Thrombocytopathy is a category of pathological conditions of the hemorrhagic category, the formation of which is caused by the disturbance of normal vital activity and functioning of blood cells of the platelet number with unchanged their quantitative composition. The presence of one of the pathogenetic forms of thrombocytopathy is accompanied by the development of pathognomonic clinical symptoms in the form of hemorrhages and bleeding in various parts of the human body, even against the background of complete well-being.
Diagnosis of hereditary thrombocytopathy is not difficult on the part of an experienced hematologist, however, a number of specific laboratory tests are required to verify the diagnosis reliably.
Causes of thrombocytopathy
The system of primary platelet-vascular hemostasis consists of a successive chain of reactions (adhesion, platelet aggregation, retraction of the blood clot, activation of the plasmogen), so excluding at least one of these physiological processes entails the development of a hemorrhagic syndrome of varying intensity.
Adhesion means "adherence of platelet cells" to each other and to the endothelium of the vessel, the provocateur of which is traumatic damage to the vascular wall. In the process of development of platelet adhesion, several haemostatic factors (von Willebrant factor, calcium ions, platelet membrane glycoproteins) indirectly participate. At the end of the adhesive reaction, a mechanism is activated to increase the platelet mass (aggregation), which is stimulated by a wide range of biologically active substances in the form of thrombin, adenosine phosphate, catecholamines and serotonin. As a result of the retraction of the blood clot, in which the liquid component in the form of serum is separated from the total platelet conglomerate, the thrombus becomes denser and tightly attached to the vascular wall.
The process of development of thrombocytopathy of congenital genesis is negatively affected by the defect of genes responsible for the coding of various factors that are involved in the implementation of hemostasis at various stages. A remarkable fact is that thrombocytopathies of congenital nature are inherited both by the recessive and dominant variant of inheritance, which explains the wide spread of this pathology in the general population.
The most common cause of thrombocytopathy secondary development is the long-term use of certain drugs of the group of anticoagulants and non-steroidal anti-inflammatory drugs. The peculiarity of acquired thrombocytopathy is that they all belong to functional pathological conditions and are leveled after elimination of the root cause of their occurrence. In addition, various blood diseases can have an indirect effect on the state of blood cells of the platelet, thereby provoking the development of thrombocytopathy, which occurs in DIC syndrome , leukemia and severe forms of anemia.
Symptoms of thrombocytopathy
Specificity and intensity of clinical manifestations in thrombocytopathy directly depends on the pathogenetic form of the disease. So, for all hereditary thrombocytopathies is characterized by the presence of a so-called clinical manifestation, which has a provocative effect on the development of a typical clinical symptom complex in a child. Such "provocateurs" include perinatal pathologies in the form of acidosis and hypoxia, birth trauma, viral and bacterial damage to the fetus, prolonged insolation, vitamin deficiency, vaccination.
The most pathognomonic symptom, allowing at an early stage of development of thrombocytopathy to suspect the presence of this formidable condition, is a hemorrhagic symptom complex. Immediately after delivery, the child can develop a cephalogram, intracranial hemorrhage and delayed healing, as well as bleeding umbilical wound. The presence of these signs requires additional examination of the patient in order to identify the form of hereditary thrombocytopathy and determine the scope of medical treatment.
Dermal manifestations of the hemorrhagic symptom complex in thrombocytopathy are common petechiae and ecchymosis, for the onset of which there should not necessarily be a traumatic effect. In some situations, these manifestations on the skin can occur against a background of complete well-being. A remarkable symptom that distinguishes hemorrhagic syndrome in thrombocytopathy from hemorrhagic manifestations that occur in other diseases, for example, in hemophilia , is the complete absence of hemarthrosis and intermuscular hematomas, so this criterion is used by hematologists for differential diagnosis.
Another specific symptom of thrombocytopathy is increased bleeding of the mucous membranes of the oral and nasal cavities, which is accompanied by a tendency to nosebleeds and bleeding gums of low intensity. Female patients of various age, suffering from thrombocytopathy, note a prolonged course and heavy bleeding during menstruation, as well as the development of short-term episodes of uterine bleeding of a dysfunctional nature, that is, the onset of them has nothing to do with the menstrual cycle.
When even small surgical intervention is applied to people suffering from thrombocytopathy, there is abundant and prolonged bleeding, which is not commensurate with the amount of traumatic effects (tooth extraction, tonsil removal). A remarkable fact is that among all the variety of manifestations of hemorrhagic syndrome with thrombocytopathy, there are practically no episodes of intracavitary bleeding and bleeding from the organs of the gastrointestinal tract.
With a long course of thrombocytopathy and frequent episodes of bleeding, there are signs of a posthemorrhagic anemic syndrome, aggravating the course of the underlying disease. Manifestations of this complication are the development of general weakness, the inability to perform habitual physical activity, a tendency to dizziness and short-term episodes of loss of consciousness.
The presence of a number of nonspecific symptoms in some situations makes it difficult to conduct a timely verification of the diagnosis, therefore the main help will be the laboratory diagnosis of thrombocytopathy, which includes not only standard blood tests and coagulogram, but also specific samples (analysis of aggregation and adhesiveness of blood cells of platelets, factor analysis coagulation). It should be borne in mind that a single laboratory examination of a patient with suspected thrombocytopathy is inadequate. For a reliable diagnosis, three-time laboratory diagnostics in full, carried out in different phases of the hemorrhagic syndrome (during the period, activity, fading and complete remission) is mandatory. In difficult situations for the purpose of determining the pathomorphological variant of thrombocytopathy, it is advisable to use such diagnostic manipulation as trepanobiopsia with subsequent examination of the myelogram.
The pathogenetic development of all variants of disaggregation thrombocytopathies is based on the violation of the aggregation capacity of blood cells of the platelet series. One of the variants of hereditary disaggregation thrombocytopathy, proceeding without violating the "liberation process" is Glanzman's thrombastenia, transmitted both by the dominant and recessive type of inheritance. At the heart of the development of this disease is the absolute insufficiency of glycoproteins on the platelet membrane, resulting in a violation of the reaction with agents provoking aggregation. The main clinical manifestations of Glanzman's thrombastenia are the appearance of skin rashes in the form of point petechiae and hematomas. To a greater extent this pathology is found among girls who have long episodes of uterine bleeding of a dysfunctional nature and the subsequent development of signs of posthemorrhagic anemic syndrome of severe severity. Patognomonic clinical manifestation of disaggregation thrombocytopathy is the development of hemorrhage into the retina of the eye, as well as intracerebral hemorrhage, which is considered an unfavorable prognostic criterion.
In addition, hereditary disaggregation thrombocytopathies can occur without disturbing the "release process", for example, the May-Hegglin anomaly, which is characterized by a significant change in the morphological characteristics of platelets. The main diagnostic criteria of this thrombocytopathy are not clinical boards, but laboratory disorders in the form of severe thrombocytopenia and giant metric parameters of platelets.
With disaggregating thrombocytopathies occurring with a violation of the "release process", the main provoker of hemorrhagic syndrome development is the weakening of the degranulation of blood cells of the platelet series and the release of physiological platelet factors, which under normal conditions are released during the interaction of platelets with the collagen of the vascular wall. This category of thrombocytopathy belongs to the group of hemorrhagic syndromes, which have a favorable course and minimal clinical manifestations.
Thrombocytopathy in children
In childhood, the main cause of hemorrhagic syndrome of different intensity is hereditary thrombocytopathy, which is difficult to diagnose and require the use of specific treatment. According to the world statistics, about 65% of all episodes of hemorrhagic syndrome are pathological states of the group of thrombocytopathies, of which at least 40% are inherited.
In most situations, hereditary thrombocytopathy in an isolated form is accompanied by the development of a mild hemorrhagic symptom complex, while those associated with other hemostasis of thrombocytopathy cause severe complications of the hemorrhagic profile.
Despite the large spread of diseases of this category among children, in most cases there is no fact of an early diagnosis of thrombocytopathy. Clinical manifestations in the form of excessive bleeding gums or dysfunctional uterine bleeding become the basis for the treatment of parents to specialists of a narrow profile, while patients of this category should be observed in the hematologist.
Classification of thrombocytopathy of hereditary genesis is based on the separation according to the principle of determining dysfunction, biochemical and pathomorphological disorders of blood cells of the platelet number. This classification is used only by specialists in the hematology profile, and practicing pediatricians share thrombocytopathy solely on the basis of the etiopathogenetic principle. For the development of thrombocytopathy, the sex of the child does not matter, therefore this pathology often occurs in both the female and the male. There is a tendency to decrease the activity of hemorrhagic syndrome with increasing age of the patient.
In regard to the manifestations of hemorrhagic syndrome in children suffering from thrombocytopathy, the priority is occupied by microcirculatory nasal bleeding and skin petechial rash. In children, almost never there is a bleeding on the background of complete well-being, in any situation there is a provocative effect of any factor, for example, bacterial infection or physiotherapy manipulation. In some situations, hereditary thrombocytopathy is combined with various anomalies in the development of connective tissue, which together has a severe clinical symptomatology and can lead to the development of patient disability.
With an additional laboratory examination of a child suffering from thrombocytopathy, it is possible to identify multiple disorders in the form of impaired morphology of blood cells of the platelet count with an absolutely unchanged quantitative composition, prolong bleeding time, reduce retraction of the blood clot, reduce aggregation and adhesion of platelets, and decrease platelet dehydrogenase activity. In addition, various pathogenetic variants of thrombocytopathy are distinguished by specific laboratory criteria, the detection of which is possible only under the conditions of a specialized laboratory of the hematological profile.
Treatment of thrombocytopathy
When deciding on the need for and scope of medical treatment needed in each specific case, the fundamental criterion is the determination of the pathogenetic form of thrombocytopathy. However, in all cases of thrombocytopathy, it is important to adjust the lifestyle of the patient, implying the restriction of the fact of any traumatic influence that can cause severe bleeding. Correction of eating behavior consists in a sharp restriction of the use of any products containing vinegar, even in the minimum concentration. In the case of acquired thrombocytopathy of a known etiology, the primary action should be the elimination of the negative effect of the provoking factor on the development of hemorrhagic syndrome (abolition of drugs that affect blood coagulability).
With regard to the drug treatment of disaggregated thrombocytopathy, the use of aminocaproic acid in a daily dosage of 12 g divided into 8 procedures should be preferred, the action of which is aimed at reducing bleeding and bleeding time. The positive effect of aminocaproic acid on the relief of intrauterine and nasal bleeding is noted to a greater degree, the scheme of application being to take the drug every six days from the beginning of the menstrual cycle, and in the minimum effective dosage. It should be borne in mind that prolonged use of aminocaproic acid may provoke the development of amenorrhea , which requires differentiation with pregnancy. The topical route of administration is oral, as parenteral administration of aminocaproic acid may be accompanied by the development of serious complications in the form of DIC syndrome.
In addition to its direct action, oral contraceptives have a stimulating effect on the process of platelet aggregation; therefore, drugs of this pharmacological category can be used in the therapy of symptomatic thrombocytopathy. However, hormonal oral contraceptives should be used with caution to persons prone to developing thrombosis and DIC syndrome (patients suffering from promyelocytic leukemia, collagenoses, chronic liver diseases). Absolutely contraindicated combination of aminocaproic acid and oral contraceptives in the treatment of thrombocytopathy.
The variants of pathogenetic therapy of disaggregation thrombocytopathy include the combined use of intramuscular injection of 1% ATP solution at a dose of 2 ml with intramuscular injection of 25% solution of Magnesium Sulphate in a dose of 5 ml.
When microcirculatory bleeding has a good local hemostatic effect, Adrose (0.025% solution subcutaneously in a dose of 1 ml). This drug is used even in the treatment of patients prone to developing DIC syndrome, which greatly expands the scope of its use. As a local therapy with bleeding gums or nose bleeding, the method of using turunds moistened in Adroxon solution is used.
In a situation where thrombocytopathy is combined with a pronounced thrombocytopenic syndrome, it is advisable to use drugs of the group of bradykinin antagonists (anginine in a daily dosage of 1 g orally), but treatment with these means implies a long course of their use.
The principal difference between thrombocytopathy and thrombocytopenia is that, with this pathology, blood transfusion is absolutely contraindicated, since this manipulation provokes the aggravation of the dysfunction of the blood cells of the platelet, which creates the conditions for the development of the DIC syndrome.
Local bleeding therapy is to irrigate the bleeding surface with aminocaproic acid using its 5% concentration and subsequent application of the fibrin film. As a preventive medicamentous treatment of thrombocytopathy, hematologists consider the appointment of a course of taking metabolic drugs according to a special scheme: the first three days of oral Riboxin intake in a daily dose of 2.4 g simultaneously with potassium Orotate in a daily dose of 1.5 g. The patient is then recommended to intramuscularly administer the vitamin B12 in a prophylactic daily dose of 1 mcg.
? Thrombocytopathy - which doctor will help ? If there is or suspected thrombocytopathy, immediately consult a doctor such as a hematologist or pediatrician.