Table differential diagnosis of hemorrhagic diathesis. Hemorrhagic diathesis classification etiology pathogenesis clinic diagnosis treatment

In the differential diagnosis of various forms of bleeding, the classification based on pathogenesis turned out to be the most satisfactory. According to it, hemorrhagic diathesis can be caused by:

Platelet damage (thrombocytopenia, thrombocythemia or thrombopathy);

Lack of any factor necessary for blood clotting;

The predominance of any of the factors that prevent coagulation (coagulopathy);

Vascular disease (vascular purpura).

This classification is somewhat arbitrary, since the relationships of the vascular wall, platelets, coagulation factors associated with cellular elements of the blood and plasma are so complex that isolated disorders can hardly be imagined. Nevertheless, such a classification is possible, since in certain cases the role of individual factors dominates.

If bleeding is determined, the first practical action should in any case be to determine the platelet count, which makes it possible to recognize diseases such as thrombocytopenic purpura, as well as those accompanied by an increase in the platelet count, primary thrombocythemia, and symptomatic thrombocytosis. If the platelet count is normal, we can still talk about platelet dysfunction or thrombopathy.

Among thrombopathies, hereditary diseases such as Glanzmann-Nägeli disease, or thrombasthenia, are known, in which platelet aggregation and blood clot retraction are pathologically altered. The bleeding time is, of course, prolonged. Von Willebrand-Jurgens disease (von Willebrand factor deficiency, hereditary pseudohemophilia, angiohemophilia, constitutional thrombopathy, which in fact is not a thrombopathy, but rather a disease resembling true hemophilia) is even more common.

Purpura is less common, the disease is characterized by bleeding in the form of spots, it is observed most often in girls (as opposed to true hemophilia), it is also characterized by prolonged bleeding time, normal clotting time, but mainly a disorder of thromboplastin formation. In fact, we are talking about secondary thrombopathy, based on the absence of factor VIII and factor IX coagulation.

The diagnosis can be made based on the determination of prolonged bleeding time and the absence of antihemophilic globulin (factor VIII).

Sometimes this disease is observed in combination with hypercholesterolemia.

A functional disorder of very large platelets is observed in May-Hegglin anomaly and other forms of hemorrhagic diathesis in which giant platelets are detected. These congenital anomalies are most often combined with thrombocytopenia.

Thrombopathies usually include Portsmouth syndrome, in which platelet aggregation does not occur as a result of the influence of collagen fibers, and Bernard-Soulier macroplatelet thrombopathy, in which the diameter of the largest part of the platelets is above 4.5 microns. This is the same hereditary thrombopathy as the May-Hegglin polyphilic disorder of platelet maturation.

Secondary thrombopathy is more often observed, in which, due to the absence of platelet factor III, neither adhesion, aggregation, nor retraction of the clot occurs, the consumption of prothrombin and the generation of thromboplastin are also impaired.

Secondary thrombopathy is observed with paraproteinemia, hypergammaglobulinemia (Waldenström disease, myeloma, cirrhosis of the liver), uremia, leukemia, myelosclerosis, malignant anemia, and congenital anomalies of the cardiovascular system. Thrombocytopenic purpura is of the same origin. Thrombopathy can also be caused by fibrinogen degradation products, as well as heparin, dextran, and aspirin.

Four main studies help to make a differential diagnosis of various forms of hemorrhagic diathesis:

Determination of platelet count;

Bleeding time;

Prothrombin time;

Partial thromboplastin time (PTT).

If the platelet count and function are normal, the other three studies are normal, and only the bleeding time is prolonged, then we may be talking about vascular purpura. With it, the Rumpel-Leede sign is positive.

If the partial thromboplastin time (PTT) or prothrombin time is prolonged, coagulopathy is possible. If both factors are prolonged, we may be talking about a deficiency of prothrombin, fibrinogen, or V, X factors.

If the partial thromboplastin time (PTT) is pathologically altered, but the prothrombin time is normal, only a deficiency of factors VIII, IX, XI, XII is possible. If the partial thromboplastin time (PTT) is normal, but the prothrombin time is prolonged, we may be talking about factor VII deficiency.

A characteristic feature of vascular purpura is the negative results of studies and the positivity of Rumpel-Leede's symptom, which, however, can be positive with thrombocytopenia and even with coagulopathies.

Petechiae primarily indicate platelet-related bleeding, but are also characteristic of vascular purpura. Large subcutaneous bleeding is characteristic of coagulopathy, but also occurs with thrombocytopenic purpura.

A number of pathologies, the distinctive characteristic of which is a tendency to increased bleeding and bleeding, is called “hemorrhagic diathesis.” The disease is multi-etiological, complex, and if left untreated can lead to serious and even unpredictable consequences.

ICD 10 code

• D 69 – purpura and other hemorrhagic phenomena;
• D 69.0 – purpura of allergic origin;
• D 69.1 – platelet quality defects;
• D 69.2 – other purpura of non-thrombocytopenic 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 – unspecified hemorrhage.

Causes of hemorrhagic diathesis

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

• diathesis caused by a disorder of platelet hemostasis. This type includes pathologies such as thrombocytopathy and thrombocytopenia. Development factors may include decreased immunity, kidney and liver diseases, viral damage, chemotherapy treatment and radiation exposure;
• a disease caused by disorders of blood clotting processes - this may be a disorder of fibrinolysis, the use of anticoagulant and fibrinolytic drugs, various types of hemophilia, etc.;
• impaired permeability of vascular walls caused by a lack of ascorbic acid, hemorrhagic angioectasia or vasculitis;
• a disease caused by platelet hemostasis - von Willebrand disease, thrombohemorrhagic syndrome, radiation sickness, hemoblastosis, etc.

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

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

In a normal healthy body, the vessel wall may be partially permeable to gaseous and soluble substances. The wall is usually impenetrable for blood elements and proteins. If its integrity is violated, then a complex coagulation process is launched, aimed at preventing blood loss - this is how the body tries to prevent the occurrence of a life-threatening condition.

Pathological bleeding itself usually occurs for two reasons - an increase in the permeability of the vessel wall and a disorder in the coagulation process. Sometimes it is also hypothesized that the mechanism of hemorrhage can be triggered due to an increase in the ability of red blood cells to pass through the vascular barrier with a slight thinning of the blood, or with severe anemia. In what cases can this happen?

• Vitamin deficiencies C (scurvy), rutin and citrine.
• Infectious diseases, sepsis, streptococcus, meningococcus, typhoid fever, etc.
• Intoxication (poisons, medications).
• Effect of high temperature.
• Allergic pathologies in the body.
• Neurotrophic disorders.

Symptoms of hemorrhagic diathesis

The first signs of hemorrhagic diathesis are most often at the same time the main symptoms of the disease. The patient develops small bluish spots (similar to bruises), a red rash on the front of the ankle, thighs, or on the outer part of the forearms.

In advanced cases, the rash is combined with areas of necrosis, and ulcers form. 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 into any tissues and organs may be accompanied by the development of anemia.

Most often, patients consult a doctor about severe bleeding, for example, after a minor injury. However, bleeding can also be arbitrary, independent of the presence of direct tissue damage.

Upon careful questioning of the patient, one can find that the symptoms of hemorrhagic diathesis have haunted him since childhood. In some cases, similar signs worried the relatives of the sick person (if the disease has a hereditary etiology).

Hemorrhagic diathesis in children

Hemorrhagic diathesis, which manifests itself in childhood, is most often hereditary. Imperfections in the coagulation system, vascular system, and platelet defects in this case are predetermined by genetics and are inherited in a recessive manner.

The disease in a child may manifest itself as follows:

• there is persistent bleeding of the gums during the period of teething or changing teeth;
• causeless bleeding from the nasal cavity often occurs;
• hemorrhagic rashes may appear;
• blood is detected in a urine test;
• the child has joint pain, and examination reveals hemorrhage and deformation of the joint capsule;
• hemorrhages in the retina can be periodically observed;
• girls develop menorrhagia.

In newborns, the disease can manifest itself already from the second or third day of life. The most common symptoms include gastrointestinal bleeding, which appears as bloody bowel movements and vomiting. These signs can occur either separately from each other or simultaneously. In addition, bleeding is observed from the umbilical wound, from the mucous membrane of the oral cavity and nasopharynx, and from the urinary system. The most dangerous are bleeding into the brain and its membranes, as well as into the adrenal glands.

Hemorrhagic diathesis during pregnancy

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

During pregnancy, a sick woman may face the following dangers:

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

Among the most serious complications are premature abruption of a normally located placenta, bleeding during childbirth and after childbirth. Such difficulties can be encountered in almost 5% of cases of illness in pregnant women.

A newborn child may be diagnosed with: chronic hypoxia, developmental delay, prematurity, neonatal thrombocytopenia, hemorrhagic syndrome, as well as the most serious complication - intracranial hemorrhage, which is found in approximately 2-4% of cases.

Hemorrhagic diathesis during lactation is less dangerous, but requires no less strict monitoring by a doctor. A woman should carefully monitor her well-being, prevent skin damage, and take medications prescribed by a doctor. Many patients with hemorrhage are able to give birth and feed healthy and healthy children.

Classification of hemorrhagic diathesis

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

• Hematoma type of hemorrhage - characterized by widespread hemorrhages into the soft tissue and joint cavities. Such hemorrhages are significant in size, they cause pain and tension in the tissues. Can be combined with gum, nasal, gastric, uterine bleeding, hematuria.
• Mixed type - occurs due to a failure of platelet function, as well as changes in plasma composition, which leads to impaired blood clotting. May be accompanied by petechiae (point hemorrhages).
• Vascular purpuric type - appears as a result of pathology of the walls of blood vessels, for example, after infectious diseases. May be accompanied by tissue inflammation and pinpoint hemorrhages (they can be felt with a finger, as they protrude slightly above the skin surface). Hematuria cannot be excluded.
• Angiomatous type - accompanied by persistent bleeding associated with vascular damage. As a rule, there are no minor rashes or hemorrhages.
• Petechial-spotted type - is not accompanied by massive bleeding, but reveals itself by the appearance of small spots, hematomas, and petechiae.

The most common type of hemorrhage is the vascular purpuric type. With this disease, small hemorrhages can be observed on the surface of the skin, where tissue compression often occurs, for example, when using a belt, when wearing tight elastic bands in clothes, as well as on the buttocks (a place of frequent sitting).

In addition, they are distinguished by:

• a hereditary variant of diathesis, which manifests itself already in childhood and continues throughout the patient’s life;
• an acquired variant of hemorrhagic diathesis, depending on disorders of the blood coagulation system and the condition of the vascular walls.

Consequences and complications of hemorrhagic diathesis

Hemorrhagic diathesis can be complicated by a 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 noted against the background of immune disorders and allergic manifestations, which sometimes themselves can provoke hemostasis, and can even complicate the disease. This is associated with sensitization of blood spilled into tissue structures.

Patients with hemorrhagic diathesis are at high risk due to the likelihood of infection by the hepatitis B virus and HIV infection (with frequent blood transfusions).

With repeated hemorrhages into the joint capsules, limited amplitude and even immobility of the joint may occur.

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

Internal bleeding invisible to the eye, especially in various parts of the brain and adrenal glands, poses a great danger.

Diagnosis of hemorrhagic diathesis

Assessing the medical history and collecting patient complaints: when the first bleeding was detected, whether there was a feeling of weakness and other signs of diathesis; How does the patient himself explain the appearance of such symptoms?

• Assessment of life history: presence of chronic diseases, long-term use of medications, heredity, presence of bad habits, history of cancer, intoxication.
• External examination of the patient: the shade and condition of the skin (pale, bluish, hyperemic, with rashes or hemorrhages), enlargement, pain and mobility of the joints, the state of the pulse and blood pressure.
• Blood tests: a decrease in the number of red blood cells and a decrease in hemoglobin levels are determined. The color indicator is normal, the number of leukocytes and platelets may differ in different patients and may not even go beyond normal values.
• Urine tests: erythrocyturia (hematuria) is determined in the presence of hemorrhages in the kidneys or urinary system.
• Biochemical blood test: the amount of fibrinogen, alpha and gamma globulins, cholesterol, sugar, creatinine, uric acid, as well as indicators of electrolytic metabolism are assessed to monitor the general condition of the body. Coagulogram, assessment of antihemophilic factors.
• Instrumental diagnostics:
  • examination of bone marrow material removed during puncture of the bone (usually the sternum). It is carried out in order to assess hematopoietic function;
  • trephine biopsy is a study that is carried out on a sample of bone marrow and a bone element with a part of the periosteum, most often removed from the ilium. For this purpose, a specific device is used - a trephine. Prescribed to assess the condition of the bone marrow.
• To determine the duration of bleeding, the method of piercing the upper digital phalanx or earlobe is used. If the function of blood vessels or platelets is impaired, then the duration indicator increases, and if there is a lack of coagulation factors, it does not change.
• Clotting time is determined by the formation of a blood clot in a sample of the patient's venous blood. The fewer coagulation factors in the blood, the longer the clotting time.
• The pinch test allows you to assess the degree of occurrence of subcutaneous effusions when squeezing a skin fold in the subclavian region. In this area, hemorrhage appears only with vascular and platelet disorders.
• The tourniquet test is similar to the previous one and consists of applying a tourniquet to the patient’s shoulder area (for about 5 minutes). When the disease occurs, the forearm becomes covered with pinpoint hemorrhages.
• The cuff test involves placing a cuff from a blood pressure monitor. The doctor pumps the air up to about 100 mmHg. Art. and saves it for 5 minutes. Next, hemorrhages on the patient’s forearm are assessed.
• Differential diagnosis consists of consulting other specialized specialists - for example, a therapist, allergist, neurologist, infectious disease specialist, etc.

Treatment of hemorrhagic diathesis

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

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

To urgently stop bleeding that has started, use:

• applying a tourniquet;
• tamponade of cavities;
• tight bandage;
• applying a cold heating pad or ice to the bleeding area.

Surgical treatment:

• Surgery to remove the spleen (splenectomy) is performed if there is significant bleeding. This intervention allows you to increase the lifespan of blood cells;
• surgery to remove diseased vessels that were sources of recurring bleeding. If necessary, vascular prosthetics are performed;
• puncture of the joint capsule with subsequent suction of accumulated blood;
• surgery to replace a joint with an artificial one if irreversible changes have occurred that cannot be treated.

Blood transfusion therapy (transfusion of donor blood):

• transfusion of plasma containing all coagulation factors (fresh frozen preparation) allows you to restore the level of all factors, as well as support the patient’s immune defense;
• platelet transfusion;
• transfusion of red blood cells (sometimes washed red blood cells are used instead of this drug, which reduces the severity of side effects during transfusion). It is used in extreme cases - for example, with severe anemia and anemic coma.

Physiotherapeutic treatment involves exposure to magnetic or electric fields, which promotes the resorption of blood clots and the restoration of damaged tissue.

Exercise therapy is prescribed during the period of remission to develop sufficient motor amplitude in the joints that have undergone hemorrhage.

Homeopathy: For increased degree of systemic hemorrhage and tendency to bleed, the following homeopathic remedies may help.

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

Traditional treatment of hemorrhagic diathesis

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

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

• collection with chistema, yarrow, nettle leaves, knotweed, strawberry and mantle leaves - 8 g of the collection is brewed in 400 ml of boiling water, left for a quarter of an hour, drunk 100 ml after meals three times a day;
• collection with agrimony, corn silk, viburnum inflorescences, raspberry leaves, rose hips and rowan berries - prepare as in the previous recipe;
• collection with St. John's wort, nettle, yarrow, jasmine, alder, chamomile and blackberry leaf - 4 g of raw material is brewed with 200 ml of boiling water, left for 3 hours, drunk a third of a glass 4 times a day after meals;
• periwinkle leaf, hazelnut, galangal root, blueberry leaf, viburnum berries, rowan and rose hips - use 7 g per 350 ml of boiling water for brewing, leave for 3 hours, take 1/3 cup three times a day after meals.

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

– a general name for a number of hematological syndromes that develop when one or another part of hemostasis is disrupted (platelet, vascular, plasma). Common to all hemorrhagic diathesis, regardless of their origin, are the syndrome of increased bleeding (recurrent, prolonged, intense bleeding, hemorrhages of various locations) and post-hemorrhagic anemic syndrome. Determination of the clinical form and causes of hemorrhagic diathesis is possible after a comprehensive examination of the hemostatic system - laboratory tests and functional tests. Treatment includes hemostatic, blood transfusion therapy, and local bleeding control.

General information

Hemorrhagic diathesis is a blood disease characterized by the body’s tendency to develop spontaneous or inadequate hemorrhages and bleeding factors. In total, over 300 hemorrhagic diathesis are described in the literature. The pathology is based on quantitative or qualitative defects of one or more blood clotting factors. In this case, the degree of bleeding can vary from small petechial rashes to extensive hematomas, massive external and internal bleeding.

According to approximate data, about 5 million people in the world suffer from primary hemorrhagic diathesis. Taking into account secondary hemorrhagic conditions (for example, disseminated intravascular coagulation syndrome), the prevalence of hemorrhagic diathesis is truly high. The problem of complications associated with hemorrhagic diathesis is in the field of view of various medical specialties - hematology, surgery, resuscitation, traumatology, obstetrics and gynecology, and many others. etc.

Classification of hemorrhagic diathesis

Hemorrhagic diathesis is usually distinguished depending on the violation of one or another factor of hemostasis (platelet, coagulation or vascular). This principle forms the basis of the widely used pathogenetic classification and, in accordance with it, 3 groups of hemorrhagic diathesis are distinguished: thrombocytopathy, coagulopathy and vasopathy.

Thrombocytopenia and thrombocytopathy, or hemorrhagic diathesis associated with a defect in platelet hemostasis (thrombocytopenic purpura, thrombocytopenia due to radiation sickness, leukemia, hemorrhagic aleukia; essential thrombocythemia, thrombocytopathies).

Coagulopathies, or hemorrhagic diathesis associated with a defect in coagulation hemostasis:

• with a violation of the first phase of blood coagulation - thromboplastin formation (hemophilia)
• with a violation of the second phase of blood coagulation - the conversion of prothrombin into thrombin (parahemophilia, hypoprothrombinemia, Stuart Prower disease, etc.)
• with a violation of the third phase of blood coagulation - fibrin formation (fibrinogenopathy, congenital afibrinogenemic purpura)
• with impaired fibrinolysis (DIC syndrome)
• with impaired coagulation in various phases (von Willebrand disease, etc.)

Vasopathies, or hemorrhagic diathesis associated with a defect in the vascular wall (Rendu-Osler-Weber disease, hemorrhagic vasculitis, vitamin deficiency C).

Causes of hemorrhagic diathesis

There are hereditary (primary) hemorrhagic diathesis, manifesting in childhood, and acquired, most often secondary (symptomatic). Primary forms are familial and are associated with a congenital defect or deficiency, usually of a single coagulation factor. Examples of hereditary hemorrhagic diathesis are hemophilia, Glanzmann thrombosthenia, Randu-Osler disease, Stuart Prower disease, etc. An exception is von Willebrand disease, which is a multifactorial coagulopathy caused by a violation of factor VIII, vascular factor and platelet adhesiveness.

The development of symptomatic hemorrhagic diathesis usually results from a deficiency of several hemostasis factors at once. In this case, there may be a decrease in their synthesis, an increase in consumption, a change in properties, damage to the vascular endothelium, etc. The causes of increased bleeding can be various diseases (SLE, cirrhosis of the liver, infective endocarditis), hemorrhagic fevers (dengue fever, Marburg, Ebola, Crimean, Omsk etc.), deficiency of vitamins (C, K, etc.). The group of iatrogenic causes includes long-term or inadequate dose therapy with anticoagulants and thrombolytics.

Most often, acquired hemorrhagic diathesis occurs in the form of disseminated intravascular coagulation syndrome (thrombohemorrhagic syndrome), complicating a variety of pathologies. Possible secondary development of autoimmune, neonatal, post-transfusion thrombocytopenia, hemorrhagic vasculitis, thrombocytopenic purpura, hemorrhagic syndrome with radiation sickness, leukemia, etc.

Symptoms of hemorrhagic diathesis

In the clinic of various forms of hemostasiopathy, hemorrhagic and anemic syndromes dominate. The severity of their manifestations depends on the pathogenetic form of hemorrhagic diathesis and associated disorders. With different types of hemorrhagic diathesis, different types of bleeding can develop.

Microcirculatory(capillary) type of bleeding occurs in thrombocytopathies and thrombocytopenias. It manifests itself as petechial-spotted rashes and bruises on the skin, hemorrhages in the mucous membranes, bleeding after tooth extraction, gingival, uterine, and nosebleeds. Hemorrhages can occur with minor trauma to the capillaries (when pressing on the skin, measuring blood pressure, etc.).

Hematoma This type of bleeding is characteristic of hemophilia and is possible with an overdose of anticoagulants. It is characterized by the formation of deep and painful hematomas in soft tissues, hemarthrosis, hemorrhages in the subcutaneous fat and retroperitoneal tissue. Massive hematomas lead to tissue dissection and the development of destructive complications: contractures, deforming arthrosis, pathological fractures. By origin, such bleeding can be spontaneous, post-traumatic, postoperative.

Capillary-hematoma(mixed) hemorrhages accompany the course of DIC syndrome, von Willebrand disease, and are observed when the dose of anticoagulants is exceeded. Petechial-spotted hemorrhages and soft tissue hematomas are combined.

Microangiomatous type of bleeding occurs in hemorrhagic angiomatosis, symptomatic capillaropathies. With these hemorrhagic diathesis, persistent recurrent bleeding occurs in one or two localizations (usually nasal, sometimes gastrointestinal, pulmonary, hematuria).

Vasculitic purpuric type of bleeding is observed in hemorrhagic vasculitis. It is a small-point hemorrhage, usually having a symmetrical location on the limbs and torso. After the disappearance of hemorrhages, residual pigmentation remains on the skin for a long time.

Frequent bleeding causes the development of iron deficiency anemia. The anemic syndrome that accompanies the course of hemorrhagic diathesis is characterized by weakness, pale skin, arterial hypotension, dizziness, and tachycardia. With some hemorrhagic diathesis, articular syndrome (swelling of the joint, arthralgia), abdominal syndrome (nausea, cramping pain), and renal syndrome (hematuria, lower back pain, dysuria) may develop.

Diagnostics

The purpose of diagnosing hemorrhagic diathesis is to determine its form, causes and severity of pathological changes. An examination plan for a patient with increased bleeding syndrome is drawn up by a hematologist together with the attending specialist (rheumatologist, surgeon, obstetrician-gynecologist, traumatologist, infectious disease specialist, etc.).

First of all, clinical blood and urine tests, platelet count, coagulogram, and feces for occult blood are examined. Depending on the results obtained and the expected diagnosis, advanced laboratory and instrumental diagnostics (biochemical blood tests, sternal puncture, trephine biopsy) are prescribed. For hemorrhagic diathesis of immune origin, the determination of anti-erythrocyte antibodies (Coombs test), anti-platelet antibodies, lupus anticoagulant, etc. is indicated. Additional methods may include functional tests for capillary fragility (tourniquet tests, pinch tests, cuff test, etc.), ultrasound of the kidneys, Ultrasound of the liver; radiography of joints, etc. To confirm the hereditary nature of hemorrhagic diathesis, consultation with a geneticist is recommended.

Treatment of hemorrhagic diathesis

When selecting treatment, a differentiated approach is practiced, taking into account the pathogenetic form of hemorrhagic diathesis. Thus, with increased bleeding caused by an overdose of anticoagulants and thrombolytics, discontinuation of these drugs or adjustment of their dose is indicated; prescription of vitamin K preparations (vicasol), aminocaproic acid; plasma transfusion. Therapy for autoimmune hemorrhagic diathesis is based on the use of glucocorticoids, immunosuppressants, and; if the effect of their use is unstable, splenectomy is required.

In case of hereditary deficiency of one or another coagulation factor, replacement therapy with their concentrates, transfusions of fresh frozen plasma, red blood cells, and hemostatic therapy are indicated. In order to locally stop minor bleeding, it is practiced to apply a tourniquet, a pressure bandage, a hemostatic sponge, and ice; carrying out nasal tamponade, etc. For hemarthrosis, therapeutic punctures of the joints are performed; for soft tissue hematomas - their drainage and removal of accumulated blood.

The basic principles of treatment of DIC include active elimination of the cause of this condition; cessation of intravascular coagulation, suppression of hyperfibrinolysis, hemocomponent replacement therapy, etc.

Complications and prognosis

The most common complication of hemorrhagic diathesis is iron deficiency anemia. With recurrent hemorrhages in the joints, joint stiffness may develop. Compression of the nerve trunks by massive hematomas is fraught with the occurrence of paresis and paralysis. Of particular danger are profuse internal bleeding, hemorrhages in the brain, and adrenal glands. Frequent repeated transfusion of blood products is a risk factor for the development of post-transfusion reactions, hepatitis B infection, and HIV infection.

The course and outcomes of hemorrhagic diathesis are different. With adequate pathogenetic, replacement and hemostatic therapy, the prognosis is relatively favorable. In malignant forms with uncontrolled bleeding and complications, the outcome can be fatal.

Hemorrhagic diathesis is a group of ailments of different nature and varying degrees of severity. Most of them pose a serious health threat to life and require immediate treatment

G Hemostasis is a system in the human body whose task is to maintain blood in a liquid state, remove blood clots if they appear, and stop bleeding when the walls of blood vessels are damaged. Disturbances at any stage of hemostasis result in an increased tendency to bleeding.

Features of the problem

Hemorrhagic diathesis is a consequence of a violation of any part of hemostasis. About 300 different forms of diathesis are described in the medical literature, however, with all the diversity of this disease, they all have one common feature: increased bleeding syndrome. The severity of the symptom can be very different - from small pinpoint rashes and hematomas to massive internal bleeding.

According to statistics, about 5 million inhabitants of the planet suffer from the primary form of diathesis. The prevalence of secondary forms is literally incalculable.

The causes of the disease are extremely varied, often representing a complex of various types of damage, which together lead to impaired blood clotting. The disease has no age restrictions and affects men and women equally. Complete healing is impossible in most cases, but timely, competent treatment helps to significantly reduce the severity of symptoms and eliminate the threat of massive bleeding.

Hemorrhagic diathesis in adults (photo)

Classification

Quantitative and qualitative types of diathesis are extremely numerous. They are classified in relation to the mechanism of stopping bleeding.

In a healthy body, restoration of a vessel after damage looks like this:

• blood pours into the perivascular space - into the tissue around the rupture, and thereby compresses the vessel;
• the damaged vessel spasms;
• under the influence of substances released into the blood, platelets stick together and, forming a blood clot, plug the gap;
• under the influence of hormones - norepinephrine, serotonin, adrenaline, released from destroyed platelets, the vessel contracts;
• the thrombus is “reinforced” by connective tissue;
• the walls of the vessel are healed and the vessel restores its integrity.

Excessive and repeated bleeding is caused by disturbances at some stage of recovery.

By platelets

The latter provide the creation of a mechanical barrier that stops bleeding and are a source of hormones necessary for vessel contraction. There are 2 types:

• thrombocytopenia– illnesses in which the concentration of platelets in the blood is below 150 10 9 / l. Most often, this indicates their increased destruction or insufficient synthesis;
• thrombocytopathy– the quantitative level of platelets may be normal or slightly reduced. But at the same time, the blood plates themselves are defective and do not perform their function.

Hemorrhagic diathesis in children - the topic of the video below:

By coagulopathies

Coagulation is blood clotting. This is the primary stage of hemostasis, in which the vessel narrows and the damaged area is clogged with platelets. Primary hemostasis in a healthy body takes 1–3 minutes. According to the degree of violation of this factor, group 2 of hemorrhagic diathesis is distinguished.

Hemorrhagic coagulopathies are caused by deficiencies or abnormalities of plasma coagulation factors. A typical representative of this group is hemophilia A. Most often, such ailments are “associated” with other diseases: skin diseases, kidney and liver failure.

Coagulopathies are distinguished according to the genesis of dysfunction:

• acquired– as a rule, appear against the background of liver dysfunction, poor absorption of vitamin K, when used in the treatment of certain anticoagulants, and so on. The most significant in this category is disseminated intravascular coagulation syndrome. The disease is extremely dangerous - the mortality rate is 30–60%. Its essence comes down to the coagulation of blood proteins, which leads to a blockade of microcirculation and causes thrombus formation;
• autoimmune– caused by the production of coagulation inhibitors, most often ATP to factor 4;
• genetic– different types of hemophilia, von Willebrand disease. The reason is a disruption of the genes that determine the production of coagulation factors.

Hemorrhagic diathesis in children

For vasopathies

The basis of such ailments is a violation of the vascular link of hemostasis. Just like the previous forms, they can act as an independent disease and one of the syndromes of the underlying disease. There are several classifications, but they are mainly divided according to etiological criteria, as this makes it possible to take into account the mechanism of manifestations.

The following types of vasopathies are distinguished:

• immunocomplex– hemorrhagic vasculitis, for example. The main damaging factor is the antigen-antibody immune complex. Its action damages blood vessels, which leads to ruptures of the walls;
• infectious– are complications of severe infections: typhus, rubella, influenza;
• metaplastic– a consequence of tumor diseases of the circulatory system. As a rule, it is combined with disorders in thrombus formation;
• diplastic– hereditary diseases, for example, Randu-Osler disease, Ehlers-Danlos disease. They are caused by improper development of connective tissue. The latter can form large compounds - macrohemangiomas in vital organs, which causes increased bleeding in defective areas and can lead to death. A feature of this group of ailments is the development of anemia, sometimes severe;
• dystrophic vasopathies– caused by cellular degeneration: disturbances in the nutrition of vascular cells lead to their low functionality and increased fragility of the walls. Dystrophic vasopathies are always a consequence of the underlying disease and often occur with vitamin deficiencies, especially with a lack of vitamin C and P;
• neuropathic- are neurogenic in nature. As a rule, this form occurs in patients with an altered psycho-emotional status or with very strong experiences. A classic example: bloody tears and stigmata on the hands and feet. The neurotic form is very rare in children.

According to the manifestation of hemorrhagic syndrome

There are 5 most common types of manifestations.

• Hematoma– a sign of severe pathology of the coagulation system. It manifests itself as a massive deep hemorrhage into soft tissues, into the peritoneum, into muscles, and into joints. It is accompanied by severe pain, causes deformation of joints, bones, disturbances in the functioning of organs, up to an abdominal catastrophe - peritonitis.
• Petechial spotted- bruised. Small pinpoint or spotty hemorrhages, do not separate tissues, do not cause pain, are formed due to microdamages: friction of clothing, a tight elastic band, a slight bruise. The syndrome often accompanies thrombocytopenia and thrombocytopathies.
• Mixed– bruise-hematogenous. Combines the symptoms of both syndromes described above. It is often secondary, observed when taking anticoagulants, disseminated intravascular coagulation syndrome, and liver diseases.
• Vasculitic purpurea– hemorrhages have the appearance of a rash, slightly raised above the skin, compacted and, as a rule, with a pigmented rim. Their appearance is provoked by inflammatory changes in blood vessels. Localization is very different.
• Angiomatous– observed with vascular dysplasia. A distinctive feature is persistent, repeating bleeding, clearly localized. The most dangerous and profuse are nosebleeds.

By origin

This refers to the sequence of appearance of signs. There are 2 types:

• primary– hemorrhagic diathesis arises and develops as an independent pathology;
• secondary– acts as a consequence of the underlying disease: past illnesses, especially infectious ones, sepsis, severe poisoning, and so on.

Causes of occurrence

All causes of the syndrome can be divided into 2 groups.

• Hereditary - appear in childhood, are hereditary and associated with some kind of genetic birth defect.
• Symptomatic hemorrhagic diathesis can appear at any age. As a rule, the appearance of any form is caused not by one, but by a combination of disturbances in the functioning of the elements of the blood coagulation system.

The list of main etiological factors includes:

• congenital disease;
• low platelet count;
• disorders in the blood coagulation system;
• allergy;
• severe liver and kidney disorders;
• lack of vitamin C;
• altered psycho-emotional state.
• taking certain medications.

Symptoms of hemorrhagic diathesis

The first, and sometimes the main symptom of the disease is a rash. The patient experiences small bluish spots or a rash on the forearms, on the front of the lower leg, and so on.

Most often, the rash is not accompanied by any painful phenomena. The appearance of the rash serves as the basis for one of the classifications and is an indication of the nature of the disease:

• with capillary diathesis, small red rashes and bruises are observed on the skin. With further development of the disease, bleeding may occur in the gums, nose, uterus, and stomach;
• The hematoma type of disease is indicated by deep, large hematomas, which are usually grouped in certain parts of the body and do not spread. Possible bleeding in the joints;
• with the microangimatous form, irregularly shaped burgundy spots are grouped in a certain area of ​​the body without spreading. As a rule, this indicates congenital pathologies in vascular development;
• the vasculitic type causes a medium-sized rash, more often on the legs than on the arms;
• mixed diathesis - rashes and hematomas of various types may be present.

The disease can manifest itself in an acute form - in the form of bleeding into the internal organs. In this case, diathesis is accompanied by anemia. As a rule, patients seek consultation not when they notice hematomas of unknown origin, but only when there is unexpectedly heavy bleeding against the background of a minor injury. Often, when making a diagnosis, it turns out that some signs of pathology have been observed since childhood.

The appearance of certain signs in children most often indicates the hereditary nature of the disease. The prevalence of diathesis is very high, its symptoms are well studied.

Signs of a violation of the coagulation system in children are as follows:

• When cutting through and changing teeth, there is a lot of bleeding from the gums;
• hemorrhagic rashes appear;
• nosebleeds for no reason are a very common symptom;
• joint pain caused by hemorrhage into the joint capsule and its deformation;
• hemorrhage in the retina;
• the appearance of menorrhagia in girls - too long periods.

Hemorrhagic diathesis can be observed in newborns as early as 2–3 days. These are bleeding of the umbilical wound, oral cavity and nasopharynx, bloody vomiting and bowel movements, which indicate gastrointestinal bleeding. In such cases, treatment begins immediately.

Diagnostics

Considering the diversity of the form of the disease and the equally diverse causes that cause it, it is quite difficult to establish an accurate diagnosis and the true cause of the syndrome. First of all, they try to establish the nature of the diathesis - hereditary or acquired, and then more accurately study its nature.

In general, diagnosis of this disease is carried out according to this scheme.

1. First of all, the current condition of the patient is assessed: the presence of bad habits, chronic diseases, hereditary ailments, it is clarified what medications were taken, and so on.
2. During a general examination, the condition of the skin is assessed: cyanosis, the number of rashes, hematomas, and also the pain and mobility of the joints, the state of the pulse and pressure are determined.
3. Laboratory tests are prescribed:
   • first of all, this is a blood test, in particular, an assessment of the number of red blood cells, leukocytes and platelets;
   • a urine test is prescribed to determine the level of red blood cells, and a stool test for occult blood;
   • – assessment of cholesterol, sugar, gamma globulin levels. The main part of the examination is to assess the rate of blood clotting - coagulogram, and determine the level of prothrombin. Coagulation time is determined by the rate of clot formation in venous blood: the lower the rate, the fewer clotting factors.
4. Tests are performed to determine the fragility of blood vessels:
   • pinch test - skin yielding in the subclavian fold. Hemorrhage in this area is possible only with vascular disorders;
   • tourniquet test - a tourniquet is applied to the shoulder for 5 minutes. With hemorrhagic diathesis, pinpoint hemorrhages appear on the forearm;
   • test with a cuff - a cuff from a pressure measuring device is attached to the forearm and inflated with air to 100 mm Hg. Art. Hold the cuff for 5 minutes. With diathesis, small hemorrhages appear on the skin.
5. Instrumental examination methods are usually prescribed:
   • Ultrasound of the liver and, since lesions of the latter are often associated with the appearance of hemorrhagic syndrome;
   • – to assess the condition of bones and joints;
   • bone marrow examination.

The final diagnosis can only be established using differential diagnosis, that is, after consultation with specialists of another profile. Without this, it is impossible to find the true cause of the acquired diathesis. The list of specialists includes a rheumatologist, a gynecologist, a traumatologist, a pediatrician, a surgeon, a specialist in infectious diseases, and a geneticist if the hereditary nature of the disease is suspected.

Treatment

Treatment of patients with such a diagnosis is determined by the mechanism of its occurrence and the nature of the primary disease, if we are not talking about a hereditary factor. Treatment is always individual.

In general, its tasks are:

• stopping bleeding, if observed;
• elimination of damaging factors - external or internal;
• the use of medications or surgical measures to eliminate the cause of low coagulation;
• blood transfusion, if necessary, treatment of anemia;
• special gymnastics to restore joint function.

By medication

Medicines used in the treatment of diathesis must solve the following problems:

• increase the number of platelets in case of their shortage or insufficient functionality;
• improve blood clotting, that is, ensure the action of coagulation factors;
• increase the tone of the walls of blood vessels and reduce their permeability.

In relatively simple cases, treatment has only one goal. Unfortunately, combined types of diathesis are more common, so the treatment of one disease includes drugs aimed at achieving different goals.

• Glucocorticoid drugs - hydrocortisone, prednisone, and others. The choice is determined by the nature of the disease: for example, glucocorticoids of medium duration of action are prescribed for long-term therapy, and for thrombocytopenic purpura, for example, pulse therapy is recommended, so the course includes short-acting drugs - cortisone, for example. Medicines in this group are prescribed to both adults and children, but taking into account the condition and age.
• Clotting factors - that is, drugs that can act as clotting factors: thrombing, pachycarpine, gluconate and calcium hydrochloride,.
• Vitamin complexes, including vitamins C and P, since the latter are an indispensable participant in the restoration of vascular walls.

The list of drugs used is much wider, but the treatment of almost every case is individual. There is no universal solution, so taking any medicine from these groups, excluding vitamins, can only be done as prescribed by a doctor.

Other methods

• In case of massive bleeding, especially internal bleeding, surgical intervention is used. This decision is forced and is used in case of a sudden acute attack of the disease or in the absence of effective therapy.
• Removing the spleen - this way it is possible to prolong the life of blood cells. Surgery is allowed only in case of massive bleeding, which cannot be prevented in any other way.
• Excision of damaged vessels - in cases where large vessels are damaged and recurrent bleeding cannot be stopped, these areas are removed. If necessary, prosthetics are performed in order to restore normal blood circulation;
• Puncture of the joint capsule is, in fact, not the treatment of the disease, but the elimination of its consequences. In this case, blood is suctioned from the bag.
• The same can be said about joint replacement - if the deformity is irreversible, the joints can be replaced with artificial ones.

Surgical methods are more effective in the acquired form of the disease. For congenital diseases, another method is used - blood transfusion. Blood transfusion therapy is also prescribed in cases of severe anemia.

There are 3 methods:

• plasma transfusion– it contains all coagulation factors, which allows you to quickly restore the coagulation system and support immunity;
• platelet transfusion– a solution for thrombopenia, for example, and other cases where diathesis is associated with an insufficient number of platelets;
• red blood cell transfusion– used in extreme cases: with anemic coma, very severe anemia. Transfusion is fraught with side effects, so they try to avoid it.

Nursing care

As a rule, nursing care includes carrying out doctor’s orders - medical and hygienic procedures, collecting material for analysis - urine, blood, feces, and timely registration of test results so that the attending physician can always assess the latest data on the patient’s condition.

However, with hemorrhagic diathesis, one often has to resort to a procedure such as blood transfusion - direct or indirect. Caring for a patient during and after a transfusion is also an element of nursing care and requires certain knowledge.

Before transfusion, a number of actions are performed:

• determines blood group and Rh factor;
• a test is carried out to determine the compatibility of donor blood and the patient’s plasma using a glass reaction;
• a biological compatibility test is carried out.

The doctor carries out the tests, and the nurse prepares the material for analysis. Blood from a vein is taken for compatibility testing one day before the intended transfusion. A urine test and a complete blood count are also performed.

The transfusion process itself is carried out like this.

1. Preparing the patient - 2 hours before the procedure, stop eating, only sweet tea is allowed. Before the actual transfusion, the temperature and pressure are measured and the bladder is emptied, saving the urine.
2. The blood required for transfusion is removed from the refrigerator and warmed at room temperature for no more than 1 hour. Blood is transfused from the same container in which it was stored.
3. Before filling the system, the formed elements and plasma are mixed.
4. A needle is inserted into the vein and connected to the system. Transfusion begins with a test for biological compatibility. To do this, blood is injected in a stream for 20–30 seconds. Then turn off the supply and observe the patient’s condition for 3–5 minutes. If there is no change in pulse, pain, nausea, or dizziness, the test is repeated: rapid injection for 20–30 s and observation for 5 minutes.
5. If after the third repetition of the test no such negative reactions are observed, you can proceed with the transfusion. The infusion is completed when 5–10 ml of blood remains in the container. The remaining blood is stored in the refrigerator for a day so that it can be analyzed if complications arise within 24 hours after the transfusion. Then remove the sticker from the container, dry it and paste it into the patient’s chart.
6. After the procedure, the patient must remain in bed for 2 hours. The first urine after transfusion is stored, diuresis is measured and sent for analysis.
7. After 2 hours the temperature is measured. If it is elevated, then measure every hour for 4 hours. The patient’s complaints must be recorded - they may be the first sign of complications.

For patients with hemophilia, a direct transfusion is required - from a donor, since the stabilizer found in frozen blood reduces the ability to clot. This transfusion is carried out using special systems that prevent the reverse flow of blood.

Prevention of disease

If we are talking about a hereditary disease, then no preventive measures will help. The only way to prevent the occurrence of such a disease in children is to plan pregnancy taking into account the heredity of both spouses.

However, hemorrhagic diathesis can also be acquired. In this state of affairs, preventive measures turn out to be quite effective.

• Standard examples of general strengthening of the body have a remarkable effect on the condition of blood vessels. Strong sports activities, physical exercise, good nutrition, hardening - from rubdowns to cold baths, all this has a great effect on hemodynamics.
• Taking vitamins – in this case, vitamin C and K are most important.
• Maintaining hygiene - with any form of hemorrhagic diathesis, the risk of contracting infectious diseases significantly increases. Following hygiene rules prevents the development of infection.
• Treatment of inflammatory diseases - foci of inflammation are constant sources of toxins that sooner or later destroy the immune system.
• Limiting time spent in the sun - the skin with this disease is very sensitive to ultraviolet radiation, which provokes hemorrhages.

Complications

• The most common complication of the disease is iron deficiency anemia. With frequent and heavy blood loss, it is not possible to restore hemoglobin naturally; transfusions or special iron supplements are necessary.
• With repeated hemorrhages into the joint capsule, joint stiffness occurs.
• Compression of nerve trunks by hematomas leads to the formation of and.
• The most dangerous complication is the adrenal glands.

The treatment itself can also be a source of complications: with transfusion there is a risk of contracting HIV infection and hepatitis.

Forecast

When such a diagnosis is made, the patient must register with hematology in his locality, as well as in the regional hemophilia center. It is necessary to periodically check the coagulation system and provide this information to health workers in cases where procedures and treatments are carried out that are potentially dangerous for such patients - surgical procedures, dental procedures.

It is necessary for the patient’s relatives to learn how to provide first aid for hemorrhagic diathesis. The prognosis depends on too many factors. With timely and adequate treatment, the prognosis is favorable in most cases. In severe cases, if it is impossible to control the strength and frequency of hemorrhages, death is possible.

Hemorrhagic diathesis is a dangerous and serious disease, despite seemingly minor changes and damage. There are many types of this disease and most of them pose a serious threat.

Hemorrhagic and other types of diathesis are discussed in this video by a pediatrician:

Hemorrhagic diathesis is a large group of diseases, the main symptom of which is increased bleeding. Bleeding can occur on its own or after some provoking factor (trauma, infection, allergy, etc.). Currently, more than 300 hemorrhagic diathesis are known. The cause and mechanism of occurrence, clinical manifestations of these diseases are different, and we will consider them in this article.

Classification

There are several classifications of hemorrhagic diathesis, since they are associated with three factors: changes in the number of platelets (blood platelets involved in blood clotting), damage to the vascular wall and disorders in the blood coagulation system.

1. Hemorrhagic diathesis depending on disorders of platelet hemostasis:

• with a reduced number of platelets (thrombocytopenia), characteristic of thrombocytopenic purpura, hemorrhage due to radiation sickness, leukemia;
• with platelet defects, disruption of their activity (thrombocytopathy): functional thrombocytopathy;
• combined disorders (both quantity and quality of platelets).

1. Hemorrhagic diathesis, depending on disorders in the blood coagulation system:

• congenital (hemophilia A, B, C);
• acquired (as a result of the action of certain medications).

1. Hemorrhagic diathesis associated with both coagulation system disorders and platelet pathology: hemorrhages in leukemia, high radiation, von Willebrand disease.

1. Hemorrhagic diathesis associated with disorders of the vascular wall:

• congenital: Rendu-Osler disease, osteogenesis imperfecta disease, Marfan syndrome, etc.

• acquired: hemorrhagic vasculitis, hemorrhages due to vitamin C deficiency, viral infections, Kaposi's sarcoma, amyloidosis.

There are primary hemorrhagic diathesis (independent diseases) and secondary (developed as a symptom of a malignant disease, allergic reaction, infectious process).

Primary diathesis, as a rule, is hereditary in nature (von Willebrand disease, hemophilia, Randu-Osler disease) or develops as a consequence (hemorrhagic vasculitis).

A separate group consists of functional thrombocytopathies in children - transient diathesis due to the functional immaturity of platelets, which disappears with age (after puberty). They are quite common - observed in 5-10% of children. Only special blood tests can distinguish them from other types of diathesis.

Causes

Increased bleeding can be congenital or acquired.

Congenital or genetically inherited bleeding is associated with the lack of formation of blood clotting factors VIII or IX in the body. This disease (hemophilia) is transmitted from a sick father to his daughter through a defective X chromosome, but the daughter remains a healthy carrier of the defective gene and passes it on to her sons.

Reasons for acquired There is a lot of increased bleeding:

• toxic effects of certain drugs;
• viral and bacterial infections (hemorrhagic fever, etc.);
• allergic reactions;
• autoimmune processes (the body begins to produce antibodies against its own platelets or blood vessel wall cells);
• blood diseases (the process of platelet formation is disrupted, or increased destruction occurs);
• vascular diseases (vasculitis);
• increased radiation.

Symptoms

Children with hemorrhagic diathesis may experience repeated nosebleeds.

The main (and very often the only) symptom of all hemorrhagic diathesis is increased bleeding: the appearance of hematomas, “bruises”, and small hemorrhages in the form of a rash on the skin; bleeding is spontaneous or inappropriate to the injuries received (bleeding from the gums, nose, minor scratches, etc.). The location, nature of bleeding and hemorrhage depend on the type of disease.

With hemorrhagic diathesis, there are 5 types of bleeding:

• Capillary bleeding: small red dots on the skin and mucous membranes and “oozing” bleeding (gastrointestinal). Such bleeding is typical for thrombocytopenia and thrombocytopathy, as well as for disorders in the coagulation system: deficiency of fibrinogen and prothrombin complex.
• Hematoma bleeding: manifested by the occurrence of subcutaneous and internal hemorrhages; characteristic of hereditary bleeding disorders () and acquired similar disorders (in case of drug overdose -).
• Mixed type: possible with hemophilia, leukemia, overdose, von Willebrand disease, disorders of the number and functions of platelets. May present with signs of both previous types of bleeding.
• Purple bleeding: the appearance of a small-spotted, symmetrically located rash with a tendency to merge into larger spots; appears at .
• Microangiomatous bleeding: the appearance of repeated capillary bleeding with the same localization; noted in Rendu-Osler disease, i.e. with hereditary pathology of blood vessels.

Other symptoms of bleeding diathesis are associated with or result from bleeding (or bleeding). Thus, joint pain or abdominal pain is associated with the appearance of a rash on the mucous membrane of the digestive organs or inside the joint.

Intra-articular hemorrhages in hemophilia can cause disability for the patient.

Treatment

Treatment of hemorrhagic diathesis depends on its type, that is, on the specific cause that caused increased bleeding, and on the severity of the disease.

The first priority is to stop the bleeding. For this purpose, drugs that increase blood clotting (Fibrinogen, Vikasol, etc.) are used. Thrombin, fibrin film, hemostatic sponge, etc. are used locally. In case of massive blood loss, plasma or whole blood transfusion is used.

If diathesis is associated with damage to the vascular wall, the child is prescribed vitamins PP, C and rutin. If the bleeding is profuse and prone to frequent recurrences, surgical treatment (removal of the spleen) is sometimes used. In severe cases of vasculitis or Werlhoff's disease, it is used in age-specific doses.

For secondary diathesis, symptomatic treatment is carried out together with treatment of the disease that caused the diathesis. For thrombocytopathies, drugs are used that improve metabolic processes in the body and promote platelet maturation. Patients need to be provided with adequate nutrition and gentle conditions that protect them from injury (in case of genetic diathesis).