Thrombocytopenia is a pathology associated with a sharp drop in the number of red blood cells or platelets. Platelets have a huge impact on the mechanism of blood clotting, which is why their deficiency can cause bleeding or sharp hemorrhages in the cavity of internal organs, which is a direct threat to human health and life.
The essence of the morphology of platelets is that they do not have a nucleus, and they are parts of the megakaryocytic cytoplasm. Their formation occurs in the red bone marrow as a result of the process of detachment of fragments of the maternal cell. Platelets live no more than 12 days. Dead platelets digest macrophages in the tissues of the body, and instead of them young people mature from the bone marrow. The cell wall is represented by a membrane containing a special kind of molecules, the function of which is to recognize damaged sections of the arteries and veins. Even capillary bleeding starts the process of plugging the affected area with a blood plate, by inserting a so-called "patch" into the wound cavity of the vessel. Proceeding from this, the main functional tasks of platelets are: the formation of a primary platelet plug, the creation of factors leading to vasoconstriction, the activation of one of the blood coagulation systems.
Physiological features in the life cycle of red blood platelets allow us to classify factors leading to thrombocytopenia in:
- reducing the formation of platelets;
- increasing the death of blood platelets;
- leading to redistribution of platelets.
The first factors in turn are divided into: thrombocytopenia caused by hypoplasia of the megakaryocytic germ, thrombocytopenia caused by ineffectiveness of thrombocytopoiesis and thrombocytopenia as a result of replacement of the megakaryocyte germ in the pituitary epiphysis, at the location of the red bone marrow.
The underdevelopment of the megakaryocytic germ results from the inability of the inert brain to start the process of daily replacement. The cause is also aplastic anemia, leading to total hypoplasia of leukocytes, platelets and erythrocytes.
Not the least role in the development of this particular type of thrombocytopenia is played by the use of antibiotics, cytostatics, antithyroid agents and gold preparations. So the group of cytostatics depresses the bone marrow, and antibiotics lead to an increase in the susceptibility of the bone marrow to this group of drugs. Hypoplasia is also caused by alcohol, viral infection, routine vaccination against measles, development of sepsis in patients, prolonged oxygen starvation of tissues and organs of the human body of various etiologies, and others. There are also cases of congenital hypoplasia, as an example - constitutional aplastic anemia.
Thrombocytopenia, caused by inhibition of thrombocytopoiesis, is caused by many causes. One of them is an inadequate amount of thrombopoietin, manifested as a rule at birth. Thrombopoietin is a substance that enhances the maturation of platelets from the megakaryocyte sprout. Currently, this pathology is well diagnosed and amenable to rapid cure.
Another cause that disrupts the formation of platelets is severe iron deficiency anemia . This is because in order to maintain a normal level of platelets in the circulatory system and cyanocobalamin, or vitamin B 12, a certain amount of iron is needed. In addition, the reduction in the processes of thrombocytopoiesis is often associated with a viral infection, with intoxication with alcohol and with hereditary diseases, the etiology of which lies in the maturation of platelets that are unsuitable for qualitative process hemopoiesis.
To the replacement of megakaryocytes of the red bone marrow lead: cancer of the blood with the formation of metastases in its final stage, proliferation of fibrous tissue and granulomas replacing the normal cells of the red bone marrow with its own cells. These pathological conditions cause a decrease in the quantitative content of all formed elements in the blood, including platelets.
Factors that increase the death of platelets can be conditionally classified into immunological and non-immunological ones. This is the most extensive group of causes caused by thrombocytopenia, since the accelerated dissolution of the blood platelets leads to an increase in the content of the structural elements of the circulatory system as a result of uncontrolled neoplasm.
Conventionally, a group of immunological factors can be divided into:
- caused by products of an aploantibodies, or isoimmune thrombocytopenia;
- autoantibody-induced autoimmune products, or autoimmune thrombocytopenia;
- caused by the prescription of medicinal products, or immune thrombocytopenia.
Isoimmune thrombocytopenia arises as a consequence of falling into the bloodstream of the platelets that are not relevant to the body. The reason for this is the asymptomatic thrombocytopenic purpura of newborns and the resistance of recipients to blood transfusions. Purple newborns develops when the mother platelet antigens are incompatible with the antigens of the baby's platelets, resulting in the body of the child getting foreign to his blood system antibodies that destroy platelets. This disease is not common and has a direct dependence on the state of immunity of a woman during pregnancy. The reasons for the formation of resistance of recipients have been little studied, but one can say with certainty that their manifestations are associated with blood transfusion incompatible in terms of blood platelet count.
Autoimmune thrombocytopenia occurs with the death of platelets due to the effect of antibodies on immune complexes. This leads to chronic lymphocytic leukemia , inflammatory diseases of the gastrointestinal tract, human immunodeficiency virus and others.
The factors leading to the progression of immune thrombocytopenia include the administration of antibiotics of the penicillin group, sulfonamides, the use of heroin, morphine and other drugs, the appointment of antiulcer drugs like Ranitidine or cimetidine. By abolishing the drug at the urging of the doctor, the patient self-healing.
The reasons not related to immunity are pathologies such as cardiovascular malformations, metastasis to the blood vessels of the body, replacement of the valvular apparatus of the heart and narrowing of the arterial lumen with a sclerotic plaque. Massive bleeding and the infusion of large volumes of transfusion drugs are also included in this group of factors.
When the redistribution of platelets in the depot of an organ such as the spleen is disturbed, more of the blood platelets accumulate than in the norm. This leads to splenomegaly. The diseases that cause this pathological process include: liver cancer , leukemia , lymphoma , infectious diseases of the respiratory system and others.
The disease manifests itself unexpectedly and in some cases is prone to exacerbations, while in others it has a prolonged course. Classify thrombocytopenia as a heteroimmune and autoimmune form. The first form arises sharply, the second passes into a chronic pathology. But this division is usually considered conditional, since the manifestation of the clinic of the disease can not always be correlated to the specific form of thrombocytopenia.
Begins thrombocytopenia with characteristic skin hemorrhages and bleeding into the mucous membranes. The picture of skin hemorrhages is most often observed in the limbs and trunk region. Sometimes they grab the border of the face and lips and meet at the injection site.
Bleeding after extraction of the tooth is rare, begins immediately after its removal and its duration from several hours to several days. In case of a complete stop to relapse is not inclined.
Such a clinical sign as splenomegaly is expressed in patients with concomitant pathology. It includes autoimmune hemolytic anemia, lymphocytic leukemia, chronic hepatitis. The liver does not increase with thrombocytopenia, and in a small number of patients, as a rule, cervical lymph nodes become inflamed, which leads to an increase in body temperature to subfebrile digits. Joining the clinic of arthralgic syndrome and increasing the rate of erythrocyte sedimentation rate suggests that the patient has a systemic disease of connective tissue, such as lupus erythematosus .
The general analysis of peripheral blood differs from the norm by the presence of a decrease in the quantitative index of platelets. Plasma clotting factors remain within the limits of acceptable figures. The morphology of platelets varies, they increase in size, the cells appear blue, their transformation is observed. The number of reticulocytes rises and erythrocytes are destroyed. Sometimes there is a shift of the leukocyte formula to the left. In most patients with thrombocytopenia, the number of megakaryocytes in the red bone marrow is higher than normal due to extensive bleeding. Blood coagulability time is longer, the fragility of capillaries is positive, blood clotting decreases.
By severity of clinical symptoms, the following degrees of thrombocytopenia are distinguished: mild degree, moderate severity and severe degree.
An easy degree of thrombocytopenia occurs as a result of prolonged and profuse female menstruation, with nasal bleeding and intradermal hemorrhages. At this stage, it is almost impossible to diagnose a disease, it is detected accidentally during routine examinations or because of a blood test when calling a doctor for another disease.
The average degree of thrombocytopenia is manifested by hemorrhagic rash, which has the appearance of numerous pinpoint hemorrhage on the skin and on mucous membranes.
Severe gastrointestinal bleeding causes a severe degree of thrombocytopenia, which occurs when the platelet count drops to 25,000 / μL.
It should be noted that there is secondary thrombocytopenia, as a kind of this pathology. Her clinic completely repeats the clinic of concomitant disease. For example, allergic thrombocytopenia, as a result of the action of allergic antigens on platelets, leading to their damage or complete dissolution.
Thrombocytopenia in pregnancy
It is noted that the number of platelets for pregnancy varies unevenly, because the individual state of the woman's organism is of great importance here. If the pregnant woman is healthy, there may be a slight decrease in platelets. This is due to the decrease in their life span and their increased participation in the process of peripheral circulation. With low platelet counts, prerequisites for the development of thrombocytopenia appear. The reason for this is a decrease in the formation of blood platelets, a high degree of their death or consumption. Clinically, the disease is marked by hemorrhage and subcutaneous hemorrhage. Factors of platelet deficiency are the inadequate nutrition of a pregnant woman, the violation of the immune etiology or chronic blood loss. Thus, platelets are not produced, or they take on an irregular shape.
For the diagnosis of thrombocytopenia, a blood coagulability test is performed. This is a very informative and accurate method. The importance of obtaining information on the content of platelets in the blood is that it makes it possible to avoid serious complications in childbirth. So in a child with a thrombocytopenia of an immune nature in childbirth, the development of internal bleeding is great, the most dangerous of which is cerebral hemorrhage. In such a scenario, the attending physician decides to deliver the surgical procedure surgically. In most cases, this disease does not threaten the health of the baby.
Thrombocytopenia in children
Statistics show that the number of children who contract thrombocytopenia among healthy children is 1 in 20,000 cases. The main risk group includes preschool children, the maximum number of identified patients is observed in winter and spring.
Most often thrombocytopenia is congenital. Such children often have an accelerated function of the spleen, or hypersplenism. This is possible because of the physiological characteristics of the organ. The spleen is a depot for exhausted thrombocytes, where they die and are regenerated. In addition, thrombocytopenia in newborns occurs in an immunological conflict, due to the lack of compatibility between the baby and the pregnant woman on platelet antigens. The sensitizing maternal organism forms antibodies to platelets penetrating through the placenta wall into the bloodstream of the fetus and leading to the destruction of its own platelets. The causes of the acquired thrombocytopenia are intoxication of the body, drug allergy , infectious diseases, high content of thyroid hormones.
Symptomatology in children does not differ significantly from such manifestations in adults. To guard parents should be permanent nasal bleeding, a small-dot rash in the area of the lower limbs of the body, instant manifestation of a hematoma with minor mechanical effects, hematuria. But such conditions do not cause concern, since there is no pain syndrome and significant clinical signs. This is a great danger to the health of young patients, because this is the primary cause of severe bleeding into the internal organs and the brain, which can end fatal.
In children, thrombocytopenia is divided into primary and secondary arisen. The first form is characterized by thrombocytopenic syndrome, accompanied by the absence of another pathology. The secondary form of thrombocytopenia is manifested by complications of the underlying disease. For example, it is cirrhosis of the liver , infection of the human immunodeficiency virus and leukemia.
Based on the degree of influence of the immune complex on the development of the disease in children, distinguish between immune and non-immune thrombocytopenia. Immune thrombocytopenia is associated with the mass death of blood platelets as a result of exposure to antibodies. The state of the child's immunity does not distinguish between platelets and takes them as foreign bodies, which leads to the production of antibodies. There is an allelic, transimmune, heteroimmune and autoimmune variety of this category of disease. Non-immune thrombocytopenia occurs with mechanical damage to platelets.
Autoimmune thrombocytopenia is characterized by the destruction of platelets as a result of the influence of autoantibodies. The main place for their maturation is the spleen. In it, the death of blood plates damaged by autoantibodies passes. In addition, platelets destroy the macrophages of organs such as the liver and lymph nodes. The frequency of the disease, according to statistics, is 1 in 15,000 cases and it is more likely to affect women of the age category from 25 to 55 years.
Autoimmune thrombocytopenia usually has an acute onset, but sometimes there may be a slow development. A typical symptom of autoimmune thrombocytopenia is hemorrhagic petechial rash, localized in the area of skin and mucous membrane. The spots are painless on palpation, the clinical picture of inflammation is absent. In patients, bleeding from the nose, from the internal organs of the gastrointestinal tract and from the uterine cavity is observed. The most serious complication of hemorrhagic diathesis is retinal hemorrhage, leading to loss of vision.
The severity of this syndrome has a dependence on the quantitative content of thrombocytes in the bloodstream. Low figures of the indicator cause spontaneous bleeding. But paradoxically it does not sound, high rates with accompanying fevers or generalized infections can cause severe bleeding.
The clinic of autoimmune thrombocytopenia begins with hemorrhages in the oral mucosa and in the area of contact of the body with clothing. Hemorrhage in the face and conjunctiva is a poor diagnostic sign.
For the diagnosis of autoimmune thrombocytopenia, in addition to taking conventional tests (general blood test, general urine analysis, and others), special tests are performed. The Steffen method is referred to the first test. It consists in the quantitative expression of antiglobulins. The test is insensitive, since the serum of patients' blood is used for its conduct.
A more reliable study of platelets provides a Dickson test. Its essence lies in the counting of antibodies on the cell wall of the blood platelets. Normally, their amount does not exceed 15 × 10-15 g. With this increase, immune thrombocytopenia develops. The method is informative, but time-consuming, and as a result, did not find wide practical application.
Therapy of thrombocytopenia begins with the discharge of hormones, in particular, prescribe prednisolone. The dose is selected individually based on the calculation of 1 ml per 1 kg of body weight of the patient per day. If the disease progresses, this dose is increased by 2 times. The effectiveness of treatment of thrombocytopenia is immediately apparent. First, the clinical picture of hemorrhagic syndrome decreases, then the quantitative index of blood platelets increases. Take the drug until the full effect is obtained. Immediately thereafter, the dose is corrected until complete elimination.
The result of the use of glucocorticosteroids in 90% of cases is positive, but in the remaining proportion of patients there is only a cessation of bleeding, and thrombocytopenia recurs.
Within three to four months after the initiation of therapy, there is an urgent need for prompt removal of the spleen. The efficacy of splenectomy has been proven in 80% of patients. With positive dynamics from glucocorticosteroid therapy, prerequisites for complete cure appear. Operation, as a rule, is carried out in parallel to increase of doses of Prednisolon in 3 and more times. If prior to surgery, it was administered intramuscularly, then before and after the operation it is necessary to switch to intravenous injections. It is only possible to say exactly how successfully the splenectomy passed after at least two years. The treatment with hormones is continued, but the doses are significantly reduced.
The most difficult in the general therapeutic sense is the presence of patients to whom the operation has not yielded significant results. Such patients are prescribed immunosuppressive chemotherapy with cytostatics lasting up to two months. They include Azathioprine, Vincristine sulfate. The presence of systemic lupus erythematosus or other diffuse connective tissue diseases leads to the need for earlier use of immunosuppressants. This is applicable to severe forms of systemic disease. If the patient is young and the degree of lesion is insignificant, then only glucocorticosteroids are used.
It is not rational to take cytotoxic drugs before surgery, because immunosuppressant therapy requires selecting the drug that has the most efficacy for a particular individual, and there is no evidence that the effect of this medication is determined by any criteria. In addition, based on practical knowledge, doctors prescribe to patients high doses of chemotherapy and hormones, which leads to the impossibility of performing an operation to remove the spleen. It should also be noted that this therapy causes mutations, infertility or pathology, inherited.
Acquired thrombocytopenia is not an immune character that is treated symptomatically. So when hemorrhagic syndrome appoint hemostatics, general and local significance. The first include 5% Aminocaproic acid, estrogens, progestinone, androxone and others. The second group consists of a sponge of hemostatic, oxidized cellulose, local cryotherapy.
If the cause of thrombocytopenia are massive bleeding, carry out blood transfusion. Transfusions are subject to "clean" red blood cells, which are selected taking into account the individual characteristics of the organism. Autoimmune thrombocytopenia serves as a contraindication for transfusion of blood-substituting solutions due to the fact that the pathogenesis of this type of disease is the dissolution of the platelet. And this aggravates thrombocytolysis. A prerequisite for successful treatment is the abolition of drugs that interfere with the ability of platelets to form a clot, such as Acetylsalicylic acid, Ibuprofen, Warfarin and many others.
Patients are placed on dispensary records after confirmation of the diagnosis by conducting all necessary clinical and laboratory tests. With all the care collected hereditary anamnesis, determine the morphological signs of platelets in the immediate family.
Children's forms of the disease are treated fairly well by hormonal drugs, and secondary thrombocytopenia requires symptomatic therapy.
It is possible to use folk remedies. A decoction of nettle leaves, hips of wild rose and strawberries helps a lot. With the goal of prevention drink the juice of birch, raspberries and beets. A good result is with honey anemia with walnuts.
Nutrition for thrombocytopenia
A specific diet for patients with a history of thrombocytopenia includes a group of products containing components involved in constructing a supporting structure of blood cells, in the separation and formation of blood cells. Very important is the rejection of substances that inhibit the degree of influence at the stage of hematopoiesis.
The food ration should be rich in protein in a greater degree of plant life, cyanocobalomin, or vitamin B 12, and foods must contain folic and ascorbic acids. The reason for this is that with this pathology there is a violation of purine metabolism. This leads to a decrease in the consumption of products of animal origin. The protein replaces the vegetable food containing soy: beans, peas and others. Food for thrombocytopenia should be dietary, include a variety of vegetables, fruits, berries and greens. Animal fats should be limited and better replaced by sunflower, olive or some other kind of oils.
An indicative list of dishes included in the menu of patients with thrombocytopenia:
- broth chicken, leading to increased appetite;
- bakery products made of rye or wheat flour;
- the first dish should be soup;
- salads in vegetable oil with greens;
- fish dishes from low-fat varieties of fish;
- meat products from poultry meat subject to various heat treatment;
- garnishes are prepared from cereals, pasta;
- There is no restriction to drinks.
In order for the therapeutic diet to be of benefit and help in the therapy of thrombocytopenia, it is important to follow certain rules. First, to control the release of new blood cells from the spleen, thiamine and nitrogen-containing products are needed. Second, the supporting structure of blood cells requires sufficient intake of lysine, methionine, tryptophan, tyrosine, lecithin and choline, belonging to the group of essential amino acids. Third, the separation of blood cells is impossible without such trace elements as cobalt, B vitamins, substances containing ascorbic acid and folic acid. And the fourth, the quality of hematopoiesis depends on the presence of lead, aluminum, selenium and gold in the blood. They have a toxic effect on the body.