Hemorrhagic diathesis

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

ICD 10 code

• D 69 – purpura and other hemorrhagic phenomena;
• D 69.0 – allergic purpura;
• D 69.1 – platelet quality defects;
• D 69.2 – other purpura of nonthrombocytopenic etiology;
• D 69.3 – idiopathic thrombocytopenic purpura;
• 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, the following types of hemorrhagic diathesis are distinguished:

• diathesis caused by a disorder of platelet hemostasis. This type includes such pathologies as thrombocytopathy and thrombocytopenia. Development factors may include decreased immunity, kidney and liver diseases, viral lesions, chemotherapy treatment and radiation exposure;
• a disease caused by disturbances in blood clotting processes – this could be a fibrinolysis disorder, 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 angiectasia or vasculitis;
• a disease caused by platelet hemostasis – von Willebrand disease, thrombohemorrhagic syndrome, radiation sickness, hemoblastoses, etc.

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

• hemorrhages caused by blood clotting disorders;
• 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 damage to blood vessels.

In a normal healthy organism, the vessel wall may be partially permeable to gaseous and soluble substances. As a rule, the wall is impermeable to blood elements and proteins. If its integrity is compromised, a complex process of coagulation 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 - increased permeability of the vessel wall and disorder of the coagulation process. Sometimes it is also hypothesized that the mechanism of hemorrhage can be triggered by an increase in the ability of red blood cells to pass through the vascular barrier with slight blood thinning, 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.

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Symptoms of hemorrhagic diathesis

The first signs of hemorrhagic diathesis are most often 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, ulcers are formed. Sometimes this condition can be accompanied by pain in the epigastric region, bouts of vomiting with elements of blood in the vomit.

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

Most often, patients seek medical attention due to severe bleeding, for example, after a minor injury. However, bleeding can also be spontaneous, not depending on the presence of direct tissue damage.

A thorough questioning of the patient may reveal that the symptoms of hemorrhagic diathesis have haunted him since childhood. In some cases, similar symptoms also bothered the relatives of the sick person (if the disease has a hereditary etiology).

Hemorrhagic diathesis in children

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

The disease in a child may manifest itself in the following ways:

• persistent bleeding of the gums is observed during the period of teething or changing teeth;
• often there are unexplained nosebleeds;
• 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 observed periodically;
• Girls experience menorrhagia.

In newborns, the disease can manifest itself as early as the second or third day of life. The most common symptoms include gastrointestinal bleeding, which is detected in the form of bloody stool and vomiting. These signs can occur both separately and simultaneously. In addition, bleeding is observed from the umbilical wound, from the mucous membrane of the oral cavity and nasopharynx, 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, therefore it 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 detachment of a normally located placenta, bleeding during and after childbirth. Such complications can be encountered in almost 5% of cases of diseases in pregnant women.

The 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 detected in approximately 2-4% of cases.

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

Classification of hemorrhagic diathesis

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

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

The most common type of hemorrhage is the vascular-purple type. In this disease, small hemorrhages can be observed on the surface of the skin where tissue is often compressed, for example, when using a belt, when wearing tight elastic bands in clothing, and also on the buttocks (a place where people often sit).

In addition, the following are distinguished:

• 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 clotting system and the condition of the vascular walls.

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

Patients with hemorrhagic diathesis belong to a high-risk group due to the likelihood of infection with the hepatitis B virus and HIV infection (with frequent blood transfusions).

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

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

The greatest danger comes from internal bleeding that is invisible to the eye, especially in various parts of the brain and the adrenal glands.

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Diagnosis of hemorrhagic diathesis

Evaluation of the medical history and collection of patient complaints: when the first bleeding was detected, whether there was a feeling of weakness and other signs of diathesis; how the patient himself explains the appearance of such symptoms.

• Assessment of life history: presence of chronic diseases, long-term use of medications, heredity, presence of bad habits, oncological history, intoxication.
• External examination of the patient: skin tone and condition (pale, cyanotic, hyperemic, with rashes or hemorrhages), enlargement, soreness and mobility of joints, pulse and blood pressure.
• Blood tests: a decrease in the number of red blood cells, a decrease in the hemoglobin level are determined. The color index is normal, the number of leukocytes and platelets in different patients may differ and even not go beyond the normal values.
• Urine tests: erythrocyturia (hematuria) is determined in the presence of hemorrhages in the kidneys or urinary system.
• Biochemical blood test: evaluates the amount of fibrinogen, alpha and gamma globulins, cholesterol, sugar, creatinine, uric acid, as well as electrolytic metabolism indicators to monitor the general condition of the body. Coagulogram, evaluation of antihemophilic factors.
• Instrumental diagnostics:
  • examination of bone marrow material removed during a puncture of the bone (usually the sternum). It is carried out in order to evaluate the hematopoietic function;
  • trephine biopsy is a study that is performed on a sample of bone marrow and a bone element with a part of the periosteum, most often taken from the ilium. A specific device, a trephine, is used for this. It is prescribed to assess the condition of the bone marrow.
• To determine the duration of bleeding, a method of pricking the upper finger phalanx or earlobe is used. If the function of the vessels or platelets is impaired, the duration indicator increases, and if there is a lack of coagulation factors, it does not change.
• The clotting time is determined by the formation of a blood clot in a sample of the patient's venous blood. The fewer clotting factors in the blood, the longer the clotting time.
• The pinch test allows one to assess the degree of occurrence of subcutaneous effusions when squeezing the skin fold in the subclavian region. In this area, hemorrhage appears only with vascular and thrombocytic 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 measuring device. The doctor pumps air to about 100 mm Hg and holds it for 5 minutes. The bleeding on the patient's forearm is then assessed.
• Differential diagnostics involves consultation with other specialized specialists, such as a therapist, allergist, neurologist, infectious disease specialist, etc.

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Treatment of hemorrhagic diathesis

Drug treatment is prescribed individually (different 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) when the number of platelets is reduced. Most often, prednisolone is used at 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 clotting factors, if there is a deficiency of them.

To urgently stop bleeding that has begun, use:

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

Surgical treatment:

• Spleen removal surgery (splenectomy) is performed when there is significant bleeding. This intervention can increase the lifespan of blood cells;
• an operation to remove the affected vessels that were the source of recurring bleeding. If necessary, vascular prosthetics are performed;
• puncture of the joint capsule followed by suction of accumulated blood;
• surgery to replace the joint with an artificial one if irreversible changes have occurred that cannot be treated.

Hemotransfusion therapy (transfusions of donor blood products):

• transfusion of plasma containing all clotting factors (fresh frozen preparation) allows to restore the level of all factors, as well as to 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, in severe anemia and anemic coma.

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

Physical therapy is prescribed during the period of remission to develop sufficient range of motion in the joints that have been subject to bleeding.

Homeopathy: In cases of increased systemic hemorrhage and bleeding tendency, the following homeopathic remedies may help.

• Phosphorus. Also used for coagulopathy, hemorrhagic fever, vitamin C deficiency.
• Lachessis, Crotalus. Can be used in hemorrhagic vasculitis and hemophilia.
• Arsenicum album. Most often combined with the above-mentioned 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.

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

• a collection with stachys, yarrow, nettle leaves, knotweed, strawberry leaves and alchemilla - 8 g of the collection is brewed in 400 ml of boiling water, infused for a quarter of an hour, drink 100 ml after meals three times a day;
• a collection of agrimony, corn silk, viburnum inflorescences, raspberry leaves, rose hips and rowan berries - prepare as in the previous recipe;
• a collection with St. John's wort, nettle, yarrow, deadnettle, alder, chamomile flowers and blackberry leaves - 4 g of raw materials are brewed with 200 ml of boiling water, infused for 3 hours, and 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 for brewing 7 g per 350 ml of boiling water, 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.

If the condition improves steadily, use plants such as periwinkle leaf, lady's mantle, knotweed, St. John's wort, corn silk and rowan berries - the components can be replaced with each new course of treatment.

In the acute period, it is important to stay in bed for at least 3 weeks.

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Prevention of hemorrhagic diathesis

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

Such activities include:

• strengthening the body's defenses (includes engaging in feasible sports, performing regular physical exercises, hardening the body, and proper nutrition);
• additional intake of vitamin supplements (in particular, vitamin K – for example, in the form of Menadione or Sinkavit);
• strict adherence to hygiene rules is a simple recommendation that helps prevent the development of infectious diseases that can give rise to pathological processes in the body;
• sanitation of foci of chronic infection in the body, timely treatment of inflammatory diseases;
• dispensary observation, regular visits to the doctor;
• avoiding sun exposure.

Secondary prevention consists of periodic medical examinations of the population for the early detection of the disease.

Prognosis of hemorrhagic diathesis

Patients with hemorrhagic diathesis should be registered with the hematology department in their home town, as well as in the regional hemophilia center. Patients undergo regular assessment of the hematopoietic and coagulation systems, provide information to both the patient and health workers at their place of residence, and (if necessary) carry out potentially dangerous types of treatment for such patients - provide dental and surgical care. Relatives and friends of patients also receive instructions and are trained in the methods of providing first aid to patients with hemorrhagic diathesis.

The outcome and prognosis of the disease may vary. If the patient has undergone adequate pathogenetic, hemostatic and replacement treatment, the prognosis can be considered quite favorable.

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