thrombocytopenic purpura - this is one of the many variants of hematological diseases, the main manifestation of which is the emergence of an increased bleeding tendency, pathogenetic substrate is thrombocytopenia.This form is the most common disease in the generalized structure hemorrhagic diathesis.The debut of the clinical and laboratory manifestations thrombocytopenic purpura falls on the early childhood period, and most of the patients were girls.The main precipitating factor, which acts as a manifestation manifestatora thrombocytopenic purpura usually serves infection of the body of the child.
reasons thrombocytopenic purpura
Diagnosis "thrombocytopenic purpura" is set Hematology when the patient has a platelet fact reduce the number of blood cells, occurs for unknown reasons and not related to any other medical condition.
Currently not reliably identified etiopathogenic factors of thrombocytopenic purpura, so a large number of studies devoted to the sea
risk of developing thrombocytopenic purpura were children 4-6 years, although in some cases hemorrhagic manifestations could make his debut in the first year of life.
thrombocytopenic purpura in children who have not attained the age of fourteen can be equally common among both sexes.Thrombocytopenic purpura in adults several times more common among the female.
The vast majority of experts in the field of hematology adhere to the so-called "infectious theory" of thrombocytopenic purpura, implying a correlation between the debut of the clinical manifestations of hemorrhagic syndrome and infection with viral agents of the body.Against the background of the transferred bacterial infection thrombocytopenic purpura is extremely rare.
main pathogenetic mechanism of manifestation thrombocytopenic purpura is a decline in the concentration of platelets due to increased destruction of platelets.
In the case of a significant excess of the amount of platelets exposed to destruction, while the depletion of the reserves of the bone marrow develops progressive thrombotic thrombocytopenic syndrome.
In the process of increased destruction of platelets in the thrombocytopenic purpura has a stimulating effect of immunological disorders character.However, the pathogenetic mechanism is not unique.In the process of destruction of platelets affects the state of the spleen and pathological changes in the conditions for increased destruction of blood cells platelet number.
etiopathogenic separate form of this disease is the so-called "autoimmune thrombocytopenic purpura," in which there is a primary lesion of thymus and spleen, which are the main bodies that produce antibodies that stimulate the destruction of platelets.
Thus, the autoimmune process triggers the synthesis of antibodies, the destructive action directed against the body's own platelets.
At some toxic effects of drugs as salicylates, sulfonamides, antibacterial agents, as well as following the introduction of foreign substances into the body in the form of viruses is the development of disorders of platelet morphology of blood cells, which stimulates the formation of the pathogenesis geteroimmunnoy form thrombocytopenic purpura.
form of immune thrombocytopenic purpura is also formed at the negative impact of bacterial and viral agents that provoke the development of lesions vascular walls, as well as basic units of blood coagulation.
In addition, the formation of hemorrhagic syndrome, which is the main manifestation of thrombocytopenic purpura, has a regulating effect of the concentration of serotonin in the patient's blood.Regulatory function of serotonin is to carry nerve impulses from internal organs to the structures of the brain and vice versa.Another function is the stimulation of serotonin platelet aggregation and vasoconstrictive action.
symptoms of thrombocytopenic purpura
Depending on the rate of increase of clinical symptoms, the patient may develop acute or chronic thrombocytopenic purpura current version.In the acute form the duration of the clinical and laboratory changes does not exceed six months, after which the patient is deemed perfectly healthy.During Acute thrombocytopenic purpura is more characteristic for patients pediatric age category.The debut of clinical manifestations usually occurs during routine immunization or a viral attack.So-called "incubation period" in this case is about 14 days.
Acute hemorrhagic syndrome as a manifestation of thrombocytopenic purpura is the appearance of spilled intradermal haemorrhage, frequent episodes of nasal bleeding, sharp deterioration of the child.Available objective evidence of increased bleeding is severe pallor, reduced numbers of blood pressure, febrile.In pediatric practice, the duration of the current acute thrombocytopenic purpura is not more than one month, after which comes the complete elimination of signs of increased bleeding.
Most patients suffering from acute thrombocytopenic purpura, note changes in the organs of the lymphatic system, results in increased, painful lymph nodes larger groups, with all the signs of inflammation.In some cases, children have been latent for thrombocytopenic purpura, in which clinical manifestations are so minimal that do not cause long-term health problems.
Chronic thrombocytopenic purpura characterized by sluggish debut slow-progressive clinical manifestations, and the initial symptoms are so non-specific and are not pathognomonic for this disease is that the formation of the diagnosis in the early stages is not possible.
most characteristic signs of chronic thrombocytopenic purpura is a hemorrhagic rash appearance of pitting on the surface of the skin, transient episodes of nasal bleeding scant nature.The main symptom exacerbation of chronic variants of thrombocytopenic purpura is the resumption of bleeding, including a leading position occupied by intradermal dots rash.For the appearance of hemorrhage on the surface of the skin or mucous membranes is not necessarily the presence of any traumatic factor in some cases of hemorrhage occur against the backdrop of well-being, especially during the nighttime hours.
extremely rare symptom thrombocytopenic purpura is a symptom of the so-called "bloody tears" morphological substrate which is bleeding in the tunica albuginea or retina.Along with hemorrhagic manifestations in the skin thrombocytopenic purpura may develop bleeding of varying intensity, which are stubborn and can provoke the development of anemia.In some situations, the bleeding vessels can develop from the oral cavity, and this state is subject to compulsory hospitalization in specialized department.Bleeding of the gastrointestinal and mother nature while thrombocytopenic purpura are very rare.
Unlike hemophilia, the main manifestation of which is also a hemorrhagic syndrome, thrombocytopenic purpura under any circumstances are not accompanied by the development of clusters of blood and intermuscular hemarthroses, subcutaneous hematoma.
In some situations, patients seek medical care only at the stage of severe post-hemorrhagic anemia, manifested by lethargy, irritability, weakness, fatigue.
Idiopathic thrombocytopenic purpura
This form of thrombocytopenia is a hemorrhagic disease, the occurrence of which is attributable to the reduction of life period 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 a tremendous amount of research devoted to studying the issue of etiopathogenesis of idiopathic thrombocytopenic purpura.
result of these studies is allocating a leading role in the development of family history of idiopathic thrombocytopenic purpura.With the combination of genetic defects and immunological disorders in the child there is a high risk of developing the hemorrhagic disease.
Given the etiopathogenetic mechanisms of development, idiopathic thrombocytopenic purpura are divided into autoimmune (there is increased production of antibodies to self antigens of platelets) and geteroimmunnuyu (antibodies produced in response to virus damage platelet antigens).A more favorable prognosis with respect to the patient's life is considered geteroimmunny version of idiopathic thrombocytopenic purpura.
autoimmune idiopathic thrombocytopenic purpura characterized by sluggish progressive malignant course and the danger of it is that the patient develops critical thrombocytopenia (less than 30 * 10 / l).
main clinical manifestations of idiopathic thrombocytopenic purpura are signs hemorrhagic symptom with petechial-spotty bleeding option.Favourite localization of cutaneous manifestations is the anterolateral surface of the lower extremities.They are polygonal shapes, sizes, sometimes merging.A characteristic feature of idiopathic thrombocytopenic purpura rash is a change in color depending on the stage of hemorrhagic syndrome of purple-brown to yellow-green.
aggressive manifestations of idiopathic thrombocytopenic purpura is also an increased tendency to develop the massive bleeding of different localization (gastrointestinal, uterine, kidney), which is not typical for the classical variant thrombocytopenic purpura.
If verification of the diagnosis haematologists differential diagnosis between idiopathic thrombocytopenic purpura and symptomatic thrombocytopenia, as these conditions require the use of different treatment methods and tactics of the patient.During an exacerbation pillars of treatment is to combat the bleeding, while in remission all efforts directed at the prevention of exacerbations and complications of the underlying disease.
Diagnosis "idiopathic thrombocytopenic purpura" means the further course of the appointment of glucocorticosteroid therapy and further splenectomy.In a situation where a patient suffering from idiopathic thrombocytopenic purpura, there are signs of intracerebral hemorrhage, splenectomy performed for urgent indications in urgent procedure.
Diagnostics thrombocytopenic purpura
disease "thrombocytopenic purpura" hematologists installed not only on the basis of pathognomonic clinical manifestations characteristic of hemorrhagic symptom, but also taking into account the performance of laboratory research.
thrombocytopenic purpura Since the beginning of its development may not have specific symptoms and simulate the clinical picture of other diseases of the blood, is a priority of the attending physician is to carry out an adequate differential diagnosis between these states.The main difference thrombocytopenic purpura from other diseases hematological profile, accompanied by the development of hemorrhagic syndrome, is the complete absence of changes in the spleen, as well as the tendency to develop macrocytic anemia, the severity of which does not correspond to the volume of blood loss.
the laboratory criteria thrombocytopenic purpura there is considerable razbezhnosti depending on the disease.Thus, chronic thrombocytopenic purpura in remission of clinical manifestations may be completely absent changes in blood.Long for thrombocytopenic purpura eventually provokes the development of laboratory signs of post-hemorrhagic anemia giporegeneratornogo and sometimes hypoplastic nature.
However, the most pathognomonic laboratory thrombocytopenic purpura is a sign of anemia, which is stubborn.In addition, a specific manifestation of hemorrhagic syndrome, as a sign of thrombocytopenic purpura is a violation of the blood clot retraction.
treatment of thrombocytopenic purpura
volume and variety of medical manipulations at some form of thrombocytopenic purpura hematologist determined on the basis of assessing the severity of the patient's condition, current and phase of disease activity.In the treatment of thrombocytopenic purpura used both conservative and surgical methods of treatment.For the first time revealed signs of acute thrombocytopenic purpura, as well as exacerbation of hemorrhagic syndrome in the chronic course of this disease is the reason for immediate hospitalization in the department of hematology profile.A prerequisite for the successful treatment of thrombocytopenic purpura is patient compliance with strict bed rest and small power adjustments.
basis of pathogenesis based treatment of thrombocytopenic purpura make medicines hormone group, as they are capable of reducing vascular permeability, enhance the clotting ability of blood, and also have an immunostimulatory effect on the body as a whole.Hormonal drugs are widely used in the massive defeat of the skin, increased bleeding vessels of the mucous membranes of the nasal and oropharyngeal massive bleeding, severe degree of thrombocytopenia.The drug of choice in this situation is prednisolone, wherein at the beginning of therapy using so-called bolus administration at a dose of 120 mg, followed by a switch to the daily dose of the drug in 60 mg.With this method of drug therapy pronounced therapeutic effect, usually occurs within two weeks, but the duration of hormone therapy may take a longer time period to complete normalization of not only the patient's health, but also laboratory manifestations thrombocytopenic purpura.
pronounced positive effect against edema manifestations hemorrhagic syndrome in thrombocytopenic purpura has aminocaproic acid in the calculated daily dose of 0.2 g / kg body weight three times a day.
In a situation where thrombocytopenic purpura is malignancy with severe hemorrhagic syndrome, provoking the development of severe post-hemorrhagic anemia, the patient is shown holding a transfusion of blood components.
Surgical treatment of thrombocytopenic purpura involves surgical removal of the spleen, since this body has a stimulating effect on the process of destruction of platelet blood cells.