Immune thrombocytopenia is an autoimmune disease in which the immune system attacks and destroys platelets, leading to low platelet counts in the blood, which can cause spontaneous bleeding, bruising, and other bleeding complications.
What is Immune Thrombocytopenia (Idiopathic Thrombocytopenic Purpura)?
Immune thrombocytopenia (ITP) is an autoimmune disease characterized by the accelerated destruction of platelets by the immune system, leading to low platelet counts in the blood. This condition can cause spontaneous bleeding, bruising, and other bleeding complications, affecting both adults and children.
Causes and Risk Factors in Immune Thrombocytopenia
Although the exact cause of ITP is not fully understood, it is believed that this condition occurs when the immune system mistakenly identifies platelets as foreign and destroys them. There are several factors that can contribute to the development of the disease, including genetic predisposition, viral infections, and certain autoimmune diseases.
Autoimmune mechanism of platelets
In ITP, the body produces antibodies against platelets, causing the spleen and other organs to prematurely eliminate them. This process reduces the number of platelets available in the blood, which affects the normal clotting ability and increases the risk of bleeding. Also, the production of platelets in the bone marrow can be affected, worsening thrombocytopenia.
Associated autoimmune diseases
Immune thrombocytopenia can be associated with other autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, antiphospholipid syndrome and autoimmune thyroiditis. In some cases, ITP can occur as a secondary manifestation of these conditions, requiring a complex approach in diagnosis and treatment.
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Infections and Possible Triggers
Some viral infections, such as hepatitis C, HIV, Epstein-Barr virus, and Helicobacter pylori, have been associated with the development of ITP. These pathogens can stimulate the immune system to attack platelets, triggering or worsening thrombocytopenia. Certain vaccines or medications can also act as triggers in some cases.
Genetic factors and family predisposition
Although ITP is not considered a strictly hereditary disease, there are studies that suggest that genetic predisposition may play a role in the development of the disease. People with a family history of autoimmune diseases may be at a higher risk of developing ITP.
Signs and Symptoms of Thrombocytopenia
The symptoms of ITP range from mild to severe, depending on the level of platelets in the blood. Some people may have obvious manifestations, while others may be asymptomatic.
H3: Skin and mucosal bleeding
Petechiae (small red dots on the skin)
Echimosis (spontaneous bruising)
Gingival or nasal bleeding
Heavy menstrual bleeding
Gastrointestinal or cerebral bleeding in severe cases
Thrombocytopenia can also be asymptomatic
In some people, ITP is discovered incidentally during a routine blood test, without obvious symptoms. Even in these cases, monitoring is essential to prevent complications.
Types of Immune Thrombocytopenia
Immune thrombocytopenia can be classified according to the duration and evolution of the disease. There are acute forms, which appear suddenly and can spontaneously resolve, and chronic forms, which require long-term monitoring and treatment. ITP can also affect both adults and children, each category having specific characteristics.
Acute thrombocytopenia
The acute form of ITP occurs suddenly and is more common in children, often after a viral infection. In most cases, it resolves spontaneously within a few months without specific treatment.
Chronic thrombocytopenia
Chronic ITP persists for more than 12 months and is more common in adults. It requires monitoring and, in some cases, treatment to prevent bleeding complications.
ITP in children
Children often develop acute ITP after viral infections, and most cases resolve without treatment. However, a small percentage of them may develop a chronic form, requiring a different approach than adults.
Establishing the Diagnosis of Immune Thrombocytopenia
The diagnosis of ITP is based on excluding other possible causes of thrombocytopenia and evaluating the patient's medical history. The doctor will perform a series of tests to confirm the disease and identify possible triggers.
Blood tests
Complete blood count: shows an isolated decrease in the number of platelets, with a normal number of white and red blood cells.
Peripheral blood smear: helps to exclude other hematological disorders.
Bone marrow function tests (if necessary): In cases where other conditions are suspected, a bone marrow aspiration may be performed.
Immunological tests for anti-platelet antibodies
Tests to detect antibodies that attack platelets can support the diagnosis of ITP, although they are not always conclusive.
Viral Screening (HIV, Hepatitis)
Viral infections such as HIV, hepatitis B and C, or Epstein-Barr virus can cause secondary thrombocytopenia, which is why screening is necessary in establishing the differential diagnosis.
How to Treat Immune Thrombocytopenia
Treatment of ITP depends on the severity of the condition and the risk of bleeding. In some cases, close monitoring is sufficient, while in others, immunosuppressive therapies or surgery are necessary.
Monitoring and Minimal Treatment
Patients with mild ITP, without significant bleeding, can be monitored without active treatment. Recommendations include avoiding medications that affect coagulation, such as aspirin and nonsteroidal anti-inflammatory drugs.
Corticosteroid and IV immunoglobulin therapy
Corticosteroids (prednisone, dexamethasone): reduce the activity of the immune system and decrease platelet destruction. They are the first line of treatment.
Intravenous immunoglobulins (IVIG): rapidly increase platelet levels and are used in cases of severe bleeding or when a rapid increase in platelets is needed (e.g. before surgery).
Splenectomy
In cases where drug treatments do not work, removal of the spleen (splenectomy) may be necessary, as this organ is responsible for the destruction of antibody-marked platelets.
Immunomodulatory agents and thrombopoietin agonists
Immunomodulatory agents (rituximab, azathioprine, cyclosporine): reduce the autoimmune response against platelets.
Thrombopoietin receptor agonists (eltrombopag, romiplostim): stimulate platelet production in the bone marrow, being used in chronic forms of ITP.
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Complications and Evolution
ITP can have a variable evolution, from mild and self-limited forms to severe cases, which require aggressive treatment. Sometimes, the disease can become chronic, requiring long-term monitoring.
Severe bleeding and intracranial hemorrhage
The most serious complication of ITP is massive hemorrhage, especially in the brain. Signs of an intracranial hemorrhage include severe headaches, confusion, difficulty speaking, weakness on one side of the body and loss of consciousness. Patients at high risk of bleeding should be monitored closely and treated promptly to prevent such complications.
Pregnancy and immune thrombocytopenia
Pregnant women with ITP require specialized supervision, as low platelet levels can increase the risk of bleeding during childbirth. In most cases, the disease does not affect the fetus, but there is a risk that the newborn may have a mild decrease in platelets, which usually resolves spontaneously. Treatment during pregnancy is carefully selected to avoid side effects on the baby.
Practical Tips and Prevention
Although ITP cannot be completely prevented, certain measures can reduce the risk of complications and improve the quality of life of patients.
Regular monitoring of platelet counts
Patients diagnosed with ITP should have regular blood tests to check platelet levels and adjust treatment if necessary. Close monitoring helps prevent severe bleeding episodes.
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Avoiding triggers
People with ITP should avoid medications that increase the risk of bleeding, such as aspirin, nonsteroidal anti-inflammatory drugs, and some anticoagulants. Exposure to infections and vaccines that can trigger autoimmune responses should also be avoided.
Medical emergencies
It is essential for patients with ITP to recognize the symptoms of a medical emergency, such as persistent bleeding, digestive bleeding, or signs of cerebral hemorrhage. In such situations, they should seek emergency medical care.
Conclusion
Immune thrombocytopenia is an autoimmune condition that can range from mild forms, which only require monitoring, to severe cases, which require aggressive treatment. With proper care, most patients can lead a normal life. Early diagnosis, regular monitoring, and adherence to medical recommendations are essential for effective disease management and prevention of complications.
References
Cooper, N., & Ghanima, W. (2019). Immune thrombocytopenia. New England Journal of Medicine, 381(10), 945-955.
Cuker, A., & Cines, D. B. (2010). Immune thrombocytopenia. Hematology 2010, the American Society of Hematology Education Program Book, 2010(1), 377-384.
Audia, S., Mahévas, M., Samson, M., Godeau, B., & Bonnotte, B. (2017). Pathogenesis of immune thrombocytopenia. Autoimmunity reviews, 16(6), 620-632.
Online Articles
https://www.mayoclinic.org/diseases-conditions/idiopathic-thrombocytopenic-purpura/symptoms-causes/syc-20352325 Accessed 03/06/2025
https://my.clevelandclinic.org/health/diseases/5726-immune-thrombocytopenia Accessed 03/06/2025
https://emedicine.medscape.com/article/202158-overview?form=fpf Accessed 03/06/2025
https://www.ncbi.nlm.nih.gov/books/NBK562282/ Accessed 03/06/2025
