Hemorrhagic syndrome - a set of pathological conditions, accompanied by a single clinical symptom complex, manifested in increased bleeding and differing polyethiologic origin. In terms of clinical and laboratory diagnosis and the prognosis regarding the patient's health, a crucial difference is the chronic and acute form of the course of hemorrhagic syndrome. Clinical and laboratory manifestations of hemorrhagic syndrome can "start" at any age, from the period of the newborn to the elderly.
Causes of hemorrhagic syndrome
All pathogenetic forms of hemorrhagic syndrome are divided into two large categories (primary or hereditary, secondary or acquired) depending on the prevailing etiologic factor, which is the cause of the development of pathological hemorrhage.
Hereditary forms of a hemorrhagic syndrome can develop on several pathogenetic mechanisms, however for all these conditions presence of genetic determination (presence of the defective gene) is characteristic. Genetic determination can affect all parts of the coagulation system, that is, the development of hereditary hemorrhagic syndrome can be due to the pathology of platelet blood cells, clotting factors or the vascular wall.
Secondary or acquired hemorrhagic syndrome most often develops as a result of pathological changes in the wall of vessels of different caliber, resulting from inflammatory, mechanical, autoimmune or toxic effects. Secondary thrombocytopathy , as one of the etiopathogenetic variants of hemorrhagic syndrome, can be provoked by prolonged use of drugs of certain pharmacological groups (eg, disaggregants), as well as as a result of dismetabolic disorders in the body.
The decreased content of platelet blood cells also provokes the development of hemorrhagic syndrome and most often develops as a result of disturbance of the processes of normal hematopoiesis in the red bone marrow or their increased destruction, which occurs in DIC syndrome , thrombocytopenic purpura and systemic lupus erythematosus .
Coagulopathy in the form of hypocoagulation manifests itself in the development of hemorrhagic syndrome and develops most often as congenital pathology ( hemophilia , Willebrand disease ). Pathogenetic mechanisms of development of this form of hemorrhagic syndrome is a significant decrease in the production of plasma factors and an increase in the activity of factors of the fibrinolytic system.
Primary vasopathies mainly provoke a hemorrhagic syndrome in children during puberty and the basis for their development is a pathological increase in the permeability of the vessel wall and the formation of telangiectasias (limited vascular wall enlargements).
It should be borne in mind that pathological bleeding can manifest not only on the skin but also mucous membranes, and as an example is hemorrhagic uterine syndrome, which is observed in neoplasms of benign and malignant nature.
Symptoms of hemorrhagic syndrome
The most frequent clinical variant of pathological hemorrhage is cutaneous hemorrhagic syndrome, which can differ significantly in intensity and morphological type of cutaneous elements. Thus, the most severe with regard to patient tolerance is the hematoma variant, which is observed in hemophilia and is manifested by the development of massive outflow and blood accumulation in intermuscular spaces and articular bags accompanied by severe pain syndrome. The pathognomonic symptom of the haematomic variant of the hemorrhagic syndrome is a prolonged significant limitation of the mobility of one or a whole group of joints.
The emergence of hemorrhages occurs, usually after some traumatic effect, and with severe hemophilia, hematomas and hemarthroses appear spontaneously against a background of complete well-being. The most pathognomonic sign of the hematoma variant of the hemorrhagic syndrome is the appearance of signs of intra-articular hemorrhages, with large groups of joints of the upper and lower extremities suffering more. Clinical criteria of hemarthrosis in hemorrhagic syndrome is a sharp intense pain syndrome, swelling of periarticular soft tissues and inability to perform the usual motor movements. In a situation where a large volume of fresh blood accumulates in the joint bag, a positive symptom of fluctuations can be observed. Consequences of such changes in hemorrhagic hematoma type syndrome is the development of chronic infectious damage to synovial membranes and the destruction of cartilaginous tissue, which inevitably leads to the development of deforming arthrosis.
When a pathological change in the number or shape of platelet cells most often develop a petechial-spotted hemorrhagic syndrome. With this pathological condition, the patient has a tendency to develop superficial intradermal hemorrhages, even with minimal traumatic effect on the skin (for example, mechanical compression of the skin with a cuff while measuring blood pressure). Petechial intracutaneous hemorrhage, as a rule, is small, not exceeding three millimeters of intense red color, which does not disappear upon palpation. For this variant of hemorrhagic syndrome, the appearance of hemorrhage in the mucous membranes of various locations, especially in the conjunctiva of the eye. In the petechial-spotted variant of hemorrhagic syndrome observed in the disease of Verlhof , there is a tendency to develop persistent nosebleeds that provoke anemia of the organism.
With a mixed bruise-hematoma variant, the patient develops both intradermal hemorrhages and intermuscular hematomas. A fundamental difference between this form of hemorrhagic syndrome and the hematoma variant is the absence of intra-articular hemorrhages.
With hemorrhagic vasculitis , as a variant of hemorrhagic syndrome, development of cutaneous hemorrhages in the form of purpura is noted. Hemorrhagic elements of the rash are formed on the inflammatory-altered areas of the skin, so they always slightly rise above the surface of the skin and have well-defined pigmented contours. The principal difference between hemorrhagic rash in vasculitis is persistent pigmentation of the skin even after leveling out acute manifestations of hemorrhagic syndrome.
The most specific in terms of clinical manifestations is angiomatous hemorrhagic syndrome, the occurrence of which is due to an anatomical change in the vascular wall. For this form of hemorrhagic syndrome is characterized by bleeding of a certain localization, for example, from the nasal cavity in the Rundu-Osler syndrome. In the angiomatous variant of hemorrhagic syndrome, intradermal and subcutaneous hemorrhages are never observed.
Since the acquired hemorrhagic syndrome develops against the background of any diseases or pathological conditions, the classical picture can be supplemented with nonspecific symptoms, characteristic for background pathology.
Qualitative diagnosis of hemorrhagic syndrome involves the evaluation of clinical, laboratory and instrumental data. The fundamental link in the diagnostic search is the careful collection of anamnestic data and a careful examination of the patient with the definition of localization, the nature of skin manifestations.
Hemorrhagic syndrome in newborns
It should be borne in mind that the child after birth noted the imperfection of virtually all organs and systems in the body, including the mechanisms of hematopoiesis. All infants have a tendency to increase the permeability of the vascular wall of the capillary network, as well as the immaturity of the processes and coagulation factors, which are extremely functional and do not provoke the development of classical hemorrhagic syndrome. In a situation where a child develops an acute hemorrhagic syndrome after birth in a short time, the genetic nature of the pathology should first be assumed. Indirect predisposing factors in the development of hemorrhagic syndrome is the long-term intake of medicines of the salicylate group by the mother during pregnancy, intrauterine fetal hypoxia and prematurity.
In the last decade, a prophylactic application of vitamin K in a minimal dosage of 1 mg is widely used in neonatological practice, which makes it possible to significantly reduce the incidence of hemorrhagic syndrome among children of the neonatal period. It is noteworthy that the clinical picture of hemorrhagic syndrome does not develop immediately after delivery, but after the seventh day when the child is already discharged from the hospital, the main task of the pediatrician who observes the child after birth is to adequately assess the severity of the child's condition and determine in a timely manner the cause of the onset of this pathological condition.
Classic clinical criteria for the development of hemorrhagic syndrome in a newborn child is the appearance of blood admixtures in feces or vaginal bleeding, prolonged healing of the umbilical wound and the discharge of blood from it, a change in the color of urine that becomes brownish-red and the discharge of fresh blood from the nasal passages.
In the long-term (several weeks after birth) hemorrhagic syndrome can develop as a result of severe dysbiosis and manifest as symptoms of an intracerebral hemorrhage, which has an extremely difficult course.
Treatment of hemorrhagic syndrome
The volume and method of treatment for each form of hemorrhagic syndrome can differ significantly, therefore, in determining the tactics of patient management, the pathogenetic variant of the course of this pathology is the determining factor.
If we consider hemophilia, it should be borne in mind that the only pathogenetically justified method of treatment is the transfusion of blood components, which is a variant of substitution therapy. Due to the fact that most clotting factors are very labile and do not retain their properties in blood preservation and production of dry plasma, direct blood transfusion to a patient from a donor is a priority.
Factor VIII has a short elimination period, therefore, adequate transfusion therapy is the transfusion of cryoprecipitate and plasma at least three times a day in a single calculated dose of 10 ml per 1 kg of recipient weight. It is inadmissible to mix antihemophilic agents with any infusion solutions, and the drug should be injected exclusively intravenously, as drip infusion does not allow to increase the level of factor VIII in the plasma.
If a patient has a combination of hemarthrosis with a marked accumulation of blood in the intermuscular spaces, it is necessary to use more intensive therapy with anti-hemophilic agents. With existing external bleeding, it is enough to use local blood-thinning drugs (Thromboplastin solution). The presence of signs of acute hemarthrosis is the basis for the application of puncture of the joint bag and blood aspiration with the subsequent intra-articular injection of Hydrocortisone. During the period of remission, it is necessary to perform exercises of physiotherapy exercises, the action of which is aimed at restoring the motor function of the joint and preventing the development of atrophic changes in the muscular mass. Surgical intervention is used only as a last resort in the formation of ankylosis and the extreme stage of osteoarthritis. In this situation, operational aids such as synovectomy and bone tissue distraction are used).
With hemorrhagic syndrome, which proceeds according to the type of coagulopathy, the basic method of therapy is the parenteral administration of Vikasol in a daily dose of 30 mg. In a situation where the hemorrhagic syndrome develops against the background of prolonged use of anticoagulants of indirect action in a high dosage, an obligatory condition is their abolition. In severe coagulopathy, substitution therapy with freshly frozen plasma intravenously is used at a calculated dose of 20 ml per 1 kg of the weight of a patient with a somatotropic hormone at a dose of 4 IU per day, the effect of which is aimed at improving the synthesis of clotting factors.
DIC-syndrome is the most difficult and difficult to eliminate form of hemorrhagic syndrome and the level of mortality in the acute period of DIC syndrome is more than 60%. The drug of choice for hemorrhagic syndrome caused by DIC syndrome is Heparin in the stage of hypercoagulation at an initial dose of 10,000 units and the subsequent transition to subcutaneous administration in a single dose of 5000 units. With massive hemorrhage observed in the hypocoagulation stage, patients are shown to administer high doses of Contrikal intravenously dropwise to 10,000 units.
When thrombocytopenic purpura pathogenetically justified is the appointment of glucocorticosteroid drugs, as well as surgical intervention in the form of splenectomy. With the complete absence of a positive effect on the treatment used, it is necessary to prescribe cytotoxic drugs of immunosuppressive action. The daily dose of Prednisol is about 60 mg, and under the condition of continuing hemorrhagic syndrome in the form of the appearance of new hemorrhages , the dose of Prednisolone should be doubled. The duration of corticosteroid therapy directly depends on the speed of arresting the clinical manifestations of hemorrhagic syndrome and the degree of normalization of the number of platelet blood cells. In a situation where a decrease in the dosage of Prednisolon provokes the recurrence of a hemorrhagic syndrome, it is necessary to increase the dose of the drug again.
An adequate dose of immunosuppressants is 5 mg per 1 kg of patient weight (Cyclosporin A). Absolute indications for the use of drugs of this group is the ongoing course of hemorrhagic syndrome and progressive thrombocytopenia even after splenectomy.
When hemorrhagic telangiectasias, as one of the variants of hemorrhagic syndrome, the application of general therapeutic measures does not have a positive effect. However, the use of estrogen or testosterone in some situations reduces bleeding. Recently, the methods of operative and laser correction of this pathology have been widely used, with good long-term results and a low percentage of relapse.
Since various variants of hemorrhagic syndrome are accompanied by the development of anemia of the organism to some extent, in most cases, patients suffering from increased bleeding should additionally be prescribed iron-containing medications (Tardiferone in a daily dose of 600 mg).
Prevention of hemorrhagic syndrome
As the primary and most effective methods for preventing the development of hemorrhagic syndrome among children of the newborn period, planned subcutaneous administration of vitamin K to all premature infants should be considered, as well as an early application of the baby to the breast.
With regard to preventive measures aimed at preventing the development of hemophilia, as the most severe form of hemorrhagic syndrome, it is necessary to consider medical genetic counseling, which allows the most accurate determination of the risk of the birth of a child with signs of hemophilia. People suffering from hemorrhagic syndrome should inform all treating specialists about the presence of this pathology, since even a banal tooth extraction in this category of patients should be accompanied by preliminary medication preparation.
With regard to the prevention of the development of repeated episodes of hemorrhagic syndrome, especially the hematoma variant, special exercises of physiotherapy exercises have a good positive effect, provided they are regularly performed. In addition, during the remission period, patients suffering from hemorrhagic syndrome are shown to use resorptive physiotherapeutic manipulations (magnetotherapy, electrophoresis).
? Hemorrhagic syndrome - which doctor will help ? If there is or suspected development of hemorrhagic syndrome, you should immediately seek advice from such doctors as a hematologist or transfusiologist.