Haemorrhagic plasma diathesis, hemophilia

Human hemorrhagic diathesis is a defect in which blood coagulation disorders are observed due to insufficient blood coagulation factors contained in the plasma. They may be genetically conditioned, have an inborn or primary character, or result from impaired production of plasma factor.

Serum hemorrhagic diathesis - features

The plasma hemorrhagic diathesis can be:

• innate,
• original,
• acquired,
• genetic,
• it can also occur as an impaired production of plasma factor.

The basis for a clinically evident haemorrhagic diathesis is usually the complete or significant lack of one or more of the plasma elements of blood coagulation, or excessive activity of humoral bodies blocking their functioning, and thus blood clotting.

Primary (congenital) plasma hemorrhagic diathesis

Bleeding A (hemophilia A)

It is the most important flaw of all congenital bleeding disorders. The cause of the discomfort is the deficiency of antihemophilic globulin. The symptoms of hemophilia A appear only in men, while women transfer this disease to their offspring. Thus, the occurrence of increased hemorrhage and prolonged clotting time, exclude women from haemophilia type A.

Symptoms of congenital hemorrhagic diathesis:

• the patient's predisposition to long-lasting bleeds, e.g. due to injury or tooth extraction,
• disorders in the formation of a clot that inhibits bleeding,
• repeated (as a rule) haemorrhage into the joint, e.g. knee or ankle.

As a consequence of continuous recurrent delivery bleeds, inflammatory-degenerative changes and stiffness of the joints are formed. The degree of symptom severity and prognosis is dependent on the level of anti-bleeding globulin deficiency. This degree of scarcity is usually expressed as a percentage.

Diagnosis of congenital diathesis

Diagnosis of hemorrhagic diathesis consists of:

• assessment of the patient's predisposition to bleeding and haemorrhage,
• long blood coagulation time (with normal bleeding time),
• deficiency or significant reduction in the concentration of anti-bleeding globulin.

Congenital haemorrhagic diathesis - treatment

Treatment of hemorrhagic diathesis is symptomatic and should take place under the supervision of a specialist. Fresh blood or blood products are administered, which in their composition have a plasma factor, in the form of anti-bleeding globulin. Therefore, patients with haemophilia A do not require virtually no treatment during the asymptomatic period. Nevertheless, periodic control of the blood-pressure globulin level is needed, and if its concentration decreases to the critical values, substitution of the appropriate dose of any of these preparations is necessary.

In the event of bleeding, immediately apply winter, pressure dressing and immobilize the area of ​​the body. It is also necessary to transport the patient to the hospital, where he will receive specialist help.

How to prevent?

In prevention, a periodic assessment of the level of anti-bleeding globulin and supplementing its deficiency is needed. This is very important, especially if surgery is planned. In addition, an important preventive factor against possible impairment of motor function after intramuscular or intra-articular haemorrhage is to avoid overloading of the joints, injuries and intramuscular or intravenous injections. In addition, the patient should be informed that his daughter may be carriers of the disease, and the female carrier may have sick carrier or sons.

Bleeding B (haemophilia B)

The cause of type B haemophilia is the lack of plasma clotting factor, more specifically Christmas factor. Hemophilia B is also innate and is transmitted in a recessive, sex-related way. There is almost eightfold less frequent hemophilia A. Complaints from the patient and the clinical picture in this form of haemophilia are analogous to that in hemophilia A. The disorder affects men and is transmitted by women (carriers) who remain healthy.

Diagnosis of bleeding B

The diagnosis can only be made by a doctor. The degree and severity of haemophilia B is dependent and proportional to the degree of Christmas's deficiency in the blood. First aid in case of haemorrhage, treatment and prophylaxis of this type of conditions are analogous to that in haemophilia A.

Von Willebrand's disease

Similarly to bleeding, this condition is an inborn hemorrhagic diathesis which has been evident since early childhood, and whose occurrence determines the innate deficiency of von Willebrand's plasma factor, necessary for the synthesis of anti-bleeding globulin and platelet function to be normal. Thus, it is a hemorrhagic diathesis of the platelet-like type, which is much more common than hemophilia B and the Owren's syndrome.

Von Willebrand's disease in laboratory tests is manifested, among others prolonged bleeding time with the correct number of plates.

This disease is inherited in a non-gender-related manner, and symptoms of this type of hemorrhagic diathesis can occur in men and women. The conclusion is that the occurrence in a family of overt coagulation disorders virtually eliminates haemophilia, and suggests the so-called pseudohemofilię.

Symptoms of von Willebrand's disease

Among the symptoms of this disease, we mention:

• predisposition to the formation of bruises and bleeding from mucous membranes,
• abundant and long menstrual periods in women,

Diagnosis and treatment

The diagnosis of von Willebrand's disease is possible on the basis of observation of the patient in the hospital and the performance of clinical-laboratory tests. First aid in bleeding and treatment and prophylaxis are similar to haemophilia A.

Secondary (acquired) plasma hemorrhagic diathesis

A typical feature of secondary hemorrhagic plasma bleeds is that in contrast to congenital defects - where the symptoms of hemorrhage occur frequently from childhood and are usually due to the lack or deficiency of a specific factor of plasma coagulation, quite often we are dealing with simultaneous shortage of many coagulation factors.

What causes a deficiency of coagulation factors?

• inhibiting the function of a given plasma factor,
• insufficient synthesis of the factor by a sick organ,
• very high consumption in the loss of diseases (e.g. during intravascular coagulation syndrome,

Diagnosis of secondary bleeding disorders

Diagnosis and differentiation of secondary hemorrhagic plasma bleeds is time-consuming and complex, laboratory and diagnostic facilities are necessary, especially that the symptoms of excessive hemorrhage in different types of bleeding disorders are similar.

The most common acquired plasma haemorrhagic diathesis

• all ailments that cause prothrombin deficiency due to the impaired synthesis in the liver. Therefore, any factors directly or indirectly leading to impaired hepatic function (eg hepatitis, toxic liver damage, cirrhosis of the liver), leading to a reduction in prothrombin in the blood may cause secondary, plasma hypoprotrombinemic hemorrhagic diathesis.
• deficiency of vitamin K necessary for its synthesis.

This kind of hemorrhagic diathesis causes factors and disease states that lead to a reduction in the body of vitamin K (e.g. diarrhea, malabsorption and too little synthesis of vitamin K in the intestines in the case of deficiency of the appropriate bacterial flora).

Important! The liver together with vitamin K also synthesizes other plasma clotting factors. Therefore, the liver has a large role in indirect protection of normal blood clotting.

Treatment of acquired plasma hemorrhagic diathesis

Treatment of this type of disorders and their prevention is the treatment and prevention of all disease states and stimuli leading to abnormal liver function, or the inadequate synthesis or absorption of vitamin K from the gastrointestinal tract.