GLANZMANN THROMBASTHENIA

Trombasteniya Glyantsmanna - a hereditary disease characterized by a qualitative deficiency of

platelets in their normal maintenance and manifested bleeding sinyachkovogo type.

Etiology and pathogenesis. Revealed two types of disease Glyantsmanna: Type A (9? Or p) and
type B (9S). The development of diseases associated with abnormalities glikoprotei-on IIb / Sha
and decreased activity glitseraldegidfosfat dehydrogenase and pyruvate kinase in platelets, which
leads to lack of blood clot retraction, disruption of the morphology of platelets, reducing the
ability of platelet adhesion, disruption of platelet aggregation, clotting time and platelet count
were normal.

The clinical picture is similar trombastenii Glyantsmanna thrombocytopenic purpura. The

disease manifests itself with bleeding of varying degree. Characteristic of hemorrhage in the
form of ecchymosis and ne-tehy. Prevail bleeding from the mucous membranes of the nose,
mouth, gastrointestinal tract and! Kidney. In girls, pubertal age may uterine bleeding. Less noted
bleeding in the sclera, optic fundi, the brain.

Diagnosis based on clinical and laboratory data. For clot-asthenia Glyantsmanna characterized
by:

presence of increased bleeding from relatives;

sinyachkovy type of bleeding;

normal platelet count, *

positive endothelial samples;

increasing the duration of bleeding;

decrease the degree of retraction of blood clot;

reduction or absence of platelet aggregation with adenosine diphosphate, collagen.

Treatment. When treating trombastenii Glyantsmanna use drugs that enhance adhesively-
aggregation properties of platelets: aminokapro-new acid etamzilat; metabolic means [for
example, trifosade-nin (ATP), magnesium preparations]. In addition, appoint calcium chloride,
al-korutin, local hemostatic means.

Prevention. For the prevention of bleeding is recommended refresher courses trifosadenina

(ATP) and magnesium products in 2-3 months. Should avoid prescribing, worsening adhesively-
aggregation ability of platelets, as well as physiotherapy (UFO, UHF), which may have a
damaging effect on them.
Forecast. The disease is incurable, but with age, bleeding may be reduced.