Hemorrhagic diathesis

A number of pathologies, the characteristic feature of which is the tendency to increased bleeding and bleeding, is called "hemorrhagic diathesis." The disease is polyethological, complex, and in the absence of treatment can lead to severe and even unpredictable consequences.

ICD Code 10

• D 69 - purpura and other phenomena of hemorrhage;
• D 69.0 - purpura of allergic genesis;
• D 69.1 - platelet quality defects;
• D 69.2 - other purpura of a non-morbocytopenic etiology;
• D 69.3 - idiopathic purpura of thrombocytopenic etiology;
• D 69.4 - other primary thrombocytopenia;
• D 69.5 - secondary thrombocytopenia;
• D 69.6 - unspecified thrombocytopenia;
• D 69.8 - other specified hemorrhages;
• D 69.9 - Hemorrhage, unspecified.

Causes of hemorrhagic diathesis

Depending on the causes and etiology of the disease, these types of hemorrhagic diathesis are distinguished:

• diathesis caused by a disorder of platelet hemostasis. This type includes such pathologies as thrombocytopathy and thrombocytopenia. Factors of development may be a decrease in immunity, kidney and liver disease, virus damage, chemotherapy treatment and irradiation with radiation;
• a disease caused by disturbances in the processes of blood coagulability - it can be a disorder of fibrinolysis, the use of anticoagulant and fibrinolytic drugs, various types of hemophilia, etc .;
• impaired vascular wall permeability, caused by a lack of ascorbic acid, hemorrhagic angioectasia, or vasculitis;
• a disease caused by platelet hemostasis - von Willebrand's disease, thrombohemorrhagic syndrome, radiation sickness, hemoblastosis, etc.

The pathogenesis of hemorrhagic diathesis can be divided into several main sections:

• hemorrhages due to blood clotting disorder;
• hemorrhages associated with changes in the processes and organics of platelet formation, as well as with disturbances in their properties;
• Hemorrhages that develop as a result of vascular damage.

In a normal healthy body, the wall of the vessel can be partially permeable to gaseous and soluble substances. For elements of blood and proteins, the wall, as a rule, is impermeable. If there is a violation of its integrity, then a difficult process of coagulation is launched, aimed at preventing blood loss - so the body tries to prevent the emergence of a state that is life-threatening.

In itself, pathological hemorrhage usually occurs for two reasons - an increase in the permeability of the vessel wall and a disorder of the process of folding. Sometimes there is also a hypothesis that the mechanism of hemorrhage can be triggered by the increase in the properties of red blood cells passing through the vascular barrier with a slight dilution of blood, or with severe anemia. In what cases can this happen?

• Avitaminosis C (scurvy), rutin and citrine.
• Infectious diseases, sepsis, streptococcus, meningococcus, typhoid fever, etc.
• Intoxication (poisons, medications).
• The effect of heat.
• Allergic pathologies in the body.
• Neurotrophic disorders.

[1], [2], [3], [4], [5], [6], [7], [8], [9], [10]

Symptoms of hemorrhagic diathesis

The first signs of hemorrhagic diathesis are often the main symptoms of the disease. The patient has small bluish spots (similar to bruising), a red rash on the anterior part of the ankle, hips or on the outside of the forearms.

In advanced cases, the rash is combined with necrosis, ulcers are formed. Sometimes this condition can be accompanied by pain in the epigastric region, attacks of vomiting with elements of blood in the vomit.

The disease can begin acutely and even suddenly. Bleeding, bleeding in any tissues and organs can be accompanied by the development of anemia.

Most often, patients turn to the doctor for severe bleeding, for example, after minor trauma. However, bleeding can be arbitrary, independent of the presence of direct tissue damage.

With a thorough questioning of the patient, it can be found that the symptoms of hemorrhagic diathesis have haunted him since childhood. In some cases, such signs disturbed the relatives of the person who fell ill (if the disease has a hereditary etiology).

Hemorrhagic diathesis in children

Hemorrhagic diathesis, which manifested itself in childhood, is most often hereditary. The imperfection of the folding system, the vascular system, platelet defects in this case are predetermined by genetics and inherited by the recessive type.

Disease in a child can manifest as follows:

• there is sustained bleeding gums during the eruption or change of teeth;
• often cause bleeding from the nasal cavity;
• hemorrhagic eruptions may appear;
• blood is detected in the analysis of urine;
• the child hurts joints, and at inspection the hemorrhage and deformation of an articulate bag is defined;
• periodically it is possible to observe hemorrhages in the retina;
• girls develop menorrhagia.

In newborns, the disease can manifest itself from the second-third day of life. The most common symptoms include gastrointestinal bleeding, which are found in the form of bloody defecation and vomiting. These signs can arise both separately from each other, and simultaneously. In addition, there is bleeding from the umbilical wound, from the mucosa of the mouth and nasopharynx, from the urinary system. The most dangerous are bleeding in the brain and its membranes, as well as in the adrenal glands.

Hemorrhagic diathesis in pregnancy

Pregnancy in patients with hemorrhagic diathesis is combined with great danger, therefore requires special attention and professional guidance. Even a small medical error can lead to terrible consequences.

During pregnancy a sick woman can be trapped by the following dangers:

• development of dropsy and preeclampsia (in 34% of cases);
• probability of spontaneous abortion (39%);
• premature birth (21%);
• development of placental insufficiency (29%).

Among the most serious complications can be called premature detachment of the normally located placenta, bleeding during childbirth and after childbirth. With such difficulties, it is possible to encounter almost 5% of cases of diseases in pregnant women.

The newborn can be diagnosed with chronic hypoxia, delayed development, prematurity, neonatal thrombocytopenia, hemorrhagic syndrome, and the most serious complication, intracranial hemorrhage, which is found in about 2-4% of cases.

Hemorrhagic diathesis during lactation is less dangerous, but requires no less strict control by the doctor. A woman should carefully monitor her health, prevent skin damage, take medicines prescribed by a doctor. Many patients, patients with hemorrhage, are able to give birth and nourish healthy and healthy children.

Classification of hemorrhagic diathesis

Types of hemorrhagic diathesis are distinguished by the types of bleeding. There are five such types:

• The hematoma type of hemorrhage is characterized by ubiquitous hemorrhages in soft tissue and articular cavities. Such hemorrhages have significant dimensions, they cause pain and tension in the tissues. Can be combined with gum, nasal, gastric, uterine bleeding, hematuria.
• Mixed type - occurs due to failure of platelet function, as well as changes in the composition of the plasma, which leads to a violation of blood clotting. Can be accompanied by petechiae (pinpoint hemorrhages).
• Vascular-purple type - appears as a result of pathology of the walls of blood vessels, for example, after infectious diseases. It can be accompanied by inflammation of the tissues, pinpoint hemorrhages (they can be finger-fingered, since they protrude slightly above the skin surface). Hematuria is not excluded.
• Angiomatous type - accompanied by persistent bleeding associated with vascular damage. Small rashes and hemorrhages, as a rule, no.
• The petechial-spotty type is not accompanied by massive bleeding, but gives itself out by the appearance of small spots, bruises, and petechia.

The most commonly found vascular-purpura type of hemorrhages. In this disease, small hemorrhages can be observed on the surface of the skin, where the tissue is often squeezed, for example, when using a strap, when wearing dense rubber bands in clothing, but also on the buttocks (the seat of frequent sitting).

In addition, they are distinguished by:

• hereditary variant of diathesis, which manifests itself in childhood and continues throughout the life of the patient;
• the acquired variant of hemorrhagic diathesis, depending on the violations of the system of blood coagulation and the state of the walls of blood vessels.

[11], [12], [13], [14], [15], [16], [17]

Consequences and complications of hemorrhagic diathesis

Hemorrhagic diathesis can complicate the chronic form of iron deficiency anemia (often with reduced acidity of gastric juice and lack of appetite).

In some cases, the course of hemorrhagic diathesis is observed against the background of immunity disorders and allergic manifestations, which sometimes themselves can provoke hemostasis, and may complicate the disease. This is associated with the sensitization of the blood spilled into the tissue structures.

Patients with hemorrhagic diathesis are at high risk because of the likelihood of HBV infection and HIV infection (with frequent blood transfusions).

With repeated hemorrhages in the articular bags, there may appear a limitation of amplitude, and even immobility of the joint.

Squeezing large blood clots of nerve fibers can cause various numbness and paralysis.

The internal bleeding is invisible to the eye, especially in various parts of the brain and adrenal glands.

[18], [19], [20], [21], [22], [23]

Diagnosis of hemorrhagic diathesis

Assessment of anamnesis of the disease and collection of patient complaints: when the first bleeding was discovered, whether there was a feeling of weakness and other signs of diathesis; The patient himself explains the appearance of such symptoms.

• Assessment of the life history: the presence of chronic diseases, long-term use of medications, heredity, the presence of bad habits, cancer history, intoxication.
• External examination of the patient: shade and skin condition (pale, cyanotic, hyperemic, with rashes or hemorrhages), increase, soreness and joint mobility, pulse and blood pressure.
• Blood tests: a decrease in the number of erythrocytes, a decrease in hemoglobin level. The color index is normal, the number of leukocytes and platelets in different patients may differ and even not exceed the limits of normal values.
• Urinalysis: erythrocyturia (hematuria) is determined in the presence of hemorrhages in the kidney or urinary system.
• Biochemical blood test: the amount of fibrinogen, alpha gamma globulin, cholesterol, sugar, creatinine, uric acid is estimated, as well as electrolytic metabolism to control the general condition of the body. Coagulogram, evaluation of antihemophilic factors.
• Instrumental diagnostics:
  • a study of the bone marrow material seized during puncture bone piercing (often sternal). It is carried out in order to evaluate the hematopoietic function;
  • trepanobiopsy - a study that is performed on a sample of the bone marrow and bone element with a part of the periosteum, most often removed from the iliac bone. For this, a specific device is used - trepan. Appointed to assess the state of the bone marrow.
• To determine the duration of bleeding, use the method of piercing the upper finger phalanx or ear lobe. If the function of blood vessels or platelets is disturbed, the duration indicator increases, and if there are not enough clotting factors, it does not change.
• The time of folding is determined by the formation of a blood clot in a sample of the patient's venous blood. The less clotting factors in the blood, the longer the folding time.
• The plaque test allows to estimate the degree of occurrence of subcutaneous effusions when squeezing the skin fold in the subclavian area. At this site, hemorrhage appears only with vascular and platelet abnormalities.
• The flagellum test is similar to the previous one and consists of applying a tourniquet to the shoulder region of the patient (approximately 5 minutes). In case of a disease, the forearm is covered with pinpoint hemorrhages.
• A test with a cuff is the application of a cuff from a blood pressure monitor. The doctor pumps the air up to about 100 mm Hg. Art. And stores it for 5 minutes. Further, an assessment of hemorrhages on the patient's forearm occurs.
• Differential diagnosis consists in consulting other specialized specialists - for example, therapist, allergist, neurologist, infectious disease specialist, etc.

[24], [25], [26], [27]

Treatment of hemorrhagic diathesis

Drug treatment is prescribed individually (different drugs are used depending on the type of disease):

• vitamin preparations (vitamins K, P, vitamin C) in case of damage to the walls of blood vessels;
• glucocorticoid hormones (hormonal drugs of the adrenal cortex) with a decrease in the number of platelets. Prednisolone from 1 mg / kg / day is most often used, with the possibility of increasing the dosage by 3-4 times (treatment course from 1 to 4 months);
• special preparations, so-called coagulating factors, with their lack.

For an urgent stop of the bleeding that begins:

• the laying of a harness;
• tamponade of cavities;
• tight bandage;
• Put a cold warmer or ice on the place with bleeding.

Operative treatment:

• surgery to remove the spleen (splenectomy) is carried out with significant bleeding. Such an intervention can increase the life span of blood cells;
• operation to remove the affected vessels, which were sources of recurring bleeding. If necessary, vascular prosthetics are performed;
• puncture of the joint bag with subsequent suctioning of accumulated blood;
• surgery to replace the joint with an artificial one, if irreversible changes occurred that are not amenable to treatment.

Blood transfusion therapy (blood transfusion of blood donors):

• plasma transfusion containing all coagulating factors (freshly frozen drug), allows to restore the level of all factors, and also to support the immune defense of the patient;
• transfusion of platelet mass;
• Transfusion of erythrocyte mass (sometimes instead of this drug eroded red blood cells are used, which reduces the severity of adverse events during transfusion). It is used in extreme cases - for example, with severe anemia and anemic coma.

Physiotherapy treatment means exposure to magnetic or electric fields, which contributes to the resorption of blood clots and the restoration of damaged tissues.

LFK is appointed during remission to develop sufficient motor amplitude in joints that have been hemorrhaged.

Homeopathy: with an increased degree of systemic hemorrhage and a tendency to bleeding, the following homeopathic remedies can help.

• Phosphorus. It is also used for coagulopathy, hemorrhagic fever, and avitaminosis C.
• Lachessis, Crotalus. Can be used for hemorrhagic vasculitis and hemophilia.
• Arsenicum album. Most often combined with the above drugs.
• Bothrops. It is prescribed to patients with hemorrhagic vasculitis in the presence of damage to the skin, including ulcers, trophic changes.

Alternative treatment of hemorrhagic diathesis

Herbal treatment involves the use of plants that stimulate metabolism, increase blood coagulation, improve tissue healing.

Some of the plants used can contain toxic substances, so treatment is carried out only with the approval of the attending physician.

It is recommended to prepare the following mixtures of medicinal herbs:

• picking with chifurium, yarrow, nettle leaves, sporrows, leaves of strawberry and cuff - 8 g of collection brewed in 400 ml of boiling water, insist for a quarter of an hour, drink 100 ml after eating three times a day;
• collection with ripe, corn stigmas, Kalina inflorescences, raspberry leaves, rose hips and mountain ash - cook, as in the previous recipe;
• collection with St. John's wort, nettle, yarrow, darkneck, alder, camomile and blackberry leaf - 4 g of raw material are brewed with 200 ml of boiling water, insist 3 hours, drink a third cup four times a day after eating;
• a leaf of vinca, hazelnut, calgan root, a leaf of a bilberry, berries of a guelder-rose, a mountain ash and a dogrose - use for brewing 7 g of 350 ml of boiling water, insist 3 hours, take 1/3 cup three times a day after a meal.

Such mixtures are taken at the rate of 2 months. After 2 weeks, the course can be repeated, if necessary, reducing it to 1 month.

With a steady improvement of the condition, plants such as a periwinkle leaf, cuff, scabby mountaineer, St. John's wort, corn stigmas and rowan berries - components can be replaced with each new course of treatment.

In the acute period, it is important to comply with bed rest for at least 3 weeks.

[28], [29], [30], [31], [32], [33], [34], [35], [36], [37], [38], [39], [40]

Prevention of hemorrhagic diathesis

Hemorrhagic diathesis is really a serious and complex disease that requires an individual and careful selection of the treatment regimen. If we talk about hereditary pathology, then it is difficult to identify preventive measures, but some of the advice will still help if you do not cure the disease, then at least slow down its development and prevent the emergence of complications, which is also important.

Such activities include:

• Strengthening the body's protective capabilities (includes exercise, exercise, regular exercise, hardening of the body, adequate nutrition);
• additional intake of vitamin products (in particular, vitamin K - for example, in the form of preparations Menadion or Sinkavit);
• strict adherence to hygienic rules - this simple recommendation helps prevent the development of infectious diseases that can give rise to pathological processes in the body;
• sanation of foci of chronic infection in the body, timely treatment of inflammatory diseases;
• dispensary supervision, regular visits to a doctor;
• exclusion of sun exposure.

Secondary prophylaxis consists in periodic medical examinations of the population, for early detection of the disease.

Prognosis of hemorrhagic diathesis

Patients with hemorrhagic diathesis should be registered in hematology in their native village, as well as in the regional center of hemophilia. Patients undergo regular evaluation of the hematopoiesis and coagulation system, provide information to both the patient himself and the community health workers, conduct (if necessary) potentially dangerous for such patients the types of treatment - they provide dental and surgical care. Close and relatives of patients also receive instructions and are trained in the technique of first aid to patients with hemorrhagic diathesis.

The outcome and prognosis of the disease can be different. If the patient has been adequately pathogenetic, hemostatic and substitution treatment, then the prognosis can be considered quite favorable.

Hemorrhagic diathesis with malignant course, when it is impossible to control the degree and frequency of hemorrhages, and also in the presence of complications, the outcome of the disease can be lethal.