Plasma hemorrhagic diathesis, haemophilia

Proven content

Plasma hemorrhagic diathesis are diathesis in which blood coagulation disorders are observed, caused by too little blood coagulation factors contained in the plasma. They may be genetically determined, congenital or primary, or result from an impairment in the production of the plasma factor.

Plasma hemorrhagic diathesis – features

Plasma haemorrhagic flaws may be of the following nature:

1. innate,
2. original,
3. acquired,
4. genetic,
5. it can also present as an impairment in the production of a plasma factor.

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

Primary (congenital) plasma hemorrhagic diathesis

Haemorrhage A (haemophilia A)

It is the most important defect of all congenital bleeding disorders. The cause of the ailments is the deficiency of anti-haemophilic globulin. The symptoms of haemophilia A appear only in men, and it is women who transmit the disease to their offspring. Hence, the occurrence of increased haemorrhage and a prolonged clotting time exclude haemophilia A in the woman.

Symptoms of congenital haemorrhagic diathesis:

1. the patient’s predisposition to prolonged bleeding, e.g. as a result of an injury or after a tooth extraction,
2. disorders in the formation of a clot to stop bleeding,
3. repeated (usually) hemorrhages into a joint, e.g. in the knee or ankle.

As a consequence of recurring intra-articular bleeding, inflammatory-degenerative changes and joint stiffness are formed. The severity of symptoms and the prognosis depend on the degree of anti-bleeding globulin deficiency. Said degree of deficiency is usually expressed as a percentage.

Diagnosis of congenital diathesis

Diagnostics of plasma hemorrhagic diathesis consists in:

1. assessment of the patient’s predisposition to bleeding and haemorrhage,
2. long time of blood clotting (with normal bleeding time),
3. deficiency or a significant reduction in the concentration of anti-bleeding globulin.

Congenital haemorrhagic diathesis – treatment

Treatment of a hemorrhagic diathesis is symptomatic and should be performed under the supervision of a specialist. Fresh blood or blood derivatives containing a plasma factor in the form of anti-bleeding globulin are administered. Therefore, patients with haemophilia A require virtually no treatment during the asymptomatic period. Nevertheless, periodic control of the concentration of anti-bleeding globulin is necessary, and if its concentration drops to critical values, it is necessary to administer a substitution of an appropriate dose of any of the preparations mentioned.

In the event of bleeding, winter compression dressing should be applied immediately and the given body area should be immobilized. The patient should also be transported to hospital, where he will receive specialist help.

How to prevent

In prophylaxis, it is necessary to periodically assess the level of anti-bleeding globulin and supplement its deficiency. This is very important, especially if an operation is planned. In addition, an important preventive factor against possible impairment of motor function after intra-muscular or intra-articular haemorrhages is the avoidance of joint overload, trauma and intramuscular or intravenous injections. In addition, patients should be informed that their daughters may be carriers of the disease and that a carrier woman may have sick daughters of a carrier or sons.

Haemorrhage B (haemophilia B)

The cause of haemophilia B disorders is the lack of a plasma coagulation factor, more specifically Christmas factor. Haemophilia B is also congenital and is transmitted in a recessive, gender-specific manner. It occurs almost eight times less often than haemophilia A. The patient’s complaints and the clinical picture of this form of haemophilia are analogous to those in haemophilia A. The disease affects men and is transmitted by women (who are carriers) who remain healthy.

Diagnosis of hemorrhage B

Only a doctor can make a diagnosis. The degree and severity of the symptoms of haemophilia B is related to and proportional to the degree of Christmas deficiency in the blood. First aid for hemorrhages, treatment and prevention of such conditions are analogous to those in haemophilia A.

Choroba von Willebrand

Like hemorrhages, this condition is also a congenital plasma hemorrhagic diathesis that manifests itself from early childhood, and the occurrence of which determines the congenital deficiency of plasma von Willebrand factor, which is needed for the synthesis of anti-bleeding globulin and the function of platelets in the blood. It is therefore a plasma-platelet type hemorrhagic disorder that occurs much more often than haemophilia B and Owren’s syndrome.

Von Willebrand disease in laboratory tests manifests itself, inter alia, in prolonged bleeding with normal platelet counts.

The disease is inherited in a gender-neutral manner, and symptoms of this type of bleeding disorder can appear in both men and women. The conclusion is that the presence of an overt coagulation disorder in any family eliminates hemophilia, and suggests the so-called pseudohemophilia.

Symptoms of von Willebrand disease

The symptoms of this disease include:

1. predisposition to bruising and bleeding from mucous membranes,
2. heavy and long menstruation in women,

Diagnosis and treatment

Diagnosis of von Willebrand disease is possible based on the observation of the patient in the hospital and the performance of clinical and laboratory tests. First aid for bleeding and treatment and prevention are similar to those in haemophilia A.

Secondary (acquired) plasma hemorrhagic diathesis

A typical feature of secondary plasma bleeding disorders is i.a. the fact that, unlike congenital disorders – where symptoms of hemorrhagic bleeding often appear from childhood and usually result from the lack or deficiency of a specific plasma coagulation factor, they quite often deal with a simultaneous deficiency of many clotting factors.

What causes a deficiency of clotting factors?

1. inhibition of the activity of a given plasma factor,
2. insufficient synthesis of the factor by the diseased organ,
3. very high wear and tear during diseases (e.g. during intravascular coagulation syndrome,

Recognition of secondary bleeding disorders

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

The most common acquired plasma hemorrhagic diathesis

1. all ailments causing prothrombin deficiency due to impairment of its synthesis in the liver. Therefore, any factors that directly or indirectly lead to impaired liver function (e.g. hepatitis, toxic liver damage, cirrhosis), leading to a decrease in blood prothrombin concentration may cause secondary, plasma hypoprothrombinemic bleeding diathesis.
2. deficiency of vitamin Kkonieczny for its synthesis.

Such haemorrhagic diathesis is caused by factors and disease states leading to the depletion of vitamin K in the body (e.g. diarrhea, malabsorption and insufficient synthesis of vitamin K in the intestines in the case of insufficient bacterial flora).

Important! The liver, together with vitamin K, also synthesizes other plasma coagulation factors. Therefore, the liver plays an important role in indirectly securing 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 to inappropriate synthesis or absorption of vitamin K from the gastrointestinal tract.

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