intermittent thrombocytopenia (trombotsitodistrofiya, syndromes Mey - Hegglina, Viskotta - Aldrich, syndrome absence radius et al.), dramatic increase (to 7- 9 m) diameter of platelets (trombotsitodistrofiya, May's syndrome - Hegglina) or, opposite, its decrease (Wiskott - Aldrich).

Ristomitsin-aggregation impaired in angiogemofilii (von Willebrand disease) due to lack of the appropriate factor in the plasma and trombotsitodistrofii due to lack of platelet membrane receptors to this factor, content in normal plasma.

Thrombocytopathy often associated with immune disorders (Wiskott - Aldrich, Chediak - Higashi et al.), bone dysplasias, ligaments, total or partial lack of pigmentation (albinism) and other pathology.

Classification thrombocytopathy

Hereditary forms

• 1. With a primary violation of platelet aggregation (dizagregatsionnye) - The absence of the first and second phases of aggregation and release reaction saved:
  • and) in violation of all kinds of aggregation and clot retraction (not in all forms), but with preserved primary adhesiveness to glass (Glanzmann thrombasthenia, Essential athrombia and other forms of);
  • to) with a partial violation of certain types of aggregation:
    • Insulated violation collagen aggregation;
    • Insulated violation ADP- and (or) Thrombin-aggregations (Meia Hegglina syndrome with platelet macrocytosis, Cause of calves in leukocytes and thrombocytopenia; Pearson anomaly-Stob, afibrinogenemia hereditary and others.).

• 2. In violation of the reaction and the release of the second phase of platelet aggregation (while maintaining the first phase):
  • and) the presence of the granules and their components and platelets, but in the absence of release (secretion) - Aspirin-like syndrome, and others.;
  • to) insufficient storage pool disease (store) granules and their components:
    • dense deficit (protein-free) granules and their components - ADP, Serotonin, catecholamine (in conjunction with albinism - a syndrome Herzhmanskogo-Pudlaka; with aplasia of the radius - TAR syndrome; immune deficiency - syndrome Chedika - Higashi et al.);
    • deficiency of α-granules (protein) and their components - β-thromboglobulin, factor a 4, growth factor, platelet fibrinogen and von Willebrand factor (gray platelet syndrome and others.);
    • deficiency of both types of granules;
    • shortage of lysosomal hydrolases and acidic.

• 3. In violation of platelet adhesion to glass and collagen, sharp weakening ristomycin aggregation (while maintaining the physiological forms of aggregation):
  • and) normal platelets and decrease in activity in plasma vWF and, not infrequently, factor VIII (angiogemofilija, or von Willebrand's disease);
  • to) with an anomaly of platelets - macrocytosis, lack receptors ristomycin and vWF, thrombocytopenia (tromʙodistrofija, or illness Bernara- With hive);
  • in) Insulated violation adhesion to collagen.

• 4. C deficiency or reduced availability factor 3 Platelet (no material breach of the adhesive and aggregation).

• 5. Other complex abnormalities and platelet dysfunction:
  • and) when immunodeficiency - Wiskott-Aldrich syndrome (with mikrotrombotsitopeniey);
  • to) in connective tissue dysplasia (Ehlers-Danlos syndrome, Marfan and others.), congenital heart disease;
  • in) congenital deafness, and nephritis;
    • when giperreninemii, hyperaldosteronism
    • cell hyperplasia and yukstglomerulyarnyh (Bartter syndrome).

Acquired (symptomatic) shape

• 1. When hemoblastoses, myeloproliferative diseases and essential thrombocythemia:
  • and) giporegeneratornye dizagregatsionnye;
  • to) consumption patterns (at DIC) ;
  • in) mixed origin.

• 2. When the general and regional DIC - forms of consumption and bundles.

• 3. At B₁₂-deficiency anemia.

• 4. When uremia.

• 5. With hemodialysis and extracorporeal circulation (extravascular forms of consumption).

• 6. When the blockade of platelet close- and paraproteins (multiple myeloma, Val disease- denstrema, gammapatii, cryoglobulinemia).

• 7. When scurvy (violation of the ADP aggregation).

• 8. When radiation sickness.

• 9. If massive transfusion (massive transfusion syndrome).

• 10. When massive thrombosis and giant angioma (consumption patterns).

• 11. Drugs and toksogennye form - when taking aspirin and other nonsteroidal anti-inflammatory drugs, penicillin and carbenicillin, dipiridamola, cytotoxic drugs, etc..

The table lists the criteria for the differential diagnosis of a number of typical laboratory parameters thrombocytopathy, representing each of the groups listed in the labeling of this pathology. The table also includes hemophilia A because of its similarity to a number of parameters angiohemophilia (von Willebrand's disease).

The most difficult diagnostics angiohemophilia (von Willebrand disease), since in recent literature describes a number of its variants, differing from each other in the pathogenesis, the type of abnormality of von Willebrand factor, and so on. d. Significant signs of the disease causes a variety of suspect, what, perhaps, in this case, there is not one disease, but a group.

The table shows the characteristics of violations, inherent in the various options angiohemophilia.

In clinical practice, the most frequent two varieties angiohemophilia - I type (about 70 % cases) and IIA type (10-12 % cases) .

These forms are characterized by identical violations and differ only in the level of antigen, bound to vWF. They should be differentiated type IIB, which is easily distinguished by increased ristomycin aggregation at a reduced activity of von Willebrand factor in the blood plasma, and von Willebrand psevdobolezn, whereby increased ristomycin-aggregation combined with normal plasma vWF and its antigen. For type IV characteristic of normal time aigiogemofilii capillary bleeding.

In von Willebrand disease transfusion of fresh frozen plasma and cryoprecipitate enhance the activity of factor VII:C for a longer period, than in hemophilia A and B at the same time improve ristomycin-cofactor activity.

Quantitative determination of the activity of the von Willebrand factor - Available technique, giving a much more accurate information, than the definition ristomycin-cofactor activity of blood plasma, which often does not reveal a moderate decline (to 35-50 % norms) von Willebrand factor. Meanwhile, such a decrease is observed in a sufficiently large number of people with angiohemophilia. Given the high incidence of the disease, apparently, need for more widespread adoption of this technique in practice. This laboratory is able to identify and increase of plasma levels of von Willebrand factor, that it is important to determine the extent of damage of the endothelium in many vascular diseases (vasculitis, atherosclerosis and others.), and to identify thrombophilia.

According to current classifications, one of which is given below, thrombocytopathy divided into hereditary (Congenital) and acquired (symptomatic), with both the first, and in the second it prevails petechial-spotted type of bleeding, inferiority is revealed microcirculatory hemostasis.