Blood clotting disorders are disorders of the homeostatic process that can affect almost any component of the blood. Haemorrhagic diathesis can be related to both blood element abundance and impairment of their function and some are based on disorders resulting from gene damage. Many of these conditions are hereditary, but they can also have an aetiology in external factors, that is, they can be acquired over the course of life.
Glanzmann's thrombasthenia
Is a condition associated with abnormal platelet function that has a genetic basis-it is inherited. The main symptoms include early bleeding from the mucous membranes of the nose, gums and genital tract. Morphological results show normal platelet counts, but have impaired homeostasis. A prolonged bleeding time and impaired coagulation are also noted. Treatment of this condition is purely symptomatic
Plasma haemorrhagic septicaemia
Haemophilia - congenital haemorrhagic diathesis
Haemophilia is a genetic condition caused by a defect in the gene responsible for coding the plasma coagulation factor protein. It is inherited recessively and is sex-linked, hence it is mainly affected in men, while women are carriers of the defective gene.
The most characteristic symptoms are delivery strokes occurring spontaneously or after slight trauma. Repetition of such strokes can lead over time to contractures and eventually joint destruction and impaired function. Bleeding from mucous membranes as well as bleeding into soft tissues are also observed.
Treatment consists mainly of supplementing the missing clotting factor. The best treatment in terms of effect is home treatment - administration, supplementation of the missing factor after the injury has occurred. Prophylactic treatment is also important and consists of administering the missing clotting factor to the patient once every 48 hours, particularly in cases of increased clotting factor activity.
Von Willebard's disease
This is a haemorrhagic diathesis that, like haemophilia, is a congenital condition that is now recognised as the most common. It is caused by a deficiency of the von Willebard factor and can affect boys and girls equally. Symptoms are highly variable and in the severe form can be similar to those of haemophilia. The mild form of this disease usually only becomes apparent during surgical procedures.
Treatment is mainly the administration of von Willebard factor supplements and, when these are not available, fresh frozen plasma is administered.
Vascular haemorrhagic septicaemia
Schönlein-Henoch syndrome
At the root of this problem, there is an allergic-inflammatory process within the small blood vessels, which is the cause of their increased permeability. The inflammatory process is caused by streptococcus, which causes acute pharyngitis or pharyngitis, and it is only after about 2 or 3 weeks that the symptoms characteristic of this syndrome appear, such as small petechiae on the feet, calves and lower legs, abdominal pain or swelling and soreness in the joints - especially the ankles.
No platelet abnormalities are observed in blood tests, but protein and erythrocyturia are found in the urine.
The mild form of this condition does not require treatment. The main therapeutic measures include the administration of preparations to seal the blood vessels. If the form of the condition becomes severe, adrenal steroids are used. [1]
