Thrombocytopenia. Causes, symptoms, diagnosis and treatment.

Thrombocytopenia

– a pathological condition characterized by a decrease in the number of platelets per unit volume of blood and the resulting increased bleeding. Platelets play an important role in stopping bleeding. The molecules of the outer membrane of this flat blood element are able to recognize damaged areas of blood vessels and integrate into the structure of the capillaries, turning into a kind of “patch”. A decrease in the concentration of platelets in the bloodstream leads to a large number of pinpoint hemorrhages.

Classification of the disease

Thrombocytopenia has its own classification, based on a number of factors, symptoms and manifestations. The mechanism of the initiation and development of thrombocytopenia may be associated with the characteristics of the life cycle of blood platelets:

• decreased platelet formation in the red bone marrow.
• increased destruction of platelets.
• redistribution of platelets in the hematopoietic system due to inflammatory processes in the human body.

According to the etiology, primary and secondary thrombocytopenia are distinguished. First type

manifests itself as an independent disease.
The second type
is as a consequence of pathological abnormalities. Thrombocytopenia can have an acute and chronic course. The acute condition lasts a short time (up to 6 months) and is characterized by a vivid clinical picture. A chronic condition may occur with a gradual increase in symptoms. The severity of the disease depends on the number of platelets in the blood and is conventionally divided into three degrees - mild, moderate and severe. Each of them has its own manifestations - from frequent nosebleeds to hemorrhagic rash (purpura) and extensive bleeding into internal organs.

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Treatment

Bleeding does not develop with mild thrombocytopenia. Drug treatment is usually not required. A wait-and-see approach and identification of the cause of the platelet decrease is recommended. With moderate severity of the disease, hemorrhages may appear in the oral mucosa, increased bleeding of the gums, and increased frequency of nosebleeds. With bruises and injuries, extensive hemorrhages may form in the skin that do not correspond to the amount of damage. Drug therapy is recommended only if there are factors that increase the risk of bleeding (ulcers of the gastrointestinal system, professional activities or sports associated with frequent injuries). For thrombocytopenia of moderate severity without pronounced manifestations of hemorrhagic syndrome, treatment at home is prescribed. Patients are advised to limit their active lifestyle for the period of treatment and take all medications prescribed by the hematologist. Patients with severe thrombocytopenia are subject to mandatory hospitalization. Such patients, in addition to medication, can be prescribed various therapeutic and surgical measures aimed at eliminating thrombocytopenia and the causes that caused it. Additional methods of treating thrombocytopenia are: transfusion therapy (transfusion of donor blood, plasma or platelets into the patient), removal of the spleen and bone marrow transplant.

Causes of thrombocytopenia

Among the various reasons causing this serious disease, the main ones are:

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. The disease manifests itself especially often when blood groups do not match. Autoimmune thrombocytopenia is most often observed in the human body . 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. If the disease has a pronounced form of an isolated disease, then it is called idiopathic thrombocytopenia or Werlhof's disease . It is believed that such thrombocytopenia occurs due to a hereditary predisposition. The manifestation of the disease is also typical in the presence of congenital immunodeficiency. 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.

Symptoms of thrombocytopenia

The clinical picture of thrombocytopenia depends on the cause of this pathology, as well as the severity of the disease. The main signs are multiple hemorrhages on the skin, as well as spontaneous bleeding - nasal, pulmonary, uterine. Other manifestations of the disease:

Publications in the media

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 when thrombocytopenia is less than 10´109/L.

Etiology and pathogenesis

• 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 •• 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 deficiency of vitamin B12 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 •• Amegakaryocytic 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 platelet destruction 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 nonimmune thrombocytopenic purpura • • Immune thrombocytopenic purpura • • • Idiopathic thrombocytopenic purpura (ITP) is the prototype of thrombocytopenia due to immune mechanisms (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 • • Nonimmune thrombocytopenic purpura ••• Infections (eg, viral or malaria) ••• Massive transfusion of preserved blood with a low platelet count ••• DIC ••• Prosthetic heart valves ••• Thrombotic thrombocytopenic purpura .

Genetic aspects

• 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, 155100, 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: t hrombocytopenia – a bsent r adius - 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.

The clinical picture is determined by the underlying disease that caused thrombocytopenia.

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 destruction of platelets, 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 • Abnormalities in the formation of the platelet pool. The diagnosis of hypersplenism is made when there is moderate thrombocytopenia, a normal number of megakaryocytes is detected in the bone marrow smear, and a significant enlargement of the spleen • Diagnosis of idiopathic thrombocytopenic purpura requires the exclusion of diseases associated 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 deficiency of vitamin B12 or folic acid disappears when their normal levels are restored.

• Amegakaryocytic thrombocytopenia responds well to treatment; antithymocyte immunoglobulin and cyclosporine are usually prescribed.

• Anomalies in the formation of the platelet pool. There is usually no treatment, although 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 • Decreased platelet production is associated with aplastic anemia, myelophthisis (replacement of bone marrow by 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

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Neonatal alloimmune thrombocytopenia and genetic syndromes: clinical cases

• Shulakova Olga Alexandrovna
• Karabanov Andrey Mikhailovich
• Zyryanov Sergey Kensarinovich
• Gurevich Konstantin Georgievich

Summary The article presents 2 clinical cases of a combination of neonatal alloimmune thrombocytopenia with genetic syndromes (Edwards and Shereshevsky-Turner syndromes).
The principles of diagnosis and treatment of a threatening condition for children in the neonatal period - neonatal alloimmune thrombocytopenia - are presented. Key words: thrombocytopenia, alloimmune thrombocytopenia, Edwards syndrome, Shereshevsky-Turner syndrome

Neonatology: news, opinions, training. 2021. T. 7. No. 1. P. 83-87. DOI: 10.24411/2308-2402-2018-00011

Thrombocytopenia in the neonatal period occurs in approximately 35% of children treated in neonatal units [1], but its true frequency remains unknown. The platelet level in the fetus reaches 150x109/L by the end of the first trimester, so any newborn (23-42 weeks of gestation) with a platelet level <150x109/L has thrombocytopenia [2].

Clinically significant thrombocytopenia, i.e. in which the likelihood of developing hemorrhagic syndrome is considered significant - a decrease in platelets to a level of <50x109/l, and therefore mild (100-150x109/l), moderate (50-100x109/l) and severe (<50x109/l) forms are distinguished thrombocytopenia. According to the time of occurrence, thrombocytopenia is divided into early (before 72 hours of life) and late (after 72 hours of life), which is important when making a differential diagnosis [3].

Early thrombocytopenia is, as a rule, thrombocytopenia associated with fetoplacental problems that arise in utero. They are usually milder and very often remain undiagnosed in newborns. The late form is more typical for severe infectious processes, sepsis, necrotizing enterocolitis, often being severe and requiring multiple transfusions of platelet concentrates [1].

One of the most common causes of early neonatal thrombocytopenia is neonatal alloimmune thrombocytopenia, a relatively rare but potentially dangerous disease in newborns, leading to the destruction of platelets in the fetus and newborn by maternal antibodies and threatening the development of severe complications in the child. The disease occurs with a frequency of 1:3000-5000 newborns, regardless of the mother’s obstetric history [4].

During the observation, it was noted that the hemorrhagic syndrome that occurs with neonatal immune thrombocytopenia is always more severe than with the development of immune thrombocytopenia in an adult, but the reason for this is unknown [4]. The main target of maternal antibodies is the glycoprotein of the child’s platelet membrane. During pregnancy, a woman develops sensitization to fetal platelet membrane antigens. The resulting maternal antibodies, penetrating the placenta, cause adhesion and lysis of platelets in the fetus and newborn. This process can develop starting from 24 weeks of gestation [5].

The main clinical manifestation of neonatal alloimmune thrombocytopenia is the development of hemorrhagic syndrome of varying severity - from the occurrence of cutaneous hemorrhagic syndrome (petechial and small-spotted rash on the skin and mucous membranes) in the first 72 hours after birth to the development of severe forms (10-12%). In severe cases, hemorrhagic syndrome intensifies in the first hours and days and can be represented by bleeding from injection sites, gastrointestinal, pulmonary bleeding and intracranial hemorrhage. Thus, 10% of children with neonatal alloimmune thrombocytopenia die as a result of hemorrhage in vital organs [3].

Most often, neonatal alloimmune thrombocytopenia is benign in nature and resolves on its own within 4-12 weeks after birth, which is why mild forms of thrombocytopenia often remain undiagnosed.

To confirm the diagnosis of neonatal alloimmune thrombocytopenia, it is necessary to conduct additional laboratory immunological tests aimed at detecting platelet-associated antibodies. In the demonstration of clinical case data, the determination of platelet-associated antibodies was carried out by radioimmunoassay using blood samples from the child, mother and father.

The main method of treatment is currently considered to be intravenous administration of immunoglobulins, the use of which significantly reduces the duration of the disease. Studies have proven that the use of randomly selected donor platelets does not have a positive clinical effect, therefore, if necessary, it is recommended to use washed maternal platelets [6, 7].

There are a number of genetic diseases for which thrombocytopenia can be part of the symptom complex, for example, trisomy 13 chromosome (Patau syndrome), trisomy 18 chromosome (Edwards syndrome), trisomy 21 chromosome (Down syndrome), Shershev syndrome Sky-Turner [8-10]. Thus, with Edwards syndrome, thrombocytopenia occurs in 83% of children [11].

However, the combination of neonatal alloimmune thrombocytopenia with genetic diseases has not been described in the literature. This article demonstrates 2 cases of the development of neonatal alloimmune thrombocytopenia in children with a syndromic form of pathology (Edwards and Shershevsky-Turner syndromes).

Clinical case 1

A girl I was admitted to the department.

at the age of 6 days.

From the anamnesis it is known that the child was born to a 22-year-old mother suffering from urolithiasis; the woman was not observed or examined during pregnancy. According to the words, the pregnancy proceeded with the development of an acute respiratory viral infection with a rise in temperature in the second trimester, otherwise without any peculiarities. A child from the 4th pregnancy (the 1st pregnancy in 2011 ended in the birth of a healthy child at term, the 2nd and 3rd pregnancies ended in a medical abortion at the request of the mother). A girl from the second surgical birth by emergency cesarean section at 42 weeks due to lack of labor and decompensation of fetoplacental blood flow, chronic fetal hypoxia. The amniotic fluid is green. Birth weight 2060 g, length 44 cm. Apgar score 3/5 points. The condition at birth was assessed as severe due to intrapartum asphyxia, respiratory failure of the third degree, congenital heart disease (?), and manifestations of immaturity by gestational age. From birth, artificial pulmonary ventilation (ALV) was performed. Convulsive syndrome from the 1st day of life. Prevention of hemorrhagic disease of the newborn was carried out.

At the end of the 1st day of life, the child developed hemorrhagic syndrome in the form of grade I-II intraventricular hemorrhage and skin hemorrhagic manifestations (petechial rash and bleeding from injection sites), the hemogram showed a platelet level of 53x109/l, a transfusion of fresh frozen plasma was performed for hemostatic purposes . On the 5th day of life, a repeated episode of hemorrhagic syndrome in the form of pulmonary hemorrhage, thrombocytopenia in the hemogram up to 37x109/l, was transfused with platelet concentrate.

During an examination in the maternity hospital, congenital heart disease was revealed (multiple ventricular septal defects, supraventricular narrowing of the aorta, anomalies of the subvalvular apparatus of the mitral and tricuspid valves, patent foramen ovale), congenital malformation of the central nervous system [Dandy-Walker syndrome (?), cerebellar hypoplasia ]. Considering multiple congenital malformations, intrauterine growth retardation, and a phenotype characteristic of Edwards syndrome, blood was taken for cytogenetic examination.

On the 6th day of life, the child was transferred from the maternity hospital to the neonatal intensive care unit. Upon admission, the child’s condition was severe due to the course of the infectious process (congenital pneumonia, necrotizing enterocolitis degree I, OU - conjunctivitis), neurological symptoms (convulsive syndrome), respiratory disorders (continued mechanical ventilation), circulatory disorders 2A degree, hemorrhagic manifestations (petechial rash, contact bleeding of mucous membranes).

Upon examination, the child's unusual phenotype was determined: low-set ears pointed upward, almond-shaped eyes, epicanthus, micrognathia, skeletal features: rotation of the xiphoid process of the sternum, flipper-shaped deformity of the upper limbs, unusual arrangement of fingers and toes, navicular feet.

In the hemogram: platelets - 42x109/l, leukopenia up to 5.0x109/l with a pronounced shift of the blood count to the left (metamyelocytes - 4%, myelocytes - 3%, band neutrophils - 24%, segmented neutrophils - 26%). The level of acute phase proteins is sharply increased: C-reactive protein - 60.2 mg/l, procalcitonin - 32.34 ng/l. The level of thrombocytopenia was regarded as a manifestation of a severe infectious process against the background of possible Edwards syndrome. Antibacterial and antifungal therapy was adjusted (ceftriaxone and vancomycin prescribed in the maternity hospital were canceled, meropenem and linezolid were added to therapy, and antifungal therapy with amphotericin).

However, given the appearance of thrombocytopenia on the 1st day of life, unknown maternal and family history, it was decided to conduct a study to determine the level of platelet-associated antibodies.

On the 3rd day of the child’s stay in the department (9th day of life), the platelet level decreased to 22x109/l with the development of hemorrhagic syndrome in the form of gastric bleeding, hemostasis was corrected with fresh frozen plasma, the effect was achieved.

A positive result was obtained about the alloimmune nature of thrombocytopenia (according to the results of radioimmunological analysis, the level of platelet-associated antibodies was 310%, the norm was up to 200%). The patient was treated with intravenous IgG immunoglobulins for 3 days at a total dose of 1.5 g/kg.

In dynamics, on the 6th day of hospitalization (12th day of life) the platelet level was 39x109/l, on the 9th day (15th day of life) - 88x109/l, on the 17th day (23rd day of life) ) - 190x109/l, against the background of persistent phenomena of the inflammatory process. The hemorrhagic syndrome did not recur over time.

On the 13th day of the child's stay in the department, a response was received from the cytogenetic laboratory confirming Edwards syndrome.

During treatment of the manifestations of infectious toxicosis with a decrease, the child was transferred to independent breathing on the 28th day of life, with radiological resolution of pneumonia. After relief of enteral insufficiency, nutrition was expanded to the age-appropriate volume, feeding was carried out through a nasogastric tube. Anticonvulsant and supportive cardiotonic therapy were selected. The girl was transferred to the palliative care department with a clinical diagnosis: intrauterine infection, pneumonia, necrotizing enterocolitis of the newborn I, OU - conjunctivitis. Candidiasis of the skin and mucous membranes. Neonatal alloimmune thrombocytopenia. Congenital malformation of the central nervous system: Dandy-Walker syndrome. Brain ischemia. Hemorrhagic brain damage: intraventricular hemorrhage of I-II degree. Congenital heart disease: multiple ventricular septal defects, supraventricular narrowing of the aorta. Left ventricular hypertrophy. Open oval window. Circulatory disorders 2A degree. Stage III intrauterine growth retardation. Edwards syndrome.

Clinical case 2

Girl F.,

transferred from the maternity hospital to stage II of nursing on the 3rd day of life.

From the anamnesis it is known that the child is from a 32-year-old mother suffering from polycystic ovary syndrome and a carrier of herpes simplex virus type I. From the 2nd spontaneous pregnancy (the 1st pregnancy in 2007 ended in spontaneous miscarriage in the early stages), was registered at the antenatal clinic from 6 weeks. Course of pregnancy: I trimester - without any peculiarities, II trimester - acute respiratory viral infection without fever, III trimester - exacerbation of herpes simplex virus type I (no therapy was carried out). Throughout her pregnancy, the woman suffered from urogenital candidiasis.

A child from the first birth by emergency cesarean section at the 37th week (foot presentation of the fetus), spontaneous discharge of amniotic fluid. The amniotic fluid is clear, the anhydrous interval is 10 hours. Body weight at birth is 2120 g, length is 43 cm. Apgar score is 7/8 points. The condition at birth was assessed as severe due to neurological symptoms and respiratory failure. Prevention of hemorrhagic disease of the newborn was carried out.

On the 3rd day of life, the child was transferred from the maternity hospital for further treatment and examination. The child’s condition upon admission is severe due to the course of the infectious process (intrauterine pneumonia, necrotizing enterocolitis NA), respiratory failure, neurological symptoms (depression syndrome), hemorrhagic syndrome (slight petechial rash in the fading stage) against the background of intrauterine growth retardation.

Upon examination, the child's phenotypical appearance attracts attention: pillow-shaped hands and feet, a wing-shaped fold on a short neck, widely spaced nipples.

Upon admission, an additional examination was carried out: the hemogram showed a platelet level of 20x109/l, leukocyte level of 10.8x109/l. Acute phase proteins (C-reactive protein and procalcitonin) are negative; coagulogram - no changes; Ultrasound examination of the abdominal organs, retroperitoneal space and pelvis revealed a cord-like uterus and echo signs of doubling of the collecting system of the left kidney. Ultrasound of the brain was unremarkable.

Based on the examination of the child and the results of the examination, Shershevsky-Turner syndrome was suspected, and blood was taken to determine the karyotype. Taking into account severe thrombocytopenia (with moderate hemorrhagic syndrome), maternal history data (when she was discharged from the maternity hospital, the platelet level was 160x109/l), it was decided to conduct a study to determine the level of platelet-associated antibodies.

On the 4th day of life, the level of platelets in the hemogram was 11x109/l, the hemorrhagic syndrome did not increase. Considering the course of the intrauterine infection and the suspicion of neonatal alloimmune thrombocytopenia in the child, it was decided to begin a course of treatment with intravenous immunoglobulins.

On the 5th day of life, a laboratory result was obtained confirming that thrombocytopenia in the newborn is alloimmune in nature (the level of platelet-associated antibodies is 420%, the norm is up to 200%). In this regard, the course of intravenous IgG immunoglobulins was continued up to 1.5 g/kg.

On the 6th day of life, when the platelet level in the general blood test was 45x109/l, gastric bleeding developed, and fresh frozen plasma was transfused for hemostatic purposes. The bleeding has stopped. A repeat ultrasound examination of the brain was performed - there was no evidence of bleeding.

In dynamics, on the 8th day of life the platelet level was 69x109/l, on the 11th - 148x109/l, on the 15th - 251x109/l.

On the 16th day of life, a karyotyping result corresponding to Shershevsky-Turner syndrome -45, X0 was obtained.

By the 20th day of life, the child's condition was assessed as satisfactory. Inflammatory changes in the hemogram have been stopped, the platelet level is normal. The hemorrhagic syndrome did not recur. The symptoms of respiratory and enteral failure have been relieved. Assimilates food in the required volume and sucks independently. The child was discharged home with a diagnosis of intrauterine infection - pneumonia, necrotizing enterocolitis NA. Neonatal alloimmune thrombocytopenia. Cerebral ischemia grade I-II, depression syndrome. Neonatal jaundice. Duplication of the collecting system of the right kidney. Shershevsky-Turner syndrome. Intrauterine growth restriction.

Conclusion

In conclusion, it is worth noting that the obvious explanation of thrombocytopenia (severe infection or characteristic changes for the syndromic form of the pathology) is not always true. In some cases, a broader additional examination is necessary. Frequent use of intravenous immunoglobulins in newborns during severe infectious processes and sepsis can often mask a disease such as neonatal alloimmune thrombocytopenia.

LITERATURE

1. Del Vecchio A. Evaluation and management of thrombocytopenic neonates in intensive care unit // Early Hum. Dev. 2014. Vol. 90. P. 51-55.

2. Pahal GS, Jauniaux E, Kinnon C, Thrasher AJ et al. Normal development of human fetal hematopoiesis between eight and seventeen weeks' gestation // Am. J. Obstet. Gynecol. 2000. Vol. 183. R. 1029-1034.

3. Ols R., Eder M. Hematology, immunology and infectious diseases. M.: Logosfera, 2013. pp. 22-35.

4. Zdravic D., Yougbare I., Vadasz B., Li C. et al. Fetal and neonatal alloimmune thrombocytopenia // Semin. Fetal Neonatal Med. 2016. Vol. 21, N 1. R. 19-27.

5. Sullivan MJ, Kuhlmann R., Peterson JA, Curtis BR Severe neonatal alloimmune thrombocytopenia caused by maternal sensitization against a new low-frequency alloantigen located on platelet glycoprotein IIIa // Transfusion. 2021. Vol. 57, N 7. R. 1847-1848.

6. Winkelhorst D., Oepkes D., Lopriore E. Fetal and neonatal alloimmune thrombocytopenia: evidence based antenatal and postnatal management strategies // Expert Rev. Hematol. 2021. Vol. 10, N 8. P. 729-737.

7. Blanchette VS Neonatal alloimmune thrombocytopenia: a clinical perspective // ​​Curr. Stud. Hematol. Blood Transfus. 2000. Vol. 54. R. 112-126.

8. Sola MC, Slayton WB, Rimsza LM A neonate with severe thrombocytopenia and radioulnar synostosis // J. Perinatol. 2004. Vol. 24. R. 528-530.

9. Grossfeld PD, Mattina T, Lia Z et al. The 11q terminal deletion disorder: a prospective study of 110 cases // Am. J. Med. Genet. A. 2004. Vol. 129. R. 51-61.

10. Drachman JG Inherited thrombocytopenia: when a low platelet count does not mean ITP // Blood. 2004. Vol. 103. R. 390-398.

11. Wiedmeier SE, Henry E., Christensen RD Hematological abnormalities during the first week of life among neonates with trisomy 18 and trisomy 13: data from a multi-hospital healthcare system // Am. J. Med. Genet. A. 2008. Vol. 146A, N 3. R. 312-320.

Diagnosis and treatment

Increased bleeding

– a reason to immediately seek advice from a specialist (hematologist), who will take a detailed medical history and prescribe the necessary examinations. The main diagnostic method is a clinical blood test, which determines the quantitative composition of platelets. The patient may also be recommended a bone marrow puncture to assess the quality of this type of hematopoiesis, ultrasound of the spleen, chest x-ray, and tests for the presence of antibodies. During pregnancy, thrombocytopenia is diagnosed using a coagulogram (blood clotting test), which allows one to determine the quantitative composition of platelets in a woman’s blood.

A serious disease such as thrombocytopenia should be treated only under the supervision of a doctor. After receiving the results of all examinations, the specialist selects individual therapeutic measures for each patient. Severe hemorrhagic syndrome requires urgent hospitalization and emergency care. For severe bleeding, platelet transfusions are indicated, and angioprotectors and fibrinolysis inhibitors are prescribed. Avoid taking acetylsalicylic acid and anticoagulants. If there is a deficiency of vitamin B12 or folic acid, medications with a high content of these elements are prescribed. This significantly improves the general condition of the patient and the results of his tests. If immune thrombocytopenia is diagnosed, a course of prednisolone will be required. The dosage is determined by the individual characteristics of the patient and his body. If drug therapy is ineffective and repeated bleeding occurs, splenectomy (removal of the spleen) is indicated. For non-immune thrombocytopenia, symptomatic hemostatic therapy is recommended.

There is no special diet for thrombocytopenia, but you should adhere to the principles of a rational, balanced diet. You should introduce more vegetables, fruits, herbs, low-fat dairy products, vegetable oils, legumes, and seafood into your diet. Alcohol consumption is strictly prohibited. Undesirable foods: white rice, sugar, baked goods, marinades and pickles.

After consulting with your doctor, you can use traditional medicine - infusions and decoctions of medicinal herbs, sesame oil.

Diagnostics

First of all, if thrombocytopenia is suspected, it is necessary to do a general blood test to determine the number of cellular elements and verify (confirm) the diagnosis of thrombocytopenia. Many diseases that occur with thrombocytopenia have fairly clear symptoms, so differential diagnosis in such cases is not difficult. This applies, first of all, to severe oncological pathologies (leukemia, metastases of malignant tumors in the bone marrow, myeloma, etc.), systemic connective tissue diseases (systemic lupus erythematosus), liver cirrhosis, etc. However, additional studies are often necessary (bone marrow puncture, immunological tests, etc.).

Causes of the disease

Causes of thrombocytopenia include:

1. Hereditary diseases. Bernard-Sourya syndrome, TAR syndrome, May-Hegglin anomalies and other diseases that can provoke pathological bleeding.
2. Diseases that interfere with the creation of new platelets. Most often, this is due to bone marrow problems, cancer, reactions to chemical and radioactive elements, and leukemia. And sometimes even against the background of alcohol and drug abuse.
3. Diagnoses that provoke excessive consumption of platelets by the body. A striking example of such a disease is DIC syndrome. There is an increase in platelet consumption, and as a result, the bone marrow increases its work on the formation of new cells. Over time, he gets tired, his reserves are depleted and thus new platelets do not appear in the blood.
4. Increased size of the spleen. The fact is that a large number of platelets are stored in the spleen, as if in a depot. But when its size is pathologically large, it takes on too many cells, which leads to a decrease in them in the blood, and the bone marrow, in turn, does not compensate for the deficiency.
5. Autoimmune thrombocytopenia. It develops when the body begins to destroy platelets in the blood.
6. Drug-induced thrombocytopenia. Some groups of drugs can destroy platelets or suppress their formation in the bone marrow. For example, long-term use of cytostatics.

Many drugs can cause thrombocytopenia, but it is still quite rare. According to research, about 10 cases per 1 million population per year. (https://pubmed.ncbi.nlm.nih.gov/15588119/)

Forms and types of thrombocytopenia

In medicine, there are several types of thrombocytopenia:

1. Autoimmune. A “failure” occurs in the body and the immune system begins to perceive its own platelets as foreign bodies. The result is the destruction of these blood cells. How to treat autoimmune thrombocytopenia? Doctors provide symptomatic therapy and administer special medications that support the immune system. The complex of treatment measures consists of a course of glucocorticosteroids, after which, in the absence of positive dynamics, surgical removal of the spleen is performed, followed by the prescription of immunosuppressants.

.

2. Essential thrombocytopenia. It is most often diagnosed in people aged 50-70 years. Its development is often associated with previous surgical interventions, chronic pathologies of internal organs, and iron deficiency. Treatment of essential thrombocytopenia is reduced to taking Aspirin. Since no serious problems in the functioning of the body are identified, the prescription of aggressive toxic drugs is considered unjustified.
3. Thrombocytopenic purpura syndrome - this type of disease was first described by doctors. It is diagnosed mainly in childhood. It occurs much more often in girls than in boys. The syndrome is associated with a blood clotting disorder, so the patient will need to not only undergo a course of treatment, but also be constantly monitored by a hematologist.
4. Thrombocytopenia in newborns. It can develop both as an accompanying condition with congenital pathologies, and as a secondary disease with infection of the baby, premature birth or asphyxia during childbirth. Treatment begins in the maternity hospital, using prednisolone, immunoglobulin, and ascorbic acid. Thrombocytopenia in newborns often requires platelet transfusion. For the entire period of treatment, the baby is removed from breastfeeding.