Immune thrombocytopenia purpura ICD code. Idiopathic thrombocytopenic purpura (ITP) - description, causes, treatment
The disease begins gradually or acutely with the appearance of hemorrhagic syndrome. The type of bleeding in thrombocytopenic purpura is petechial-spotted (bluish). According to clinical manifestations, two variants of thrombocytopenic purpura are distinguished: “dry” - the patient develops only skin hemorrhagic syndrome; “wet” - hemorrhages combined with bleeding. Pathognomonic symptoms of thrombocytopenic purpura are hemorrhages in the skin, mucous membranes and bleeding. The absence of these signs raises doubts about the correctness of the diagnosis.
Cutaneous hemorrhagic syndrome occurs in 100% of patients. The number of ecchymosis varies from single to multiple. The main characteristics of cutaneous hemorrhagic syndrome in thrombocytopenic purpura are as follows.
- Discrepancy between the severity of hemorrhage and the degree of traumatic impact; their spontaneous appearance is possible (mainly at night).
-Polymorphism of hemorrhagic eruptions (from petechiae to large hemorrhages).
- Polychrome skin hemorrhages (color from purple to blue-greenish and yellow, depending on the prescription of their appearance), which is associated with the gradual conversion of hemoglobin through intermediate stages of decay into bilirubin.
- Asymmetry (no favorite localization) of hemorrhagic elements.
- Painless.
Often there are hemorrhages in the mucous membranes, most often in the tonsils, soft and hard palate. Possible hemorrhages in the eardrum, sclera, vitreous body, fundus.
Hemorrhage in the sclera may indicate the threat of the most severe and dangerous complication of thrombocytopenic purpura - hemorrhage in the brain. As a rule, it occurs suddenly and progresses rapidly. Clinically, cerebral hemorrhage is manifested by headache, dizziness, convulsions, vomiting, and focal neurological symptoms. The outcome of cerebral hemorrhage depends on the volume, localization of the pathological process, timely diagnosis and adequate therapy.
Thrombocytopenic purpura is characterized by bleeding from the mucous membranes. They are often profuse in nature, causing severe posthemorrhagic anemia, which threatens the patient’s life. In children, bleeding most often occurs from the nasal mucosa. Bleeding from the gums is usually less profuse, but it can also become dangerous during tooth extraction, especially in patients with an undiagnosed disease. Bleeding after tooth extraction in thrombocytopenic purpura occurs immediately after the intervention and does not resume after its cessation, unlike late, delayed bleeding in hemophilia. In girls of puberty, severe menorrhagia and metrorrhagia are possible. Gastrointestinal and renal bleeding occur less frequently.
There are no characteristic changes in internal organs with thrombocytopenic purpura. Body temperature is usually normal. Sometimes tachycardia is detected, during auscultation of the heart - systolic murmur at the apex and at Botkin's point, weakening of the first tone, caused by anemia. An enlarged spleen is uncharacteristic and rather excludes the diagnosis of thrombocytopenic purpura.
According to the course, acute (lasting up to 6 months) and chronic (lasting more than 6 months) forms of the disease are distinguished. During the initial examination, it is impossible to determine the nature of the course of the disease. Depending on the degree of manifestation of the hemorrhagic syndrome and blood parameters during the disease, three periods are distinguished: hemorrhagic crisis, clinical remission and clinical-hematological remission.
Hemorrhagic crisis is characterized by severe bleeding syndrome and significant changes in laboratory parameters.
During clinical remission, hemorrhagic syndrome disappears, bleeding time is reduced, secondary changes in the blood coagulation system are reduced, but thrombocytopenia persists, although it is less pronounced than during a hemorrhagic crisis.
Clinical and hematological remission implies not only the absence of bleeding, but also the normalization of laboratory parameters.
In Russia, the International Classification of Diseases, 10th revision (ICD-10) has been adopted as a single normative document for recording morbidity, reasons for the population's visits to medical institutions of all departments, and causes of death.
ICD-10 was introduced into healthcare practice throughout the Russian Federation in 1999 by order of the Russian Ministry of Health dated May 27, 1997. No. 170
The publication of a new revision (ICD-11) is planned by WHO in 2017 2018.
With amendments and additions by WHO.
Processing and translation of changes © mkb-10.com
Thrombocytopenia - description, causes, symptoms (signs), diagnosis, treatment.
Short description
Thrombocytopenia is a low platelet count in the peripheral blood, the most common cause of bleeding. When the platelet count decreases to less than 100 ´ 109/l, the bleeding time lengthens. In most cases, petechiae or purpura appear when the platelet count drops to 20–50 ´ 109/l. Serious spontaneous bleeding (eg, gastrointestinal) or hemorrhagic stroke occurs with thrombocytopenia less than 10 ´ 109/L.
Causes
Thrombocytopenia can occur as a manifestation of drug allergies (allergic thrombocytopenia), caused by the production of antiplatelet antibodies (autoimmune thrombocytopenia), caused by infections, intoxications, thyrotoxicosis (symptomatic).
In newborns, thrombocytopenia can be caused by the penetration of autoantibodies of a sick mother through the placenta (transimmune thrombocytopenia).
Pathology of thrombocytopoiesis The maturation of megakaryocytes is selectively suppressed by thiazide diuretics and other drugs, especially those used in chemotherapy, ethanol. A special cause of thrombocytopenia is ineffective thrombopoiesis associated with the megaloblastic type of hematopoiesis (occurs with a deficiency of vitamin B 12 and folic acid, as well as with myelodysplastic and preleukemic syndromes). Morphologically and functionally abnormal (megaloblastic or dysplastic) megakaryocytes are identified in the bone marrow, giving rise to a pool of defective platelets that are destroyed in the bone marrow. Amegakaryocyte thrombocytopenia is a rare cause of thrombocytopenia caused by a congenital deficiency of megakaryocyte colony-forming units.
Anomalies in the formation of the platelet pool occur when platelets are eliminated from the bloodstream; the most common cause is deposition in the spleen. Under normal conditions, the spleen contains a third of the platelet pool. The development of splenomegaly is accompanied by the deposition of a larger number of cells with their exclusion from the hemostasis system. With a very large size of the spleen, it is possible to deposit 90% of the total platelet pool. The remaining 10% in the peripheral bloodstream has a normal circulation duration.
Increased destruction of platelets in the periphery is the most common form of thrombocytopenia; Such conditions are characterized by a shortened platelet lifespan and an increased number of bone marrow megakaryocytes. These disorders are referred to as immune or non-immune thrombocytopenic purpura Immune thrombocytopenic purpura Idiopathic thrombocytopenic purpura (ITP) is the prototype of thrombocytopenia caused by immune mechanisms (there are no obvious external causes of platelet destruction). See Idiopathic thrombocytopenic purpura Other autoimmune thrombocytopenias caused by the synthesis of antiplatelet antibodies: post-transfusion thrombocytopenia (associated with exposure to isoantibodies), drug-induced thrombocytopenia (for example, caused by quinidine), thrombocytopenia caused by sepsis (the incidence can reach 70%), thrombocytopenia in combination with SLE and other autoimmune diseases. Treatment is aimed at correcting the underlying pathology. It is necessary to stop taking all potentially dangerous drugs. GK therapy is not always effective. Transfused platelets undergo the same accelerated destruction Non-immune thrombocytopenic purpura Infections (eg, viral or malaria) Massive transfusion of preserved blood with low platelet content DIC Prosthetic heart valves Thrombotic thrombocytopenic purpura.
Thrombocytopenia (*188000, Â). Clinical manifestations: macrothrombocytopenia, hemorrhagic syndrome, rib aplasia, hydronephrosis, recurrent hematuria. Laboratory tests: autoantibodies to platelets, shortened platelet life, increased clotting time, normal tourniquet test, defects in the plasma component of hemostasis.
May–Hegglin anomaly (Hegglin syndrome, Â). Macrothrombocytopenia, basophilic inclusions in neutrophils and eosinophils (Döhle bodies).
Epstein syndrome (153650, Â). Macrothrombocytopenia in combination with Allport syndrome.
Fechtner family syndrome (153640, Â). Macrothrombocytopenia, inclusions in leukocytes, nephritis, deafness.
Congenital thrombocytopenia (600588, deletion 11q23.3–qter, Â). Clinical manifestations: congenital dysmegakaryocyte thrombocytopenia, mild hemorrhagic syndrome. Laboratory studies: 11q23.3-qter deletion, increased number of megakaryocytes, giant granules in peripheral blood platelets.
Cyclic thrombocytopenia (188020, Â). Hemorrhagic syndrome, cyclic neutropenia.
Thrombocytopenia Paris–Trousseau (188025, deletion 11q23, defect of the TCPT gene, Â). Clinical manifestations: hemorrhagic syndrome, thrombocytopenia, hypertelorism, ear abnormalities, mental retardation, coarctation of the aorta, developmental delay in the embryonic period, hepatomegaly, syndactyly. Laboratory studies: giant granules in platelets, megakaryocytosis, micromegakaryocytes.
TAR syndrome (from: thrombocytopenia–absent radius - thrombocytopenia and absence of the radius, *270400, r). Congenital absence of the radius in combination with thrombocytopenia (pronounced in children, later smoothed out); thrombocytopenic purpura; in the red bone marrow there are defective megakaryocytes; Anomalies of kidney development and congenital heart disease are sometimes noted.
Symptoms (signs)
The clinical picture is determined by the underlying disease that caused thrombocytopenia.
Diagnostics
Diagnostics Thrombocytopenia is an indication for examining the bone marrow for the presence of megakaryocytes; their absence indicates a violation of thrombocytopoiesis, and their presence indicates either peripheral platelet destruction or (in the presence of splenomegaly) platelet deposition in the spleen. Pathology of thrombocytopoiesis. The diagnosis is confirmed by identifying megakaryocytic dysplasia in a bone marrow smear. Anomalies in the formation of the platelet pool. The diagnosis of hypersplenism is made when moderate thrombocytopenia is detected in the bone marrow smear of a normal number of megakaryocytes and a significant enlargement of the spleen. Diagnosis of idiopathic thrombocytopenic purpura requires the exclusion of diseases that occur with thrombocytopenia (for example, SLE), and thrombocytopenia caused by taking medications (for example, quinidine). Available but nonspecific methods for detecting antiplatelet antibodies are known.
Treatment
Pathology of thrombocytopoiesis. Treatment is based on eliminating the offending agent, if possible, or treating the underlying disease; The half-life of platelets is usually normal, allowing platelet transfusions in the presence of thrombocytopenia and signs of bleeding. Thrombocytopenia caused by a deficiency of vitamin B 12 or folic acid disappears with the restoration of their normal levels.
Amegakaryocytic thrombocytopenia responds well to treatment; antithymocyte immunoglobulin and cyclosporine are usually prescribed.
Anomalies in the formation of the platelet pool. Treatment is usually not given, although a splenectomy may resolve the problem. During transfusions, some platelets are deposited, making transfusions less effective than in states of reduced bone marrow activity.
Treatment of idiopathic thrombocytopenic purpura - see Idiopathic thrombocytopenic purpura.
Complications and associated conditions Reduced platelet production is combined with aplastic anemia, myelophthisis (replacement of bone marrow with tumor cells or fibrous tissue) and some rare congenital syndromes Evans syndrome (Fisher-Evans syndrome) - a combination of autoimmune hemolytic anemia and autoimmune thrombocytopenia.
ICD-10 D69 Purpura and other hemorrhagic conditions
Thrombocytopenia: symptoms and treatment
Thrombocytopenia - the main symptoms:
- Red spots on the skin
- Enlarged lymph nodes
- Fever
- Enlarged lymph nodes in the neck
- Minor hemorrhages on the skin and mucous membranes
- Blue spots on the skin
A disease that causes a decrease in the number of platelets in the blood is called thrombocytopenia. This is exactly what the article will talk about. Platelets are small blood cells that have no color and are significant components involved in blood clotting. The disease is quite serious, since the disease can lead to hemorrhage in the internal organs (especially in the brain), and this is fatal.
Classification
Like most medical diseases, thrombocytopenia has its own classification, which is formed on the basis of pathogenetic factors, causes, symptoms and various manifestations.
According to the criterion of etiology, two types of disease are distinguished:
They are characterized by the fact that the primary type manifests itself as an independent illness, and the secondary type is provoked by a number of other diseases or pathological abnormalities.
According to the duration of the disease in the human body, there are two types of malaise: acute and chronic. Acute - characterized by a short duration of impact on the body (up to six months), but manifested by immediate symptoms. The chronic form is characterized by a prolonged decrease in platelets in the blood (over six months). It is the chronic type that is more dangerous, since treatment takes up to two years.
According to the criteria for the severity of the disease, characterized by the quantitative composition of platelets in the blood, three degrees are distinguished:
- I - composition is 150–50x10 9 /l - the severity criterion is satisfactory;
- II - 50–20x10 9 /l - reduced composition, which manifests itself with minor damage to the skin;
- III - 20x10 9 /l - characterized by the appearance of internal bleeding in the body.
The norm of blood cells in the body is equal to / mkl. But it is in the female body that these indicators are constantly changing. Changes are influenced by the following factors:
Platelets appear in the body from the bone marrow, which synthesizes blood cells by stimulating megakaryocytes. The synthesized blood plates circulate through the blood for seven days, after which the process of their stimulation is repeated.
According to the International Classification of Diseases of the Tenth Convocation (ICD-10), this disease has its own codes:
- D50-D89 - diseases of the circulatory system and other types of insufficiency.
- D65-D69 - blood clotting disorders.
Causes
Often the cause of the disease is an allergic reaction of the body to various medications, resulting in drug-induced thrombocytopenia. With such ailment, the body produces antibodies directed against the drug. Medicines that affect the occurrence of blood cell insufficiency include sedatives, alkaloids and antibacterial agents.
The causes of deficiency may also be problems with the immune system caused by the consequences of blood transfusions.
Especially often the disease manifests itself when there is a mismatch of blood groups. Most often observed in the human body is autoimmune thrombocytopenia. In this case, the immune system is unable to recognize its platelets and rejects them from the body. As a result of rejection, antibodies are produced to remove foreign cells. The causes of such thrombocytopenia are:
- Pathological kidney failure and chronic hepatitis.
- Lupus, dermatomyositis and scleroderma.
- Leukemic diseases.
If the disease has a pronounced form of an isolated disease, then it is called idiopathic thrombocytopenia or Werlhof's disease (ICD-10 code: D69.3). The etiology of idiopathic thrombocytopenic purpura (ICD-10:D63.6) remains unclear, but medical scientists are inclined to believe that the cause is a hereditary predisposition.
The manifestation of the disease is also typical in the presence of congenital immunodeficiency. Such people are most susceptible to the factors causing the disease, and the reasons for this are:
- damage to the red bone marrow from exposure to drugs;
- immunodeficiency leads to damage to megakaryocytes.
There is a productive nature of the disease, which is caused by insufficient production of platelets by the bone marrow. In this case, their insufficiency occurs, which ultimately develops into malaise. The causes are considered to be myelosclerosis, metastases, anemia, etc.
A lack of platelets in the body is observed in people with a reduced composition of vitamin B12 and folic acid. Excessive radioactive or radiation exposure to cause blood cell deficiency cannot be ruled out.
Thus, two types of causes influencing the occurrence of thrombocytopenia can be distinguished:
- Leading to the destruction of blood cells: idiopathic thrombocytopenic purpura, autoimmune disorders, cardiac surgery, clinical circulatory disorders in pregnant women and side effects of drugs.
- Contributing to a decrease in the production of antibodies by the bone marrow: viral influences, metastatic manifestations, chemotherapy and radiation, as well as excessive alcohol consumption.
Symptoms
Symptoms of thrombocytopenia disease have different types of manifestations. It depends:
- firstly, from the cause of occurrence;
- secondly, on the nature of the disease (chronic or acute).
The main signs of damage to the body are manifestations on the skin in the form of hemorrhages and bleeding. Hemorrhages are most often observed on the limbs and torso. Damage to the face and lips of a person is not excluded. To illustrate the manifestation of hemorrhages on the human body, the photo below is presented.
Thrombocytopenia is characterized by symptoms of prolonged hemorrhages after tooth extraction. Moreover, the duration of hemorrhage can be either one-day or accompanied for several days. It depends on the degree of the disease.
With symptoms, there is no increase in the size of the liver, but very often doctors observe an enlargement of the lymph nodes of the cervical region. This phenomenon is often accompanied by an increase in body temperature to subfebrile levels (from 37.1 to 38 degrees). An increase in the rate of red blood cell accumulation in the body is evidence of the presence of a disease called lupus erythematosus.
Symptoms of platelet deficiency are quite easily observed after taking blood for analysis. The quantitative composition will differ significantly from the maximum standards. When the number of platelets in the blood decreases, their size increases. This is reflected on the skin as the appearance of red and bluish spots, which indicates the transformation of blood cells. There is also destruction of red blood cells, which leads to a decrease in the quantitative composition, but at the same time the number of reticulocytes increases. There is a phenomenon of a shift in the leukocyte formula to the left.
The human body with a reduced composition of blood cells is characterized by an increase in the composition of megakaryocytes, which is caused by frequent and extensive bleeding. The duration of blood clotting is noticeably increased, and the reduction in clotting of blood released from the wound is reduced.
According to the symptoms of the disease, three degrees of complications are distinguished: mild, moderate and severe.
Mild degrees are typical causes of the disease in women with prolonged and heavy menstruation, as well as with intradermal bleeding and nasal hemorrhages. But at the mild stage, diagnosing the disease is extremely difficult, so the presence of the disease can be confirmed only after a detailed medical examination.
The average degree is characterized by the appearance of a hemorrhagic rash throughout the body, which consists of numerous pinpoint hemorrhages under the skin and on the mucous membrane.
Severe grades are characterized by gastrointestinal disorders caused by hemorrhages. The platelet count in the blood is up to 25x10 9 /l.
Symptoms of secondary thrombocytopenia have similar features.
Pregnancy and illness: symptoms
Thrombocytopenia in pregnant women is characterized by significant changes in the quantitative composition of cells in the blood of women. If there is no diagnosis of the disease in pregnant women, but the platelet composition indicator decreases slightly, this indicates that their vital activity is decreasing and their participation in the periphery of the blood circulation is increasing.
If there is a reduced composition of platelets in the blood of a pregnant woman, then these are direct prerequisites for the development of the disease. The reasons for the reduced number of platelets are the high rates of death of these bodies and low rates of formation of new ones. Clinical signs are characterized by subcutaneous hemorrhages. The causes of insufficiency of colorless cells are incorrect composition and nutritional standards or a small amount of food consumption, as well as damage to the immune system and various blood losses. Through this process, the corpuscles are produced by the bone marrow in small quantities or have an irregular shape.
Thrombocytopenia during pregnancy is very dangerous, so the issue of diagnosis, and especially treatment, is given maximum attention. The danger is that a lack of platelets in the mother’s blood during pregnancy contributes to hemorrhage in the child. The most dangerous hemorrhage in the womb is cerebral, the result of which is characterized by fatal consequences for the fetus. At the first sign of such a factor, the doctor makes a decision about premature birth in order to eliminate the consequences.
Childhood thrombocytopenia: symptoms
Thrombocytopenia in children is quite rare. The risk group includes school-age children, whose incidence is more common in winter and spring.
Thrombocytopenia and its symptoms in children are practically no different from adults, but it is important for parents to diagnose it based on the first signs in the early stages of development of the disease. Children's symptoms include frequent bleeding from the nasal cavity and the appearance of small rashes on the body. Initially, the rash occurs on the lower extremities of the body, and then they can be observed on the hands. With minor bruises, swelling and hematomas occur. Such signs most often do not cause concern in parents, due to the absence of pain symptoms. This is an important mistake, because any disease in its advanced form is dangerous.
Bleeding gums indicate a lack of platelets in the blood, both in a child and in adults. Cal at the same time in a sick person, and more often in children, is excreted together with blood clots. Hemorrhage due to urination cannot be ruled out.
Depending on the degree of impact of the disease on the immune system, a distinction is made between immune and non-immune platelet deficiency. Immune thrombocytopenia is caused by massive death of blood cells under the influence of antibodies. In such a situation, the immune system’s own blood cells are not recognized and are rejected from the body. Non-immune disease manifests itself through physical impact on blood platelets.
Diagnostics
A person should be diagnosed at the first signs and symptoms of the disease. The main method of diagnosis is a clinical blood test, the results of which show a picture of the quantitative composition of platelets.
If a deviation in the number of blood cells in the body is detected, an indication is given for a bone marrow examination. Thus, the presence of megakaryocytes is determined. If they are absent, then thrombus formation is impaired, and their presence indicates the destruction of platelets or their deposition in the spleen.
The causes of deficiency are diagnosed using:
- genetic tests;
- electrocardiograms;
- tests for the presence of antibodies;
- ultrasound examinations;
- X-ray and endoscopy.
Thrombocytopenia during pregnancy is diagnosed using a coagulogram, or, in simple terms, a blood clotting test. This analysis allows you to accurately determine the composition of platelets in the blood. The course of the birth process depends on the number of platelets.
Treatment
Treatment of thrombocytopenia begins with therapy, in which a drug called Prednisolone is prescribed in the hospital.
Important! Treatment methods are prescribed strictly by the attending physician only after undergoing an appropriate examination and diagnosing the disease.
The dosage of the medication is indicated in the instructions, according to which 1 ml of the drug is taken per 1 kg of body weight. As the disease progresses, the dose increases by 1.5–2 times. In the initial stages, the ailment is characterized by a quick and effective recovery, so after taking the drug, you can notice an improvement in health within a few days. The drug continues until the person is completely cured, which must be confirmed by the attending physician.
The effect of glucocorticosteroids has a positive effect on the fight against malaise, but in most cases only the symptoms disappear, and the disease remains. Used to treat deficiency in children and adolescents.
Treatment of idiopathic chronic thrombocytopenia is carried out by removing the spleen. This procedure in medicine is called splenectomy and is characterized by its positive effects. In advance of surgery, the dosage of Prednisolone is increased threefold. Moreover, it is injected not into a muscle, but directly into a human vein. After splenectomy, administration of the drug in the same doses continues for up to two years. Only after the specified period has passed is an examination and certification of the success of the splenectomy performed.
If the removal operation is unsuccessful, the patient is prescribed immunosuppressive chemotherapy with cytostatics. These drugs include: Azathioprine and Vincristine.
When an acquired deficiency of a non-immune nature is diagnosed, thrombocytopenia is treated symptomatically by taking estrogens, progestins and androxons.
More severe forms of idiopathic thrombocytopenia are caused by excessive hemorrhages. A transfusion is performed to restore blood. Treatment of severe cases requires discontinuation of medications that may negatively affect the ability of platelets to form clots.
After diagnosing the disease, the patient is registered and an examination procedure takes place not only of the patient, but also of his relatives to collect a hereditary history.
In children, the malaise can be treated well and without complications, but in some cases the possibility of symptomatic therapy cannot be ruled out.
Treatment of thrombocytopenia using traditional medicine also has its considerable achievements. First of all, to get rid of the problem of platelet deficiency in the blood, you should include honey and walnuts in your diet. Decoctions of nettle and rosehip leaves also help well. For preventive measures, birch, raspberry or beet juice is used.
If you think that you have Thrombocytopenia and the symptoms characteristic of this disease, then a hematologist can help you.
We also suggest using our online disease diagnostic service, which selects probable diseases based on the entered symptoms.
Diphtheria is an infectious disease provoked by exposure to a specific bacterium, the transmission of which (infection) is carried out by airborne droplets. Diphtheria, the symptoms of which are the activation of the inflammatory process mainly in the nasopharynx and oropharynx, is also characterized by concomitant manifestations in the form of general intoxication and a number of lesions that directly affect the excretory, nervous and cardiovascular systems.
Measles is an acute infectious disease, the degree of susceptibility to which is almost 100%. Measles, the symptoms of which include fever, an inflammatory process affecting the mucous membranes of the oral cavity and upper respiratory tract, the appearance of a maculopapular rash on the skin, general intoxication and conjunctivitis, is one of the main causes of mortality among young children.
Leptospirosis is an infectious disease that is caused by specific pathogens of the Leptospira genus. The pathological process primarily affects the capillaries, as well as the liver, kidneys and muscles.
Pharyngomycosis (tonsillomycosis) is a pathology of the mucous membrane of the pharynx of an acute or chronic nature, the main reason for the development of which is infection of the body by fungi. Pharyngomycosis affects people from absolutely all age groups, including young children. Rarely does the disease occur in an isolated form.
Toxic erythema is a disease, as a result of the progression of which a polymorphic rash appears on the human skin. The disease most often affects newborn children, but its occurrence in adult patients is possible. Erythema toxicum of the newborn develops in 50% of babies in the first few days of their life. This condition reflects the child’s adaptation process to the environment, as well as to external factors.
With the help of exercise and abstinence, most people can do without medicine.
Symptoms and treatment of human diseases
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Coding of thrombocytopenia according to ICD 10
Platelets play a vital role in the human body and are a group of blood cells.
- 0 – purpura caused by an allergic reaction;
- 1 – defects in the structure of platelets with a normal number;
- 2 – purpura of another, non-thrombocytopenic origin (in case of poisoning);
- 3 – idiopathic thrombocytopenic purpura;
- 4 – other primary platelet deficiencies;
- 5 – secondary lesions;
- 6 – unspecified variants of pathologies;
- 7 – other types of hemorrhages (pseudogemophilia, increased fragility of blood vessels, and so on);
- 8 – unspecified hemorrhagic conditions.
This group of diseases is located under the heading of pathologies of the blood, hematopoietic organs and immune disorders of cellular origin.
Danger of thrombocytopenia
Due to the severity of clinical manifestations, thrombocytopenia in the international classification of diseases contains emergency care protocols for severe hemorrhagic syndromes.
A danger to life with a strong decrease in the number of platelets appears even when scratches appear, since the wound is not healed by primary blood clots and continues to bleed.
People with a lack of white blood cells can die from spontaneous internal hemorrhages, so the disease requires timely diagnosis and adequate treatment.
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Self-medication can be hazardous to your health. At the first sign of disease, consult a doctor.
Secondary thrombocytopenia
Definition and general information [edit]
Drug-induced immune thrombocytopenia is most often caused by antibodies to the drug that cross-react with platelet antigens. Less commonly, the drug is fixed on platelets to form a complete antigen, where it serves as a hapten and platelets serve as a carrier.
The drugs that most often cause thrombocytopenia are listed in Table. 16.5.
Heparin-induced thrombocytopenia is a heparin-induced, immune-mediated prothrombotic disorder accompanied by thrombocytopenia and venous and/or arterial thrombosis.
Approximately 1% of patients develop heparin-induced thrombocytopenia for at least one week after heparin use, and approximately 50% of these experience thrombosis. Heparin-induced thrombocytopenia is slightly more common in women.
Etiology and pathogenesis[edit]
Heparin-induced thrombocytopenia results from a humoral immune reaction directed against a complex involving endogenous platelet factor 4 and exogenous heparin; autoantibodies recognize endogenous platelet factor 4 only when it is combined with heparin. This immune complex activates circulating platelets through their surface FcγRIIA receptors, leading to thrombocytopenia and hypercoagulability. The characteristics of heparin (bovine > porcine), its composition (unfractionated > low molecular weight > fondaparinux), dose (prophylactic > therapeutic > single), route of administration (subcutaneous > intravenous) and duration of administration (more than 4 days > less than 4 days) are all factors determining the development and severity of thrombocytopenia.
Clinical manifestations[edit]
With drug-induced thrombocytopenia, petechiae, gastrointestinal bleeding and hematuria usually appear several hours after drug administration. The duration of thrombocytopenia depends on the rate of drug elimination. Usually 7 days after its discontinuation, the platelet count returns to normal.
Heparin-induced thrombocytopenia can occur at any age (> 3 months), but cases are rare in children. Moderate thrombocytopenia usually begins 5-10 days after heparin administration. If the patient has already been exposed to heparin within the last 100 days, a rapid reaction may occur, with a drop in platelet count occurring within minutes to hours of heparin administration. Delayed heparin-induced thrombocytopenia is also possible; thrombocytopenia develops after discontinuation of the drug. Thrombocytopenia is usually asymptomatic and bleeding is rare. Heparin-induced thrombocytopenia is associated with a high risk of thrombotic complications (eg, pulmonary embolism, myocardial infarction, thrombotic stroke) with a strong predilection for arterial thrombosis of the extremity arteries and deep vein thrombosis. Additional microvascular thrombosis can lead to the development of venous gangrene/limb amputation. Other complications include skin necrosis at heparin injection sites and anaphylactoid reactions (eg, fever, hypotension, joint pain, dyspnea, cardiopulmonary failure) following intravenous bolus administration.
Secondary thrombocytopenia: Diagnosis[edit]
The diagnosis of heparin-induced thrombocytopenia can be suspected based on the clinical picture - thrombocytopenia, thrombosis, the absence of another cause of thrombocytopenia. The diagnosis is confirmed by the detection of antibodies to the endogenous platelet factor 4/heparin complex and is confirmed by the detection of pathological platelet-activating antibodies using a serotonin release assay or a heparin-induced platelet activation test.
Differential diagnosis[edit]
Differential diagnoses include non-immune heparin-associated thrombocytopenia (due to the direct interaction of heparin with circulating platelets occurring in the first days after heparin administration), as well as postoperative hemodilution, sepsis, non-heparin-induced thrombocytopenia, disseminated intravascular coagulation, and multiple organ failure.
Secondary thrombocytopenia: Treatment[edit]
For some patients receiving heparin, regular monitoring of platelet counts is recommended. If heparin-induced thrombocytopenia is suspected or confirmed, treatment is to discontinue heparin and use an alternative anticoagulant, either non-heparin anti-factor Xa (danaparoid, fondaparinux) or direct thrombin inhibitors (eg, argatroban, bivalirudin). Warfarin is contraindicated during the acute thrombocytopenic phase because it can cause microvascular thrombosis, with the potential for necrosis of the ischemic limb (venous gangrene syndrome). Thrombocytopenia usually resolves after an average of 4 days, with values greater than 150 x 109/L, although in some cases it may take 1 week to 1 month.
The prognosis for platelet count recovery is good, but postthrombotic complications may occur (eg, limb amputation in 5-10% of patients, stroke, bilateral hemorrhagic adrenal necrosis with adrenal insufficiency). Mortality from heparin-induced thrombocytopenia (eg, fatal pulmonary embolism) occurs in 5-10% of cases.
Prevention[edit]
Other [edit]
Thrombocytopenic purpura caused by red blood cell transfusion
1. Clinical picture. Thrombocytopenic purpura is a rare complication of red blood cell transfusion. It is manifested by sudden thrombocytopenia, bleeding from the mucous membranes and petechiae, which occur 7-10 days after transfusion. The diagnosis is based on medical history. This form of thrombocytopenic purpura most often occurs in multiparous women and people who have undergone multiple red blood cell transfusions. According to the mechanism of development, it is similar to thrombocytopenia of newborns caused by maternal antibodies. Thrombocytopenic purpura, caused by red blood cell transfusion, occurs in individuals who lack the Zw a antigen. It has been shown that this antigen is part of glycoprotein IIb/IIIa. Transfusion of red blood cells mixed with platelets carrying the Zw a antigen leads to the appearance of antibodies to this antigen. It is believed that they cross-react with glycoprotein IIb/IIIa of the patient’s own platelets.
A. Platelet transfusions are not performed because they are usually ineffective. In addition, platelet donors in this disease can only be 2% of people whose platelets do not carry the Zw a antigen.
b. Prednisone, 1-2 mg/kg/day orally, reduces hemorrhagic syndrome and increases the platelet count.
V. The disease goes away on its own after the patient’s blood is freed from the donor’s platelets.
d. Subsequently, red blood cells from donors who lack the Zw a antigen should be used for transfusion.
The code must contain an additional digit after the period, which will clarify the diagnosis:
- 0 – purpura caused by an allergic reaction;
- 1 – defects in the structure of platelets with a normal number;
- 2 – purpura of another, non-thrombocytopenic origin (in case of poisoning);
- 3 – idiopathic thrombocytopenic purpura;
- 4 – other primary platelet deficiencies;
- 5 – secondary lesions;
- 6 – unspecified variants of pathologies;
- 7 – other types of hemorrhages (pseudogemophilia, increased fragility of blood vessels, and so on);
- 8 – unspecified hemorrhagic conditions.
This group of diseases is located under the heading of pathologies of the blood, hematopoietic organs and immune disorders of cellular origin.
Danger of thrombocytopenia
Due to the severity of clinical manifestations, thrombocytopenia in the international classification of diseases contains emergency care protocols for severe hemorrhagic syndromes.
A danger to life with a strong decrease in the number of platelets appears even when scratches appear, since the wound is not healed by primary blood clots and continues to bleed.
People with a lack of white blood cells can die from spontaneous internal hemorrhages, so the disease requires timely diagnosis and adequate treatment.
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Secondary thrombocytopenia
Definition and general information [edit]
Drug-induced immune thrombocytopenia is most often caused by antibodies to the drug that cross-react with platelet antigens. Less commonly, the drug is fixed on platelets to form a complete antigen, where it serves as a hapten and platelets serve as a carrier.
The drugs that most often cause thrombocytopenia are listed in Table. 16.5.
Heparin-induced thrombocytopenia is a heparin-induced, immune-mediated prothrombotic disorder accompanied by thrombocytopenia and venous and/or arterial thrombosis.
Approximately 1% of patients develop heparin-induced thrombocytopenia for at least one week after heparin use, and approximately 50% of these experience thrombosis. Heparin-induced thrombocytopenia is slightly more common in women.
Etiology and pathogenesis[edit]
Heparin-induced thrombocytopenia results from a humoral immune reaction directed against a complex involving endogenous platelet factor 4 and exogenous heparin; autoantibodies recognize endogenous platelet factor 4 only when it is combined with heparin. This immune complex activates circulating platelets through their surface FcγRIIA receptors, leading to thrombocytopenia and hypercoagulability. The characteristics of heparin (bovine > porcine), its composition (unfractionated > low molecular weight > fondaparinux), dose (prophylactic > therapeutic > single), route of administration (subcutaneous > intravenous) and duration of administration (more than 4 days > less than 4 days) are all factors determining the development and severity of thrombocytopenia.
Clinical manifestations[edit]
With drug-induced thrombocytopenia, petechiae, gastrointestinal bleeding and hematuria usually appear several hours after drug administration. The duration of thrombocytopenia depends on the rate of drug elimination. Usually 7 days after its discontinuation, the platelet count returns to normal.
Heparin-induced thrombocytopenia can occur at any age (> 3 months), but cases are rare in children. Moderate thrombocytopenia usually begins 5-10 days after heparin administration. If the patient has already been exposed to heparin within the last 100 days, a rapid reaction may occur, with a drop in platelet count occurring within minutes to hours of heparin administration. Delayed heparin-induced thrombocytopenia is also possible; thrombocytopenia develops after discontinuation of the drug. Thrombocytopenia is usually asymptomatic and bleeding is rare. Heparin-induced thrombocytopenia is associated with a high risk of thrombotic complications (eg, pulmonary embolism, myocardial infarction, thrombotic stroke) with a strong predilection for arterial thrombosis of the extremity arteries and deep vein thrombosis. Additional microvascular thrombosis can lead to the development of venous gangrene/limb amputation. Other complications include skin necrosis at heparin injection sites and anaphylactoid reactions (eg, fever, hypotension, joint pain, dyspnea, cardiopulmonary failure) following intravenous bolus administration.
Secondary thrombocytopenia: Diagnosis[edit]
The diagnosis of heparin-induced thrombocytopenia can be suspected based on the clinical picture - thrombocytopenia, thrombosis, the absence of another cause of thrombocytopenia. The diagnosis is confirmed by the detection of antibodies to the endogenous platelet factor 4/heparin complex and is confirmed by the detection of pathological platelet-activating antibodies using a serotonin release assay or a heparin-induced platelet activation test.
Differential diagnosis[edit]
Differential diagnoses include non-immune heparin-associated thrombocytopenia (due to the direct interaction of heparin with circulating platelets occurring in the first days after heparin administration), as well as postoperative hemodilution, sepsis, non-heparin-induced thrombocytopenia, disseminated intravascular coagulation, and multiple organ failure.
Secondary thrombocytopenia: Treatment[edit]
For some patients receiving heparin, regular monitoring of platelet counts is recommended. If heparin-induced thrombocytopenia is suspected or confirmed, treatment is to discontinue heparin and use an alternative anticoagulant, either non-heparin anti-factor Xa (danaparoid, fondaparinux) or direct thrombin inhibitors (eg, argatroban, bivalirudin). Warfarin is contraindicated during the acute thrombocytopenic phase because it can cause microvascular thrombosis, with the potential for necrosis of the ischemic limb (venous gangrene syndrome). Thrombocytopenia usually resolves after an average of 4 days, with values greater than 150 x 109/L, although in some cases it may take 1 week to 1 month.
The prognosis for platelet count recovery is good, but postthrombotic complications may occur (eg, limb amputation in 5-10% of patients, stroke, bilateral hemorrhagic adrenal necrosis with adrenal insufficiency). Mortality from heparin-induced thrombocytopenia (eg, fatal pulmonary embolism) occurs in 5-10% of cases.
Prevention[edit]
Other [edit]
Thrombocytopenic purpura caused by red blood cell transfusion
1. Clinical picture. Thrombocytopenic purpura is a rare complication of red blood cell transfusion. It is manifested by sudden thrombocytopenia, bleeding from the mucous membranes and petechiae, which occur 7-10 days after transfusion. The diagnosis is based on medical history. This form of thrombocytopenic purpura most often occurs in multiparous women and people who have undergone multiple red blood cell transfusions. According to the mechanism of development, it is similar to thrombocytopenia of newborns caused by maternal antibodies. Thrombocytopenic purpura, caused by red blood cell transfusion, occurs in individuals who lack the Zw a antigen. It has been shown that this antigen is part of glycoprotein IIb/IIIa. Transfusion of red blood cells mixed with platelets carrying the Zw a antigen leads to the appearance of antibodies to this antigen. It is believed that they cross-react with glycoprotein IIb/IIIa of the patient’s own platelets.
A. Platelet transfusions are not performed because they are usually ineffective. In addition, platelet donors in this disease can only be 2% of people whose platelets do not carry the Zw a antigen.
b. Prednisone, 1-2 mg/kg/day orally, reduces hemorrhagic syndrome and increases the platelet count.
V. The disease goes away on its own after the patient’s blood is freed from the donor’s platelets.
d. Subsequently, red blood cells from donors who lack the Zw a antigen should be used for transfusion.
Purpura and other hemorrhagic conditions (D69)
Excluded:
- benign hypergammaglobulinemic purpura (D89.0)
- cryoglobulinemic purpura (D89.1)
- idiopathic (hemorrhagic) thrombocythemia (D47.3)
- fulminant purpura (D65)
- thrombotic thrombocytopenic purpura (M31.1)
In Russia, the International Classification of Diseases, 10th revision (ICD-10) has been adopted as a single normative document for recording morbidity, reasons for the population's visits to medical institutions of all departments, and causes of death.
ICD-10 was introduced into healthcare practice throughout the Russian Federation in 1999 by order of the Russian Ministry of Health dated May 27, 1997. No. 170
The publication of a new revision (ICD-11) is planned by WHO in 2017 2018.
With amendments and additions by WHO.
Processing and translation of changes © mkb-10.com
ICD code: D69.6
Thrombocytopenia, unspecified
Thrombocytopenia, unspecified
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Thrombocytopenia and platelet dysfunction
A disorder of the blood system, in which an insufficient number of platelets circulates in it - cells that provide hemostasis and play a key role in the process of blood clotting, is defined as thrombocytopenia (ICD-10 code - D69.6).
Why is thrombocytopenia dangerous? A reduced concentration of platelets (less than 150 thousand/μl) impairs blood clotting so much that there is a threat of spontaneous bleeding with significant blood loss at the slightest damage to blood vessels.
Platelet diseases include abnormally elevated platelet levels (thrombocythemia in myeloproliferative disorders, thrombocytosis as a reactive phenomenon), decreased platelet levels - thrombocytopenia, and platelet dysfunction. Any of these conditions, including a condition with an increase in platelet levels, can cause impaired hemostatic clot formation and bleeding.
Platelets are fragments of megakaryocytes that provide hemostasis to circulating blood. Thrombopoietin is synthesized by the liver in response to a decrease in the number of bone marrow megakaryocytes and circulating platelets and stimulates the bone marrow to synthesize platelets from megakaryocytes. Platelets circulate in the bloodstream for 7-10 days. About 1/3 of platelets are temporarily deposited in the spleen. The normal platelet count is 40,000/µl. However, the platelet count may vary slightly depending on the phase of the menstrual cycle, decrease in late pregnancy (gestational thrombocytopenia) and increase in response to inflammatory cytokines of the inflammatory process (secondary or reactive thrombocytosis). The platelets are ultimately destroyed in the spleen.
ICD-10 code
Causes of thrombocytopenia
Causes of thrombocytopenia include impaired platelet production, increased platelet sequestration in the spleen with normal platelet survival, increased platelet destruction or consumption, platelet dilution, and a combination of the above. Increased platelet sequestration in the spleen suggests the presence of splenomegaly.
The risk of bleeding is inversely proportional to the platelet count. A platelet count of less than/µL easily causes minor bleeding and increases the risk of major bleeding. With platelet levels between i/µl, bleeding may occur even with minor trauma; when the platelet level is less than / µl, spontaneous bleeding is possible; when the platelet level is less than 5000/μl, severe spontaneous bleeding is likely to develop.
Platelet dysfunction can occur when there is an intracellular defect in the platelet abnormality or when there is an external influence that damages the function of normal platelets. Dysfunction can be congenital or acquired. Of the congenital disorders, von Willebrand disease is the most common and intracellular platelet defects are less common. Acquired disorders of platelet function are often caused by various diseases, taking aspirin or other medications.
Other causes of thrombocytopenia
Platelet destruction can occur due to immune causes (HIV infection, drugs, connective tissue diseases, lymphoproliferative diseases, blood transfusions) or as a result of non-immune causes (Gram-negative sepsis, acute respiratory distress syndrome). Clinical and laboratory features are similar to those found in idiopathic thrombocytopenic purpura. Only a study of the medical history can confirm the diagnosis. Treatment is associated with correction of the underlying disease.
Acute respiratory distress syndrome
Patients with acute respiratory distress syndrome may develop nonimmune thrombocytopenia, possibly due to platelet deposition in the capillary beds of the lungs.
Blood transfusions
Post-transfusion purpura is caused by immune destruction similar to ITP, except for the presence of a history of blood transfusion within 3 to 10 days. Patients are predominantly women and lack platelet antigen (PLA-1), which is present in most people. Transfusions of PLA-1-positive platelets stimulate the production of PLA-1 antibodies, which (mechanism unknown) may react with the patient's PLA-1-negative platelets. The result is severe thrombocytopenia, which resolves within 2-6 weeks.
Connective tissue and lymphoproliferative diseases
Connective tissue (eg, SLE) and lymphoproliferative diseases can cause immune thrombocytopenia. Glucocorticoids and splenectomy are often effective.
Drug-induced immune destruction
Quinidine, quinine, sulfonamides, carbamazepine, methyldopa, aspirin, oral antidiabetic drugs, gold salts, and rifampin can cause thrombocytopenia, usually due to an immune reaction in which the drug binds to the platelet to form a new “foreign” antigen. This condition is indistinguishable from ITP except for the history of drug use. When you stop taking the medication, your platelet count increases within 7 days. Gold-induced thrombocytopenia is an exception, since gold salts can remain in the body for many weeks.
Thrombocytopenia develops in 5% of patients receiving unfractionated heparin, which is possible even when very low doses of heparin are given (for example, when flushing an arterial or venous catheter). The mechanism is usually immune. Bleeding may occur, but more often platelets form aggregates that cause vascular occlusion with the development of paradoxical arterial and venous thrombosis, sometimes life-threatening (for example, thrombotic occlusion of arterial vessels, stroke, acute myocardial infarction). Heparin should be discontinued in all patients who develop thrombocytopenia or a decrease in platelet count by more than 50%. Since 5 days of heparin is sufficient to treat venous thrombosis, and most patients begin taking oral anticoagulants at the same time as taking heparin, heparin withdrawal is usually safe. Low molecular weight heparin (LMWH) is less immunogenic than unfractionated heparin. However, LMWH is not used for heparin-induced thrombocytopenia because most antibodies cross-react with LMWH.
Gram-negative sepsis
Gram-negative sepsis often causes nonimmune thrombocytopenia, which corresponds to the severity of the infection. Thrombocytopenia can be caused by many factors: disseminated intravascular coagulation, the formation of immune complexes that can interact with platelets, complement activation, and platelet deposition on damaged endothelial surfaces.
HIV infection
Patients infected with HIV may develop immune thrombocytopenia, similar to ITP, except in association with HIV. The platelet count can be raised by administering glucocorticoids, which are often withheld until the platelet count drops below 1/µL, as these drugs can further reduce immunity. The platelet count also usually increases after the use of antiviral drugs.
Pathogenesis of thrombocytopenia
The pathogenesis of thrombocytopenia lies either in the pathology of the hematopoietic system and a decrease in platelet production by myeloid cells of the bone marrow (megakaryocytes), or in impaired hemodieresis and increased destruction of platelets (phagocytosis), or in sequestration pathologies and platelet retention in the spleen.
In the bone marrow of healthy people, an average of platelets are produced daily, but not all of them circulate in the systemic circulation: reserve platelets are stored in the spleen and released when needed.
When a patient's examination does not reveal diseases that cause a decrease in platelet levels, a diagnosis of thrombocytopenia of unknown origin or idiopathic thrombocytopenia is made. But this does not mean that the pathology arose “just like that.”
Thrombocytopenia, associated with a decrease in platelet production, develops with a lack of vitamins B12 and B9 (folic acid) in the body and aplastic anemia.
Leukopenia and thrombocytopenia are combined with impaired bone marrow function associated with acute leukemia, lymphosarcoma, and cancer metastases from other organs. Suppression of platelet production may be due to changes in the structure of hematopoietic stem cells in the bone marrow (the so-called myelodysplastic syndrome), congenital hypoplasia of hematopoiesis (Fanconi syndrome), megakaryocytosis or myelofibrosis of the bone marrow.
Symptoms of thrombocytopenia
Platelet disorders result in a typical bleeding pattern of multiple petechiae on the skin, usually more prominent on the legs; scattered small ecchymoses in places of minor injuries; bleeding of mucous membranes (nosebleeds, bleeding in the gastrointestinal tract and genitourinary tract; vaginal bleeding), severe bleeding after surgical interventions. Severe bleeding in the gastrointestinal tract and central nervous system can be life-threatening. However, manifestations of significant tissue bleeding (eg, deep visceral hematoma or hemarthrosis) are atypical for platelet pathology and suggest the presence of disorders of secondary hemostasis (eg, hemophilia).
Autoimmune thrombocytopenia
The pathogenesis of increased platelet destruction is divided into immune and non-immune. And the most common is autoimmune thrombocytopenia. The list of immune pathologies in which it manifests itself includes: idiopathic thrombocytopenia (immune thrombocytopenic purpura or Werlhof's disease), systemic lupus erythematosus, Sharpe's or Sjögren's syndromes, antiphospholipid syndrome, etc. All these conditions are united by the fact that the body produces antibodies that attack its own healthy cells, including platelets.
It should be borne in mind that when antibodies from a pregnant woman with immune thrombocytopenic purpura enter the bloodstream of the fetus, transient thrombocytopenia is detected in the child during the neonatal period.
According to some reports, antibodies against platelets (their membrane glycoproteins) can be detected in almost 60% of cases. The antibodies have immunoglobulin G (IgG), and as a consequence, platelets become more vulnerable to increased phagocytosis by splenic macrophages.
Congenital thrombocytopenia
Many deviations from the norm and their result - chronic thrombocytopenia - have a genetic pathogenesis. The protein thrombopoietin, synthesized in the liver, encoded on chromosome 3p27, stimulates megakaryocytes, and is responsible for the effect of thrombopoietin on a specific receptor protein encoded by the C-MPL gene.
It is assumed that congenital thrombocytopenia (in particular, amegakaryocytic thrombocytopenia), as well as hereditary thrombocytopenia (with familial aplastic anemia, Wiskott-Aldrich syndrome, May-Hegglin syndrome, etc.) is associated with a mutation of one of these genes. For example, an inherited mutant gene produces persistently activated thrombopoietin receptors, which causes overproduction of abnormal megakaryocytes that are unable to produce sufficient platelets.
The average lifespan of circulating platelets is 7-10 days; their cell cycle is regulated by the antiapoptotic membrane protein BCL-XL, which is encoded by the BCL2L1 gene. In principle, the function of BCL-XL is to protect cells from damage and induced apoptosis (death), but it turns out that when the gene is mutated, it acts as an activator of apoptotic processes. Therefore, platelet destruction may occur faster than platelet formation.
But hereditary disaggregation thrombocytopenia, characteristic of hemorrhagic diathesis (Glanzmann's thrombasthenia) and Bernard-Soulier syndrome, has a slightly different pathogenesis. Due to a gene defect, thrombocytopenia is observed in young children, associated with a disorder in the structure of platelets, which deprives them of the ability to “stick together” to form a blood clot, which is necessary to stop bleeding. In addition, such defective platelets are rapidly utilized in the spleen.
Secondary thrombocytopenia
By the way, about the spleen. Splenomegaly - an increase in the size of the spleen - develops for various reasons (due to liver pathologies, infections, hemolytic anemia, obstruction of the hepatic vein, infiltration of tumor cells in leukemia and lymphomas, etc.), and this leads to the fact that it can retain up to a third of the total platelet mass. The result is a chronic disorder of the blood system, which is diagnosed as symptomatic or secondary thrombocytopenia. When this organ is enlarged, in many cases splenectomy for thrombocytopenia or, simply put, removal of the spleen for thrombocytopenia is indicated.
Chronic thrombocytopenia can also develop due to hypersplenic syndrome, which means hyperfunction of the spleen, as well as premature and too rapid destruction of blood cells by its phagocytes. Hypersplenism is secondary in nature and most often occurs due to malaria, tuberculosis, rheumatoid arthritis or tumor. So, in fact, secondary thrombocytopenia becomes a complication of these diseases.
Secondary thrombocytopenia is associated with bacterial or systemic viral infection: Epstein-Barr virus, HIV, cytomegavirus, parvovirus, hepatitis, varicella-zoster virus (the causative agent of chickenpox), or rubivirus (which causes rubella measles).
When the body (directly on the bone marrow and its myeloid cells) is exposed to ionizing radiation and consumes large amounts of alcohol, secondary acute thrombocytopenia can develop.
Thrombocytopenia in children
According to studies, in the second trimester of pregnancy the level of platelets in the fetus exceeds 150 thousand/μl. Thrombocytopenia in newborns is present after 1-5% of births, and severe thrombocytopenia (when platelets are less than 50 thousand / μl) occurs in 0.1-0.5% of cases. At the same time, a significant proportion of infants with this pathology are born prematurely or have had placental insufficiency or fetal hypoxia. In 15-20% of newborns, thrombocytopenia is alloimmune - as a result of receiving antibodies to platelets from the mother.
Neonatologists consider genetic defects of bone marrow megakaryocytes, congenital autoimmune pathologies, the presence of infections, as well as DIC syndrome (disseminated intravascular coagulation) to be other causes of thrombocytopenia.
Most cases of thrombocytopenia in older children are symptomatic, and possible pathogens include fungi, bacteria, and viruses, such as cytomegalovirus, toxoplasma, rubella, or measles. Acute thrombocytopenia occurs especially often with a fungal or gram-negative bacterial infection.
Vaccinations for thrombocytopenia in children are given with caution, and in severe forms of the pathology, preventive vaccination by injection and cutaneous applications (with skin scarification) may be contraindicated.
Thrombocytopenia during pregnancy
Thrombocytopenia during pregnancy can have many causes. However, it must be taken into account that the average platelet count during pregnancy decreases (up to 215 thousand / μl), and this is normal.
Firstly, in pregnant women, a change in the number of platelets is associated with hypervolemia - a physiological increase in blood volume (on average by 45%). Secondly, platelet consumption during this period is increased, and bone marrow megakaryocytes produce not only platelets, but also significantly more thromboxane A2, which is necessary for platelet aggregation during blood coagulation (clotting).
In addition, in the α-granules of pregnant platelets, the dimeric glycoprotene PDGF, a platelet-derived growth factor, is intensively synthesized, which regulates cell growth, division and differentiation, and also plays a critical role in the formation of blood vessels (including in the fetus).
As obstetricians note, asymptomatic thrombocytopenia is observed in approximately 5% of pregnant women during normal gestation; in 65-70% of cases, thrombocytopenia of unknown origin occurs. 7.6% of pregnant women experience a moderate degree of thrombocytopenia, and 15-21% of women with preeclampsia and gestosis develop severe thrombocytopenia during pregnancy.
Classification of thrombocytopenias
Impaired platelet production Reduction or absence of megakaryocytes in the bone marrow.
Decreased platelet production despite the presence of megakaryocytes in the bone marrow
Leukemia, aplastic anemia, paroxysmal nocturnal hemoglobinuria (in some patients), myelosuppressive drugs.
Alcohol-induced thrombocytopenia, thrombocytopenia in megaloblastic anemia, HIV-associated thrombocytopenia, myelodysplastic syndrome
Sequestration of platelets in an enlarged spleen
Cirrhosis with congestive splenomegaly, myelofibrosis with myeloid metaplasia, Gaucher disease
Increased platelet destruction or immune platelet destruction
Idiopathic thrombocytopenic purpura, HIV-associated thrombocytopenia, post-transfusion purpura, drug-induced thrombocytopenia, neonatal alloimmune thrombocytopenia, connective tissue diseases, lymphoproliferative diseases
Destruction not caused by immune mechanisms
Disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, thrombocytopenia in acute respiratory distress syndrome
Massive blood transfusions or exchange transfusions (loss of platelet viability in stored blood)
Thrombocytopenia due to sequestration in the spleen
Increased sequestration of platelets in the spleen occurs in various diseases accompanied by splenomegaly. It appears in patients with congestive splenomegaly caused by advanced cirrhosis. The platelet count is usually high until the disease causing splenomegaly impairs platelet production (eg, myelofibrosis with myeloid metaplasia). During stress, platelets are released from the spleen after exposure to adrenaline. Therefore, thrombocytopenia, caused only by sequestration of platelets in the spleen, does not lead to increased bleeding. Splenectomy normalizes thrombocytopenia, but its implementation is not indicated until there is severe thrombocytopenia, additionally caused by a disorder of hematopoiesis.
Drug-induced thrombocytopenia
Drug-induced or drug-induced thrombocytopenia is due to the fact that many common pharmacological drugs can affect the blood system, and some can suppress the production of megakaryocytes in the bone marrow.
The list of drugs that cause thrombocytopenia is quite extensive, and includes antibiotics and sulfonamides, analgesics and NSAIDs, thiazide diuretics and antiepileptic drugs based on valproic acid. Transient, that is, transient thrombocytopenia can be provoked by interferons, as well as proton pump inhibitors (used in the treatment of gastric and duodenal ulcers).
Thrombocytopenia after chemotherapy is also a side effect of antitumor cytostatic drugs (Methotrexate, Carboplatin, etc.) due to their inhibition of the functions of hematopoietic organs and myelotoxic effects on the bone marrow.
And heparin-induced thrombocytopenia develops due to the fact that heparin, used for the treatment and prevention of deep vein thrombosis and pulmonary embolism, is a direct anticoagulant, that is, it reduces platelet aggregation and prevents blood clotting. The use of heparin causes an idiosyncratic autoimmune reaction, which is manifested by the activation of platelet factor-4 (PF4 cytokine protein), which is released from the α-granules of activated platelets and binds to heparin to neutralize its effect on the endothelium of blood vessels.
Degrees of thrombocytopenia
It should be remembered that the platelet count is considered normal from 150 thousand/µl to 450 thousand/µl; and there are two pathologies associated with platelets: thrombocytopenia, discussed in this publication, and thrombocytosis, in which the number of platelets exceeds the physiological norm. Thrombocytosis has two forms: reactive and secondary thrombocythemia. The reactive form may develop after removal of the spleen.
Degrees of thrombocytopenia range from mild to severe. With a moderate degree, the level of circulating platelets is 100 thousand/μl; for moderately severe – thousand/µl; in severe cases – below 50 thousand/µl.
According to hematologists, the lower the level of platelets in the blood, the more severe the symptoms of thrombocytopenia. With a mild degree, the pathology may not show anything, but with a moderate degree, a rash appears on the skin (especially on the legs) due to thrombocytopenia - these are pinpoint subcutaneous hemorrhages (petechiae) of red or purple color.
If the platelet count is below thousand/µl. Spontaneous formation of hematomas (purpura), bleeding from the nose and gums occurs.
Acute thrombocytopenia is often a consequence of infectious diseases and has spontaneous resolution within two months. Chronic immune thrombocytopenia lasts longer than six months, and often its specific cause remains unclear (thrombocytopenia of unknown origin).
For extremely severe thrombocytopenia (with platelet count
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Idiopathic thrombocytopenic purpura (ITP)- a disease of unknown etiology, characterized by the development of thrombocytopenia and hemorrhagic syndrome. Most often, platelet destruction is caused by an autoimmune process provoked by an infectious agent or drug intake. Predominant age- up to 14 years old. Predominant gender- female.
Code according to the international classification of diseases ICD-10:
- D69.3
Causes
Genetic Aspects. Hereditary thrombocytopenic autoimmune idiopathic purpura has been described (188030, B), manifested by hemorrhagic syndrome, thrombocytopenia, and the presence of antibodies to platelets.
Classification. Downstream: acute (less than 6 months), chronic (more than 6 months). Periods of the disease.. Period of exacerbation (crisis).. Clinical remission.. Clinical and hematological remission. According to the clinical picture... Dry (isolated skin manifestations) .. Wet (addition of bleeding from the mucous membranes).
Clinical picture
. Acute onset with hemorrhagic syndrome. It is possible to increase body temperature to subfebrile values.
. The patient's condition often does not change significantly.
. Petechial - ecchymotic rash localized on the buttocks, inner thighs, chest, face.
. A positive pinch symptom is also possible in the stage of clinical remission.
. Bleeding from mucous membranes. The most common occurrence is intense nosebleeds; in girls of puberty - uterine bleeding.
. Internal bleeding in the gastrointestinal tract and central nervous system is extremely rare.
Laboratory research. CBC: posthemorrhagic anemia, thrombocytopenia. OAM - possible hematuria due to renal bleeding. Immunogram: increase in CEC content. Myelogram: “irritation” of the megakaryocyte lineage, the appearance of “inactive” megakaryocytes.
Treatment
TREATMENT
Mode bed rest for severe thrombocytopenia.
Diet with the exception of obligate allergens.
Drug therapy
. For bleeding - etamsylate, aminocaproic acid, carbazochrome, hemostatic agents for topical use, for intense nosebleeds - nasal tamponade. For uterine bleeding - oxytocin (as prescribed by the gynecologist).
. Antihistamines.
. Vitamin B15, Eleutherococcus.
. GC, for example prednisolone.. Indications - wet form of ITP, abundant skin manifestations on the face, scalp with platelet content less than 0.051012/l.. Prescribed in courses of 2-3 mg/kg/day for 5-7 days with breaks of 5-7 days.. Indication for cancellation is clinical and hematological remission by the first day of the next course.. If thrombocytopenia persists and there is no hemorrhagic syndrome, therapy is stopped after 4-5 courses.
. Inosine; orotic acid, potassium salt; lipoic acid.
. Immunosuppressive therapy - effectiveness is questionable.
. Alternative drugs. Recombinant IFN preparations for chronic conditions.. Induction: 3 million units/m2 3 r/week. The duration of the course depends on the “response” (recovery period and platelet count). Maintenance therapy for 12 weeks.
. Intravenous IgG infusion is a new and effective method to increase platelet counts during an acute attack. ATs block Fc receptors of phagocytes, which play an important role in antiplatelet cytotoxic reactions; this method has gained popularity as a preoperative preparation for patients with ITP requiring surgical intervention. A new method of treating refractory ITP, which has given encouraging preliminary results, is plasmapheresis through a column with staphylococcal proteins.
Operative treatment. Splenectomy is indicated for the chronic form with severe bleeding and unsuccessful conservative therapy. Cure with splenectomy does not always occur. Occlusion of splenic vessels.
Observation. In the stage of clinical remission - control of platelet levels 1 time per month. If clinical and hematological remission lasts more than 5 years, the patient is removed from the register.
Recommendations. Observation by a hematologist at the place of residence. Physiotherapeutic treatment and insolation are contraindicated. The use of acetylsalicylic acid and carbenicillin is contraindicated. Prevention of bleeding - herbal medicine (infusions of chamomile, nettle, rosehip) in courses of 15 days every 3 months. Physical education and sports should be avoided. Registration of disability in case of persistent chronic disease.
Complications. Hemorrhages in the central nervous system. Severe posthemorrhagic anemia.
Course and prognosis. In most patients (80-90%), self-healing occurs within 1-6 months. When the process is chronic, the treatment regimen with prednisolone is similar. The mortality rate for ITP is less than 1%. Causes of death- hemorrhages in the central nervous system, severe posthemorrhagic anemia.
Reduction. ITP - idiopathic thrombocytopenic purpura
ICD-10. D69.3 Idiopathic thrombocytopenic purpura
