Immune thrombocytopenic purpura (ITP) is an autoimmune disease in which there is an excess of platelet destruction, leading to a decrease in their number in the blood (thrombocytopenia). This condition is defined as being in the field of clinical hemostasis and is manifested by various symptoms, such as easy bruising, spontaneous bleeding, and a possible cause of thrombus formation. At the cellular level, the main cause of the disease is the increased activity of antibodies that destroy platelets, as well as the disruption of the cortical and bone marrow mechanisms responsible for their production. In turn, ITP is divided into primary and secondary forms, depending on the presence of other autoimmune diseases or induction by external factors.
History of the disease and interesting historical facts
The term immune thrombocytopenic purpura was introduced into medical usage in the 1900s, although the condition of low platelet counts has been described in the medical literature for much longer. In the first half of the 20th century, the first studies were conducted to characterize the mechanism of ITP development and the role of antibodies in pathogenesis. Unique cases, such as the disease occurring in children with viral infections, led to further understanding of the clinical aspects. Research in the 1970s confirmed the presence of an autoimmune response to one’s own platelets, and this initiated research related to the treatment of the disease. In the 1980s, it was shown that platelets can be destroyed by the complement mechanism, which was an important step in the diagnosis and treatment of ITP.
Epidemiology
According to epidemiological studies, the overall prevalence of immune thrombocytopenic purpura varies from 4 to 10 per 100,000 people per year in different populations. The disease is most often diagnosed in children aged 2 to 5 years and in adults aged 20 to 50 years. The prevalence of the disease among women is approximately 2:1 compared to men. There is also a natural geographical difference in the incidence, which may be associated with ethnic, cultural, and environmental factors.
Genetic predisposition to this disease
Research suggests that ITP may have a hereditary predisposition, especially in families with other autoimmune diseases. Scientists have identified associations with certain genes, such as HLA and several genes associated with the immune response. Examples include polymorphisms in the IL-10 and TNF-α genes, which may influence the development of an inflammatory response and excessive antibody production. It is important to note that genetic predisposition does not always lead to the development of the disease, but can increase the risk of developing it when interacting with external factors.
Risk factors for the development of this disease
There are various risk factors that may contribute to the development of ITP:
- Infectious diseases, both viruses (eg, Epstein-Barr virus, hepatitis and HIV) and bacteria.
- The presence of autoimmune diseases such as systemic lupus erythematosus and thyroiditis.
- A drug reaction to certain medications, including antibiotics.
- Presence of predisposing genetic mutations.
- Exposure to physical factors such as radiation or heavy metals.
Diagnosis of this disease
Diagnosis of ITP begins with a thorough history and physical examination. Common symptoms include:
- Ease of bruising, even with minor injuries.
- Spontaneous nosebleeds and gum bleeding.
- Blood in urine or stool.
Laboratory tests include:
- Complete blood count to confirm thrombocytopenia.
- Platelet antibody tests.
- Bone marrow examination to rule out other causes of thrombocytopenia.
Radiological examinations are not applicable to ITP. In the differential diagnosis, other causes of thrombocytopenia such as myelodysplastic syndrome, aplastic anemia, and complications of leukemia must be excluded.
Treatment
Treatment of ITP consists of several directions, depending on the severity of the disease and accompanying symptoms. Common treatment methods include:
- Monitoring the patient's condition in mild cases of the disease.
- Pharmacological treatment such as corticosteroids to reduce autoimmune activity.
- Immunosuppressive drugs such as azathioprine or cyclosporine if corticosteroids are ineffective.
- Surgical treatment is splenectomy, removal of the spleen, if other methods have failed.
Also being considered as alternative treatments are plasmapheresis to remove antibodies and the use of romiplast to stimulate platelet production.
List of medications used to treat this disease
Drugs used to treat ITP include:
- Dexamethasone
- Prednisolone
- Azathioprine
- Cyclosporine
- Romiplastim
Disease monitoring
Monitoring of a patient with ITP includes regular check-ups of platelet counts, evaluation of clinical symptoms, and the effectiveness of treatment. The prognosis depends on the cause and form of the disease. In most cases, the disease is spontaneous in children and the condition normalizes, while in adults it can be chronic with a risk of bleeding and complications such as transient cavitary thrombocytopenia.
Age-related features of the disease
Immune thrombocytopenic purpura can present with different features depending on the age group. In children, the disease often resolves on its own, without requiring serious intervention. In contrast, in adults, ITP can be more severe, require long-term treatment, and be associated with other chronic diseases. In older patients, the disease may be accompanied by a higher risk of thrombosis and is associated with concomitant pathologies.
Questions and Answers
- What is immune thrombocytopenic purpura? It is an autoimmune disease in which there is a decrease in the level of platelets in the blood due to their destruction by the immune system.
- What are the main symptoms of ITP? The main symptoms include easy bruising, spontaneous nosebleeds and gum bleeding.
- How is ITP diagnosed? Diagnosis is based on a complete blood count, antibody testing and bone marrow analysis.
- What is the treatment for ITP? Treatment may include corticosteroids, immunosuppressive drugs, and surgery if needed.
- What is the prognosis for patients with ITP? The prognosis depends on the form of the disease, but many children grow up with normal platelet levels, while in adults the disease can become chronic.
Advice from Dr. Oleg Korzhikov
With ITP it is important:
- Get regular check-ups to monitor your platelet levels.
- Avoid traumatic physical activities to minimize the risk of bleeding.
- A balanced diet and health care can help support your overall health and improve your overall well-being.
- Maintain a rest regimen and avoid stress, as they can affect your overall health.
The questions that most often concern patients concern diagnostic methods, prognosis and the need for treatment, while it is important to remember the need for an individual approach to each patient.
