thrombocytopathy - a category of pathological conditions hemorrhagic category, the formation of which is due to violation of normal life and functioning of blood cells in the platelet number unchanged their quantitative composition. presence of a pathogenic form thrombocytopathy pathognomonic accompanied by the development of clinical symptoms in the form of appearance of hemorrhage and bleeding in various parts of the human body, even against the backdrop of well-being.
Diagnostics thrombocytopathy not hereditary nature of the difficulties experienced by the hematologist, but for reliable verification of the diagnosis requires a number of specific laboratory tests.
primary platelet-vascular hemostasis consists of a sequential chain reaction (adhesion, platelet aggregation, a retraction clot activation plazmenogena), so the exclusion of at least one of these physiological processes entails the development of hemorrhagic syndrome of varying degr
By adhesion is understood "the adhesion of platelet cells," to each other and to the vascular endothelium, which is the agent provocateur traumatic vascular wall.In the development of platelet adhesion indirectly participates several haemostatic factors (factor Villebranta, calcium ions, platelet membrane glycoproteins).Upon completion of the reaction adhesive mechanism starts increasing platelets (aggregation), which exerts stimulatory effect a wide spectrum of biologically active compounds in a thrombin-adenosine, catecholamines and serotonin.As a result of blood clot retraction, in which the total of platelet conglomerate separated liquid component in the form of a serum, a blood clot is sealed and tightly attached to the vascular wall.
on development thrombocytopathy genesis of congenital defect negatively affects the genes responsible for coding the various factors involved in the implementation of the various stages of hemostasis.A notable fact is that the inherent nature thrombocytopathy inherited as a recessive or dominant inheritance variant, which explains the wide spread of this disease in the general population.
most common cause of secondary genesis thrombocytopathy is a long reception of certain drugs anticoagulant and nonsteroidal antiinflammatory drugs.Thrombocytopathy acquired peculiarity is that they all belong to the functional and pathological conditions leveled after the elimination of the root causes of their occurrence.In addition, a variety of blood diseases may have an indirect impact on the number of platelet blood cells, thus provoking the development thrombocytopathy that occurs in DIC, leukemia and severe anemia.
The specificity and intensity of clinical manifestations at thrombocytopathy depends on the pathogenic form of the disease.So for all hereditary thrombocytopathy characterized by the presence of so-called clinical manifestation, which has a provocative effect on the development of a typical clinical symptom in children.These "provocateurs" should include perinatal pathology in the form of acidosis and hypoxia, birth trauma, viral and bacterial damage the fetus, prolonged sun exposure, vitamin deficiency, vaccination.
most pathognomonic symptom allowing infancy thrombocytopathy suspect the presence of this terrible state, is a hemorrhagic syndrome.Immediately after delivery, the child may experience the development kefalogematomy, intracranial hemorrhage, and slow healing, and bleeding of the umbilical wound.The presence of these symptoms requires further examination of the patient to identify the forms of hereditary thrombocytopathy and determine the amount of medical treatment.
cutaneous manifestations of hemorrhagic symptom when thrombocytopathy are widespread petechiae and ecchymosis, for the occurrence of which need not be a traumatic effect.In some situations, these manifestations on the skin may occur against the backdrop of well-being.A notable sign of distinguishing hemorrhagic syndrome in thrombocytopathy by hemorrhagic manifestations that occur in other diseases, such as hemophilia, is the complete absence of hemarthrosis, and intermuscular hematomas, so this criterion hematologists used in the differential diagnosis.
Another thrombocytopathy specific symptom is increased bleeding of mucous membranes of the oral and nasal cavities, which is accompanied by a tendency to nosebleeds and bleeding gums of low intensity.Patients females of different ages suffering thrombocytopathy, noted for long and heavy bleeding during menstruation, as well as the development of short-term episodes of dysfunctional uterine bleeding nature, that is, their offensive has nothing to do with the menstrual cycle.
In applying even a small surgical intervention in relation to persons suffering thrombocytopathy, there is abundant and prolonged bleeding, which is not commensurate with the volume of the traumatic effects (tooth extraction, tonsillectomy).A notable fact is that among the variety of manifestations of hemorrhagic syndrome in thrombocytopathy, almost never occur episodes of intracavitary bleeding and bleeding from the gastrointestinal tract.
thrombocytopathy In the long and frequent bleeding episodes, there are signs of hemorrhagic anemia syndrome, exacerbate the underlying disease.Manifestations of this complication is the development of general weakness, inability to perform usual physical activity, tendency to short episodes of dizziness and loss of consciousness.
presence of a number of non-specific symptoms in some situations makes it difficult to timely diagnosis verification, so the main help will be a laboratory diagnosis thrombocytopathy, which includes not only the standard blood tests and coagulation, but also for specific samples (analysis of the aggregation ability and adhesiveness blood cell platelet number,analysis of the coagulation factors).Note that holding a single laboratory examination of the patient with suspected thrombocytopathy is insufficient.For reliable diagnosis is mandatory three-time laboratory diagnosis fully carried out in different phases of hemorrhagic syndrome (during the period of activity, decay and complete remission).In difficult situations, to determine the pathological variants thrombocytopathy advisable to use such diagnostic procedures as the trepanobiopsy followed research myelogram.
The pathogenetic basis of all variants of disaggregation thrombocytopathy supposed violation of the aggregation ability of blood cells platelet number.One option hereditary disaggregation thrombocytopathy flowing without disturbing the "process of liberation" is trombastenii Glantsmana, is transmitted as a dominant or recessive inheritance.The basis of this disease put an absolute lack of glycoproteins on platelet membrane, resulting in a violation of the reaction with the agents that provoke aggregation.The main clinical manifestations trombastenii Glantsmana is the appearance of skin rash in the form of point petechiae and hematoma.In this pathology more common among girls, who observed prolonged episodes of uterine bleeding dysfunctional nature and subsequent development of the symptoms of anemia hemorrhagic severe.Pathognomonic clinical manifestation thrombocytopathy disaggregation is the development of retinal hemorrhage, and intracerebral hemorrhage, which are considered a poor prognostic criteria.
In addition, hereditary disaggregation thrombocytopathy can occur without violating the "process of liberation", such an anomaly Mey - Hegglina, which is characterized by a significant change in the morphological characteristics of the platelets.The major diagnostic criteria are not given thrombocytopathy clinical governance, and laboratory abnormalities in the form of severe thrombocytopenia and giant platelets metric parameters.
When disaggregation thrombocytopathy proceeding in violation of "the process of liberation," the chief provocateur of hemorrhagic syndrome is a weakening of the blood cell degranulation and the release of platelet number of physiological platelet factors that are released under normal conditions the interaction of platelets with collagen vascular wall.This category thrombocytopathy belongs to the group of hemorrhagic syndromes, with favorable course and minimal clinical manifestations.
In children, the main cause of hemorrhagic syndrome of varying intensity is a hereditary thrombocytopathy that are difficult to diagnose and require the application of specific treatment.According to world statistics, about 65% of all episodes of hemorrhagic syndrome constitute pathological conditions thrombocytopathy group, of which the share of inherited not less than 40%.
In most situations, hereditary thrombocytopathy in isolation accompanied by the development of hemorrhagic symptom of mild, while combined with other disorders of hemostasis thrombocytopathy become a cause of serious complications of hemorrhagic profile.
Despite the large spread of disease among this category of childhood, in most cases, there is no fact of early diagnosis thrombocytopathy.Clinical manifestations of excessive bleeding gums or dysfunctional uterine bleeding are grounds for the parents to specialists narrow profile, while the patients in this category have seen a hematologist.
Classification thrombocytopathy hereditary origin is based on the principle of the division of the definition of dysfunction, biochemical and pathological disorders blood cell platelet number.This classification shall be confined to experts hematological profile, and practicing pediatricians share thrombocytopathy exclusively Etiopatogenetichesky principle.To develop thrombocytopathy not important sex of the baby, so this pathology is equally often occurs in people both female and male.There is a trend to reduced activity of hemorrhagic syndrome with increasing age of the patient.
of manifestations of hemorrhagic syndrome in children suffering thrombocytopathy, most take priority microcirculatory nosebleeds and skin petechiae.Individuals childhood almost never bleeding occurs on a background of complete well-being, in every situation there is provokatornoe impact of any factor, such as a bacterial infection or physical therapy manipulation.In some situations thrombocytopathy hereditary origin combine with a variety of developmental abnormalities of the connective tissue, all of which have severe clinical symptoms and can cause the development of the patient's disability.
further laboratory examination of a child suffering thrombocytopathy, possible to identify multiple violations in the form of impaired morphology of blood cell platelet number when absolutely unchanged its quantitative composition, prolonged bleeding, reduce the retraction of a blood clot, reducing the aggregation and adhesive reaction of platelets and reduce the activity of dehydrogenases platelets.In addition, various pathogenic variants thrombocytopathy different specific laboratory criteria, the identification of which is possible only in conditions of a specialized laboratory hematology profile.
When deciding on the need and extent of medical treatment required in each case, the fundamental criterion is to determine the pathogenic form thrombocytopathy.However, all variants thrombocytopathy important correction of the patient's lifestyle, which implies limiting fact of any traumatic influence, which can cause the development of severe bleeding.Correction of eating behavior is the severe restriction of the use of any products containing vinegar, even in minimal concentration.In the case of acquired thrombocytopathy known etiology, priority actions should be to prevent the negative impact of a provoking factor for the development of hemorrhagic syndrome (abolition of drugs that affect blood clotting).
in respect of medical treatment disaggregation thrombocytopathy should favor the use of aminocaproic acid in a daily dosage of 12 grams divided into 8 receptions, the effect of which is to reduce the bleeding and bleeding time.The more marked positive effect of aminocaproic acid in relation to endometrial edema and nasal bleeding, with the pattern of use is taking the drug every first six days from the beginning of the menstrual cycle, and in the lowest effective dose.Note that prolonged use of aminocaproic acid can induce amenorrhea requiring differentiation pregnancy.By priority administration is oral, as parenteral administration of aminocaproic acid may be associated with the development of serious complications such as DIC.Oral contraceptives
addition to its direct effect have a stimulating effect on the process of platelet aggregation, so the drugs are given pharmacological categories can be used in the treatment of symptomatic trombotsitopaty.However, hormonal oral contraceptives should be used with caution in people prone to the development of thrombosis and disseminated intravascular coagulation (patients with promyelocytic leukemia, connective tissue diseases, chronic liver disease).Absolutely contraindicated combination of aminocaproic acid and oral contraceptives in the treatment thrombocytopathy.
to variants of pathogenic therapy thrombocytopathy disaggregation is the combined use of intramuscular injection of a 1% solution of ATP at a dose of 2 ml intramuscular injection of a 25% solution of magnesium sulfate at a dose of 5 mL.When
microcirculatory bleeding good local hemostatic effect has Adrokson (0.025% solution subcutaneously in a dose of 1 ml).This drug is used even in the treatment of patients prone to the development of DIC, which significantly expands the scope of its application.As a topical therapy with bleeding gums or epistaxis the method of use turundas moistened with a Adroksona.
In situations when combined with the expressed trombotsitopaty thrombocytopenic syndrome advisable to use preparations of antagonists of bradykinin (Anginin at a dose of 1 g orally), but the treatment with these agents involves long course of their application.