Thrombocytopenic purpura is a disorder that causes moderate to excessive bruising and bleeding. The disease is fairly common, but treatment options are currently available.
What is thrombocytopenic purpura in children?
Thrombocytopenic purpura (TP), or Werlhof's diseaseis a blood clotting disorder characterized by a decrease in the number of platelets in the blood.
Platelets are cells in the blood that help stop bleeding. Their reduction leads to easy bruising, bleeding gums and internal bleeding.
The term “thrombocytopenia” refers to a decrease in the number of platelets in the blood, and “purpura” refers to the presence of purple spots and petechiae on the skin (hemorrhages) caused by internal bleeding from small blood vessels.
The disease is caused by an immune response to one's own platelets.
Causes
Etiological factors
The exact cause of TP is unknown, therefore it is sometimes called idiopathic... The disease is not contagious, that is, the child cannot “catch” it while playing with other children with TP.
Often a child may have viral infection three weeks before the development of TP. It is believed that the body, by creating antibodies against the virus, "accidentally" also produces an antibody that adheres to platelets.
The immune system identifies any cells with antibodies as foreign bodies and destroys them.
Infection Helicobacter pylori is associated with autoimmune diseases, including TP, pathology can also occur in HIV and other hematological disorders, including chronic lymphocytic leukemia, lymphogranulomatosis, non-Hodgkin's lymphoma.
Genetic variations in several genes are found in some patients with LT and may increase the risk of developing abnormal immune responses. However, the contribution of these genetic changes to the development of immune thrombocytopenia is unclear.
There were studies related to the study some drugs that cause TP... Certain medications can alter platelet function. Currently, no direct link has been found to any specific drug that can cause LTBI.
In newborns, TP can be caused by maternal antibodiestransferred to embryonic platelets. Differences between embryonic and maternal platelet antigens can lead to destruction of fetal platelets.
Predisposing factors for the development of chronic AT in children
They are as follows:
- frequent and persistent bleeding, observed six months before exacerbated TP;
- there is no connection between exacerbation and the impact of any provoking criteria;
- the patient has chronic foci of infection;
- development of TP in girls during puberty.
Classification
| Criterion | Characteristic | ||
| Development mechanism | Heteroimmune, occurs when the antigenic structure of platelets is modified due to the influence of certain factors (viruses, new antigens, semi-antigens), an acute course is characteristic | Autoimmune, occurs when autoantibodies are produced that attack their own unchanged platelets; characterized by a chronic, recurrent course | |
| Period | Exacerbations (crisis) | Remissions | |
| Clinical: there are no hemorrhages, but the number of platelets is reduced | Clinical and hematological - there are no clinical and laboratory signs of platelet deficiency | ||
| The severity of the pathology | Mild: bruising and petechiae, occasional minor nosebleeds, very little disruption to daily life | Moderate - more serious damage to the skin and mucous membranes, more profuse nosebleeds; platelet count 55 - 105 x 10 * 9 / l | Severe - long and heavy bleeding, skin manifestations, platelet count 35 - 55 x 10 * 9 / l, post-hemorrhagic anemia, symptoms that seriously affect the quality of life |
| Flow | Sharp (less than six months) | Chronic (more than six months): with rare relapses or regularly recurring | |
Symptoms
Manifestations of purpura in newborns
In an infant, TP should be suspected if petechiae develops throughout the body soon after birth and blood tests show severe thrombocytopenia. Clinical manifestations can range from localized or widespread cutaneous petechiae or bruising on the skin to profuse bleeding or intracranial hemorrhage.
Manifestations in children
A previously healthy child suddenly has generalized petechiae. Parents often state that the child felt good the day before, and now is covered with bruises and purple dots. Bleeding from the gums and mucous membranes is common, especially with deep thrombocytopenia. There may be a previous viral infection 1-4 weeks before the onset of TP. Splenomegaly, lymphadenopathy (enlarged spleen and lymph nodes, respectively), bone pain, and pallor are rare.
The presence of abnormalities such as hepatosplenomegaly (enlargement of the liver and spleen), bone or joint pain, or severe lymphadenopathy indicates other conditions (such as leukemia).
Manifestations in adults
Chronic TP is common in adults and develops slowly.
Symptoms include:
- petechiae;
- bruising;
- bleeding from the nose and gums;
- black blisters in the mouth;
- fatigue;
- heavy menstrual periods in women.
Rarely, the following manifestations may occur:
- hemorrhages in the retina and brain;
- bleeding from the ears;
- blood in the urine;
- bleeding in the intestines.
Diagnostics
To diagnose AT, you need to rule out other possible causes of bleeding and low platelet counts, such as an underlying medical condition or medications your child may be taking.
The specialist will need to take a history, conduct a physical examination, and perform several laboratory tests.
Complete blood count
This test is used to determine the number of blood cells, including platelets, in a sample. With LT, the white and red blood cell counts are usually normal, but the platelet count is reduced.
Blood smear examination
This method is often used to confirm platelet counts found on a complete blood count. The blood sample is placed on a slide and examined under a microscope.
Bone marrow examination
This test helps determine the cause of the low platelet count, although some hematologists do not recommend this method for children with LT.
In patients with LT, the bone marrow will be normal, because the low platelet count is due to the destruction of these cells in the bloodstream and spleen, and not to abnormalities in the bone marrow.
Other studies
An HIV blood test should be done in at-risk groups, especially sexually active adolescents. A direct antiglobulin test should be done if there is unexplained anemia to rule out Evans syndrome (autoimmune hemolytic anemia and thrombocytopenia).
Treatment
The goal of care for a patient with LT is to increase the platelet count to a safe level. There is no cure for TP, and relapses can occur years after seemingly successful medical or surgical treatment.
Drug therapy
Steroids
Steroid use is the mainstay of treatment for this disorder. This group of drugs helps suppress the immune system. The route of administration and dosage is determined by the number of platelets and the presence of any active bleeding. Intravenous infusions of methylprednisolone or dexamethasone may be given in emergencies. For milder forms, oral prednisolone can be used.
As the platelet count returns to normal, the steroid dosage is gradually reduced. Nearly 60% -90% of patients experience relapses during dosage reduction or discontinuation. Long-term use of steroids is not recommended as it can cause various side effects.
Immunosuppressants
Immunosuppressants such as azathioprine and mycophenolate mofetil are becoming more popular due to their effectiveness in the treatment of LT. For chronic and persistent pathologies, when the immune pathogenesis has already been established, a chemotherapeutic agent, vincristine, can be used. However, the drug causes serious side effects and should therefore only be used with caution when treating TP. This is especially important for children as they are more vulnerable.
Immunoglobulin
It is a medicine containing antibodies from human blood, which means that antibodies have been collected from multiple donors. Exactly how it works in treating LT is not known exactly, but additional antibodies are believed to stop white blood cells from breaking down platelets. The drug works fairly quickly, usually within a few days. Unfortunately, the effect does not last long (only a few weeks). The medication is usually given before surgery, or if there is significant bleeding and platelet counts need to be raised urgently.
Rituximab
This drug was originally used to treat cancer, but it has also been used in TP therapy for almost 20 years. Similar to steroids, it stops the immune system from destroying platelets, but has fewer side effects than steroids. This is an artificially created antibody that affects white blood cells. It is not made from human donated blood. Usually, the effect is achieved after a few weeks, although some people respond to treatment after several months.
Thrombopoietin receptor agonists Romiplostim and Eltrombopag
These two drugs have become available in the past few years. Thrombopoietin receptors are found on the surface of cells that make platelets in the bone marrow; these drugs use these receptors to tell the cell to make more platelets. The drugs can only be used in combination with other treatments. Romiplostim is used as a subcutaneous injection, usually once a week. Eltrombopag comes in pill form that must be taken once a day. The substance is not absorbed by the intestines without calcium, therefore it is necessary to eat foods with a high calcium content within four hours before or after taking the drug.
The therapy should be continued until the disappearance of clinical and laboratory manifestations, this may take several years. Medicines do not cure the underlying problem, they simply encourage the body to produce more platelets to replace those that are being destroyed.
Helicobacter Pylori Treatment
Some children with LT have a stomach infection called Helicobacter pylori. Sometimes her antibiotic and antacid therapy for two weeks can cure or improve TP. The increase in platelets after treatment of an infection is not always permanent, but therapy is relatively safe and therefore may be recommended for children.
Dapsone
It is antibacterial, but it can also be used to treat LT. It is not known exactly how the drug works; it appears to decrease the autoimmune process, which can stop the immune system from attacking platelets. It is taken as a pill once a day and has several side effects.
Tranexamic acid
This medication helps blood clots to last longer as they form. The clots are more stable than usual and more stable. The drug does not cure TP, but may be helpful for bleeding while the platelet count is low. The tablet is taken 3 or 4 times a day. There are contraindications.
Why shouldn't platelet transfusion be done?
The platelets produced by the bone marrow are healthy. Their low number is due to the attack of the immune system on them. If a patient receives platelets from a donor, they will be destroyed by your immune system. Platelet transfusions are useful as an emergency treatment if there is heavy bleeding, as they will help to form a clot, but this method is not effective for long-term prevention of bleeding.
Surgery
Since platelets are mainly destroyed when they are in the spleen, removing it (splenoectomy) can cure the pathology. Splenectomy has been used to treat LT for decades and offers the best chance of healing. For every three people who have surgery, two will not need further treatment.
Splenectomy results in a lifelong increased risk of sepsis from bacterial infection.
In children, the risk of bacterial sepsis after splenectomy is estimated to be 1–2%. Many pediatricians recommend postponing surgery until children are 5 years old.
If a splenectomy is planned, the child should be given the Haemophilus influenzae vaccine 14 days before surgery. Also, children over two years old should be vaccinated against Streptococcus pneumoniae and meningococcal infection.
Diet
Diet plays a role in the treatment of TP. Eating the right foods will help your child feel better and more energized.
Certain nutrients, such as vitamin K and calcium, have natural components that are important for blood clotting. They are rich in dark leafy greens (spinach). A lot of calcium is found in dairy products, but experts do not recommend excessive use of it, as this can worsen the symptoms of autoimmune diseases. Vitamin D supplementation also plays a role in boosting the immune system in LT.
Catering tips are as follows:
- whenever possible, it is recommended to use only natural products;
- replace animal and artificial fats with vegetable fats;
- it is necessary to limit the consumption of red meat;
- potentially antiplatelet fruits such as berries, tomatoes, and grapes should be avoided.
Forecast
Correct treatment guarantees a high likelihood of remission. Rarely, thrombocytopenic purpura in children can become chronic with frequent relapses. In general, the prognosis is good, but it is variable and depends on the intensity of the symptoms. Patients may respond differently to the same treatment regimen.
Recommendations for parents
If a child has TP, parents need to learn how to best prevent injury and bleeding:
- for a small child, make the environment as safe as possible. Padding the bed with soft materials, helmets and protective clothing are necessary when the platelet count is low;
- contact sports, cycling, and rough play should be limited;
- Avoid medicines containing aspirin as they can interfere with the body's ability to control bleeding.
- until the child recovers from illness, the child should not be vaccinated, especially those that contain live attenuated viruses. These include measles, mumps, rubella and oral polio vaccines. Also, do not inject into the muscle.
Conclusion
Although there is no known cause of LT and there is no cure for the disease, the prognosis is usually good.
Usually, the body stops producing antibodies that attack platelets, and the pathology disappears on its own.The goal of treatment is to keep the baby's platelets in a safe range until the body corrects the problem.
Overall, prevention of serious bleeding is the most important predictor of prognosis. Providing a safe environment, timely medical attention and ongoing medical care will help your child live a long, healthy life.
