Hemorrhagic diatheses

Hemorrhagic diatheses

Hemorrhagic diatheses - bunch of hereditary and acquired diseases. The basic which clinical sign is the raised staxis-bent of an organism to repeated bleedings and hemorrhages, spontaneous or after insignificant traumas. Primary hemorrhagic diatheses carry to the congenital heredo-familial diseases, which characteristic sign - deficiency of any one blood-coagulation factors; an exception is the angiohemophilia at which some factors of a hemostasis are broken. Symptomatic hemorrhagic a diathesis are characterised by insufficiency of several blood-coagulation factors.

Classification

The hemorrhagic diathesis can be put In a basis of working classification the scheme of normal process of coagulation of blood. Diseases are grouped according to phases of process of coagulation of blood.

The hemorrhagic diatheses caused by disturbance of the first phase of coagulation of blood.

  • Deficiency of plasma components of a thromboplastinopoiesis
  • Deficiency trombocytic components trombostyne formations - quantitative insufficiency of thrombocytes, qualitative insufficiency of thrombocytes
  • Angiohemophilia
  • The hemorrhagic diatheses caused by disturbance of the second phase of coagulation of blood
  • Deficiency of plasma components trombinoobrazovanija - the factor II
  • Presence of antagonists formation Thrombinum
  • Presence of inhibitors to factors II, V, VII and X
  • The hemorrhagic diatheses caused by disturbance of the third phase of coagulation of blood: deficiency of plasma components formation fibrin
  • Hemorrhagic diatheses caused by the accelerated fibrinolysis
  • Hemorrhagic diatheses caused by development of disseminated intravascular coagulation: a defibrination syndrome (disseminated intravascular coagulation, a consumption coagulopathy)

Clinic

Deficiency of plasma components thromboplastinopoieses-factors VIII, IX, XI and XII. Disease symptoms remind a hemophilia. The staxis is expressed moderately: usually bleedings after traumas and small surgical interventions. Spontaneous hemorrhages arise was rarely. Working capacity of patients is not broken. Deficiency of the factor XII clinically does not show. Disease clinically remind a hemophilia, educe at any age against a basic disease; the family anamnesis is not burdened. Angtogemofilija - family - hereditary form a hemorrhagic diathesis caused by congenital deficiency in plasma of the antihemorrhagic vascular factor angiohemophilia. Deficiency of plasma components trombinoobrazovanija - factors II, V, VII and X. Signs of the raised staxis which appear sometimes at the moment of a birth in the form of a bleeding from an umbilical funicle are observed, later at a cutting and change of dens, at sick women - with the beginning of a menses. There are nasal bleedings, menorrhagias, bleedings after sorts, bruises, exodontias, surgical interventions. There can be intermuscular hematomas and hemarthrosises, usually without disturbance of functions of joints. Disease symptoms can appear at the moment of a birth. Disease usually easier, than at deficiency of other factors of a prothrombin complex. In the majority of patients find hemorrhages in a skin, nasal bleedings. Women often have menorrhagias. Clinically insufficiency of the factor X was rarely shows hemorrhages. Only at its almost full absence there are nasal bleedings, a menorrhagia, bleedings from mucosas GASTROINTESTINAL TRACT and nephroses, intracraneal hemorrhages, hemarthrosises and intermuscular hematomas. The maintenance of the factor X can is enlarged at pregnancy and consequently during bleeding sorts, as a rule, are absent. However in a puerperal period serious bleedings that is bound to falling of concentration of the factor X are observed. After the surgical interventions executed without the conforming preparation, bleedings also are possible.

Treatment

Deficiency of plasma components tromboplastichnoobrazovanija - factors VII, IX, XI and XII. Patients usually it is not required. The Basic disease, on depressing of hemorrhages. On purpose depressing of production of antibodies and cupping of hemorrhages. Immunodepressants: azathioprine (Immunal) on 100-200 mg and Prednisolonum on 1-1,5 mg/kg daily before full disappearance of antibodies.

Deficiency of plasma components trombinoobrazovanija - factors II, V, VII and X. At bleedings spend a plasma transfusion or bloods. At the big surgical interventions it is more preferable to pour concentrates of the scarce factor, introducing PPSВ-a preparation keeping a prothrombin, a proconvertin. Should include active therapy of a basic disease; with the haemostatic purpose spend transfusion plasmas or bloods.

Presence of antagonists is reduced to intravenous introduction of solution of a protamine sulphate of 1 %, the quantity of an introduced preparation depends on hyperheparinemia degree; the control over treatment consists in definition of level of a heparin in blood.

Deficiency of plasma components formation fibrin. Treatment is necessary at carrying out and a staxis or at carrying out by this patient of surgical interventions. Apply transfusion integral blood, plasma, and in serious cases of a cryoprecipitate.

Defibrination syndrome: at a defibrination syndrome basic disease treatment against which it has educed first of all is necessary. For cupping of hemorrhages introduction of anticoagulants of direct action. Usually introduce intravenously a heparin: an initial dose 50-100 Unit. On 1 kg of weight;

Then hourly till 10-15 Unit. On 1кг. Its intramuscular introduction is not recommended, since. Because of its slowed down absorption it is difficult to check hyperheparinemia offensive. At a combination of a syndrome of a defibrination to a sharp thrombocytopenia a heparin dose reduce twice, simultaneously prescribing a hemotransfusion. Fibrinations are aggravated with a staxis and can harm to the patient. Coumarin preparations apply to long treatment, but, to brake a defibrination, high doses which, sharply reducing the maintenance of factors of coagulation, enhance bleedings are necessary. Fibrinolysis inhibitors are contraindicative, since they conduct to formation in vascular thrombuses, their introduction can be accompanied by staxis advance.

Complication

Complications at hemorrhagic diatheses depend on localisation of hemorrhages. At repeated hemorrhages in joints there are hemarthrosises, at formation of extensive hematomas in the field of transit of large excitatory fulcrums probably prelum of nerves with development of paralyses, paresises at hemorrhages in a brain there are symptoms, characteristic for cerebral circulation disturbance. At repeated hemotransfusions and plasmas the serumal hepatitis can educe. At patients with full absence of factors of coagulation the antibody formation is possible, that considerably reduces efficacyy of transfusions; posttransfusion reactions are possible.

Preventive maintenance

Relapses consists in transfusion of the conforming transfusion mediums which raise level of the scarce factor and stop hemorrhages. The medicogenetic consultations focusing spouses of monogynopaediums with a congenital pathology in system of coagulation of blood concerning planning of brooding have great value.

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