ITP is an autoimmune disorder characterized by a low platelet count (thrombocytopenia). In ITP, an abnormal immune response occurs in which the body’s own antibodies bind to platelets produced in the bone marrow as they are released into the bloodstream. The spleen identifies these antibody-coated platelets as foreign and destroys them. As a result, the platelet count decreases.
In patients with ITP, the platelet count typically ranges from 0–100 × 10⁹/L (from severe to mild ITP). The platelet level can be determined with a complete blood count (PVK).
Patients with ITP may experience a wide variety of symptoms. These can include petechiae (small red pinpoint spots on the skin), increased bruising tendency, bleeding tendency, and chronic fatigue.
Heredity of ITP
The cause of ITP may vary from hereditary factors to viral infections. In children, ITP may be triggered by a viral infection.
In hereditary thrombocytopenias, bleeding tendency and low platelet count are present from childhood. In addition to reduced platelet production, platelet function may also be impaired. Diagnosis of hereditary thrombocytopenias requires specialized laboratory investigations, such as genetic testing.
Clinical Presentation of ITP
In patients with ITP, the platelet count typically ranges from 0–100 × 10⁹/L (from severe to mild ITP), whereas in healthy individuals the normal range is 150–450 × 10⁹/L. The platelet level can be determined with a complete blood count (PVK).
Patients with ITP may present with a wide range of symptoms, including spontaneous bruising and bleeding, petechiae (small red spots on the skin, especially on the lower limbs), bleeding gums and blood blisters in the mouth, internal bleeding, heavy or irregular menstrual bleeding, nosebleeds, fatigue, and low mood (depression).
Treatment of ITP
There are several treatment options for ITP, including corticosteroids, intravenous immunoglobulin (IVIg), rituximab, TPO-RAs, immunosuppressive agents, and splenectomy.
Corticosteroids are given orally to suppress the immune system and thereby increase platelet counts. Corticosteroids are used for long-term management.
Intravenous immunoglobulin (IVIg) is administered intravenously in acute situations. The solution contains plasma proteins and IgG antibodies.
Rituximab is a monoclonal antibody targeting B-lymphocytes. It is most commonly used when treatment beyond first-line therapy is required.
TPO-RA therapy is hormone-based and aims to stimulate platelet production in the bone marrow.
Immunosuppressive medication can be used in various autoimmune diseases. It works by reducing the activity of the immune system.
Splenectomy (removal of the spleen) is a surgical procedure in which the spleen is removed. In approximately two-thirds of cases, splenectomy leads to long-term elevation of the platelet count, making further medical treatment for ITP unnecessary.
The treatments for ITP and their effectiveness are as individual as the patients themselves. What works for one patient may not be equally effective for another.