Thrombocytopenic purpura is one of the numerous variants of hematological diseases, the main manifestation of which is the appearance of a tendency to increased bleeding, the pathogenetic substrate of which is thrombocytopenia. This form is the most common in the generalized structure of the incidence of hemorrhagic diathesis. The debut of clinical and laboratory manifestations of thrombocytopenic purpura occurs in the early childhood, with the majority of patients being girls. The main provoking factor that acts as a manifestator of manifestations of thrombocytopenic purpura, as a rule, is the infection of the child's body.
Causes of thrombocytopenic purpura
The diagnosis of "thrombocytopenic purpura" is established by hematologists when the patient has a fact of a decrease in the number of platelet blood cells, arising for an unknown reason and not associated with any other pathological condition.
At present, the etiopathogenetic factors of thrombocytopenic purpura are not reliably determined, therefore a large number of studies are devoted to the search for the main causes of the development of this serious disease.
The risk group for the development of thrombocytopenic purpura is children 4-6 years, although in some situations manifestations of hemorrhagic nature can make their debut in the first year of the child's life.
Thrombocytopenic purpura in children under the age of fourteen can be equally common among both sexes. Thrombocytopenic purpura in adults is several times more common among women.
The vast majority of specialists in the field of hematology adhere to the so-called "infectious theory" of the development of thrombocytopenic purpura, implying a correlation between the onset of clinical manifestations of hemorrhagic syndrome and the infection of the body with viral agents. Against the background of a bacterial infection, thrombocytopenic purpura develops extremely rarely.
The main pathogenetic mechanism of manifestation of thrombocytopenic purpura is a decrease in the platelet concentration, caused by increased destruction of blood platelets.
In the case of a significant excess of the number of blood plates that have undergone destruction, with simultaneous depletion of bone marrow reserves, a progressive thrombocytopenic syndrome develops.
The process of increased destruction of blood platelets with thrombocytopenic purpura has a stimulating effect of immunological disorders. However, this pathogenetic mechanism is not unique. The process of destruction of blood platelets is influenced by the state of the spleen and, with pathological changes, conditions are created for increased destruction of blood cells of the platelet series.
A separate etiopathogenetic form of this disease is the so-called "autoimmune thrombocytopenic purpura", in which primary lesion of the thymus and spleen takes place, which are the main organs producing antibodies that stimulate the destruction of thrombocytes.
Thus, the autoimmune process triggers the synthesis of antibodies, the destructive effect of which is directed against the platelets of one's own organism.
With the toxic effect of certain medicinal preparations in the form of salicylates, sulfonamides, antibacterial agents, and after introduction of foreign substances into the body of viruses, development of violations of the morphology of platelet blood cells, which provokes the formation of pathogenesis of the heteroimmune form of thrombocytopenic purpura.
The immune form of thrombocytopenic purpura is also formed with the negative influence of bacterial and viral agents provoking the development of vascular wall damage, as well as the main parts of the blood coagulation system.
In addition, the process of formation of hemorrhagic syndrome, which is the main manifestation of thrombocytopenic purpura, has a regulating effect of serotonin concentration in the patient's blood. The regulating function of serotonin is to conduct nerve impulses from the internal organs to the structures of the brain and back. Another function of serotonin is the stimulation of platelet aggregation and vasoconstrictor action.
Symptoms of thrombocytopenic purpura
Depending on the rate of increase in clinical manifestations, the patient may develop an acute or chronic variant of the course of thrombocytopenic purpura. In acute form, the duration of clinical and laboratory changes does not exceed six months, after which the patient is absolutely healthy. The acute course of thrombocytopenic purpura is more typical for patients of children's age category. The debut of clinical manifestations, as a rule, falls on the period of routine vaccination or transferred virus attacks. The so-called "incubation period" in this case is approximately 14 days.
Acute hemorrhagic syndrome, as a manifestation of thrombocytopenic purpura, consists in the appearance of diffuse intradermal hemorrhages, frequent episodes of nasal bleeding, and a sharp deterioration in the child's condition. Objective signs of the existing increased bleeding is pronounced pallor of the skin, low blood pressure, febrile fever. In pediatric practice, the duration of the acute course of thrombocytopenic purpura is not more than one month, after which the complete elimination of signs of increased bleeding occurs.
The majority of patients suffering from acute thrombocytopenic purpura, note changes in the organs of the lymphatic system, manifested in an increase in the morbidity of large groups of lymph nodes that have all the signs of inflammation. In some cases, children have a latent flow of thrombocytopenic purpura, in which the clinical manifestations are so minimal that they do not cause a prolonged health disorder.
The chronic form of thrombocytopenic purpura differs in the slow slow progressing debut of clinical manifestations, the primary symptoms being so nonspecific and not pathognomonic for this pathology that it is not possible to make a diagnosis in the early stages.
The most characteristic signs of chronic thrombocytopenic purpura is the appearance of a punctate hemorrhagic rash on the surface of the skin, short-term episodes of nasal bleeding of an uninvited nature. The main sign of exacerbation in the chronic variant of the course of thrombocytopenic purpura is the resumption of hemorrhages, among which the leading positions are occupied by intradermal point elements of the rash. For the appearance of hemorrhages on the surface of the skin or mucous membranes there should not necessarily be the presence of any traumatic factor, in some situations, hemorrhages arise against a background of complete well-being, especially at night.
An extremely rare symptom of thrombocytopenic purpura is the so-called "bloody tear" symptom, the morphological substrate of which is hemorrhages in the belly or retina. Along with skin hemorrhagic manifestations with thrombocytopenic purpura, hemorrhages of varying intensity can develop, which are of a persistent nature and can provoke the development of anemia. In some situations, bleeding can develop from the vessels of the oral cavity, and this condition is subject to mandatory hospitalization of the patient in the profile department. Bleeding gastrointestinal and uterine nature with thrombocytopenic purpura are extremely rare.
In contrast to hemophilia , the main manifestation of which is also hemorrhagic syndrome , thrombocytopenic purpura under no circumstances is accompanied by the development of intermuscular clusters of blood and hemarthrosis, subcutaneous hematomas.
In some situations, patients seek medical help only at the stage of development of severe posthemorrhagic anemia, which is manifested by lethargy, irritability, weakness, rapid fatigue.
Idiopathic thrombocytopenic purpura
This form of thrombocytopenia is a hemorrhagic disease, the occurrence of which is due to a reduction in the life span of platelet blood cells and a sharp decrease in their concentration in the blood. The name "idiopathic" is due to the inability to reliably determine the cause of the disease. However, in recent years an enormous amount of scientific research has been devoted to the study of the etiopathogenesis of the development of idiopathic thrombocytopenic purpura.
The result of these studies is the allocation of the main role of burdened heredity in the development of idiopathic thrombocytopenic purpura. When a combination of hereditary defects and immunological disorders in a child, there is a large risk of developing this hemorrhagic disease.
Given etiopathogenetic mechanisms of development, idiopathic thrombocytopenic purpura is usually divided into autoimmune (there is an increased production of antibodies to its own platelet antigens) and heteroimmune (antibodies are produced in response to virus-damaged platelet antigens). A hetero-immune variant of idiopathic thrombocytopenic purpura is more favorable for the prognosis for the patient's life.
Autoimmune idiopathic thrombocytopenic purpura is characterized by a slow progressing malignant course, and its danger is that the patient develops critical thrombocytopenia (less than 30 * 10 / L).
The main clinical manifestations of idiopathic thrombocytopenic purpura are signs of a hemorrhagic symptom complex with a petechial-spotted variant of hemorrhage. The preferred localization of cutaneous manifestations is an anterolateral surface of the lower extremities. They are polygonal in shape, of various sizes, sometimes merging. A characteristic feature of idiopathic thrombocytopenic purpura is a change in the color of the rash, depending on the stage of the hemorrhagic syndrome from purple-brown to yellow-green.
Aggressiveness of manifestations of idiopathic thrombocytopenic purpura also lies in the increased propensity to develop massive bleeding of various locations (gastrointestinal, uterine, renal), which is not typical for the classical version of thrombocytopenic purpura.
When verifying the diagnosis, hematologists perform differential diagnosis between idiopathic thrombocytopenic purpura and symptomatic thrombocytopenia, as these conditions require the use of various treatment methods and patient management tactics. During the period of acute exacerbation, the main areas of treatment are the fight against bleeding, while in the period of remission all efforts are directed to the prevention of exacerbations and complications of the underlying disease.
The diagnosis of "idiopathic thrombocytopenic purpura" implies the further administration of a course of glucocorticosteroid therapy and the further conduct of splenectomy. In a situation where a patient suffering from idiopathic thrombocytopenic purpura appears to have signs of an intracerebral hemorrhage, splenectomy is performed on urgent indications in an urgent order.
Diagnosis of thrombocytopenic purpura
The disease "thrombocytopenic purpura" is established by hematologists not only on the basis of pathognomonic clinical manifestations characteristic of the hemorrhagic symptom complex, but also taking into account the indices of laboratory studies.
Since thrombocytopenic purpura at the beginning of its development may not have specific symptoms and simulate the clinical picture of other blood diseases, the primary task of the attending physician is to conduct an adequate differential diagnosis between these conditions. The main difference between thrombocytopenic purpura and other hematological diseases accompanied by the development of hemorrhagic syndrome is the complete absence of changes in the spleen, as well as a tendency to develop macrocytic anemia, the severity of which does not correspond to the volume of blood loss.
With regard to laboratory criteria for thrombocytopenic purpura, there are significant differences in the course of the course of the disease. Thus, with chronic thrombocytopenic purpura in the remission stage of clinical manifestations, there can be no changes in blood tests completely. Prolonged flow of thrombocytopenic purpura sooner or later provokes the development of laboratory signs of posthemorrhagic anemia of hyporegulatory, and sometimes hypoplastic.
However, the most pathognomonic laboratory sign of thrombocytopenic purpura is thrombocytopenia, which has a persistent character. In addition, a specific manifestation of hemorrhagic syndrome, as a sign of thrombocytopenic purpura is a violation of the retraction of the blood clot.
Treatment of thrombocytopenic purpura
The volume and variety of therapeutic manipulations in this or that form of thrombocytopenic purpura is determined by the hematologist on the basis of an assessment of the severity of the patient's condition, the course and phase of the disease activity. In the treatment of thrombocytopenic purpura, both conservative and surgical therapies are used. For the first time, the manifestations of acute thrombocytopenic purpura, as well as exacerbation of hemorrhagic syndrome in the chronic course of this pathology, are the basis for the immediate hospitalization of the patient in the hematology department. A prerequisite for the successful treatment of thrombocytopenic purpura is the patient's adherence to strict bed rest and small adjustments to nutrition.
The basis of pathogenetically grounded treatment of thrombocytopenic purpura is the drugs of the hormonal group, as they are able to reduce the permeability of the vascular wall, enhance the blood clotting capacity, and also have immunostimulating effects on the body as a whole. Hormonal preparations are widely used for massive lesions of the skin, increased bleeding of the vessels of the mucous membranes, massive nasal and oropharyngeal bleeding, thrombocytopenia of the expressed degree of development. The drug of choice in this situation is Prednisolone, and at the beginning of therapy the so-called bolus method of administration in a dose of 120 mg is used, after which it is necessary to switch to a daily dosage of the preparation in 60 mg. With this method of drug therapy, a pronounced therapeutic effect usually occurs within two weeks, but the duration of hormone therapy can take a longer period of time to complete normalization of not only the patient's health state, but also laboratory manifestations of thrombocytopenic purpura.
A pronounced positive effect on the relief of manifestations of hemorrhagic syndrome in thrombocytopenic purpura has Aminocaproic acid in a daily calculated dose of 0.2 g / kg of patient weight three times a day.
In a situation where thrombocytopenic purpura is of a malignant nature with a pronounced hemorrhagic syndrome that provokes the development of severe posthemorrhagic anemia, the patient is shown performing blood transfusion.
Surgical treatment of thrombocytopenic purpura implies the operative removal of the spleen, since this organ has a stimulating effect on the process of destruction of platelet blood cells. In a situation where the applied medical and surgical methods of treatment do not bring a proper positive result in the form of elimination of signs of thrombocytopenic purpura, it is advisable to use drugs of the group of cytostatics, whose action is aimed at reducing the growth and multiplication of cells, as well as inhibiting the activity of autoimmune processes. (Cyclosporin A in a daily dose of 5 mg per 1 kg of the patient's weight). After arresting the acute process, all patients with thrombocytopenic purpura must be put on dispensary records for at least 5 years.
? Thrombocytopenic purpura - which doctor will help ? If there is or suspected the development of thrombocytopenic purpura, you should immediately seek advice from such doctors as a hematologist or transfusiologist.