Immune Thrombocytopenia Treatment, also known as immune thrombocytopenic purpura or ITP, occurs when the immune system mistakenly attacks and destroys platelets. Platelets are blood cell fragments that help control bleeding. The exact cause of ITP is unknown in most cases, but it is considered an autoimmune disorder where the immune system malfunctions and produces antibodies against the body’s own platelets. Some risk factors and potential causes that can trigger ITP include:
– Viral or bacterial infections: Some viruses like hepatitis C, HIV, or bacterial infections have been linked to ITP in rare cases by triggering the immune system.
– Vaccines: Very rarely, some vaccines may act as triggers for ITP in susceptible individuals.
– Other autoimmune diseases: People with lupus or rheumatoid arthritis have a higher risk of also developing ITP due to a preexisting immune imbalance.
– Medications: Certain drugs like quinine for malaria or antipsychotics have been associated with drug-induced ITP.
– Pregnancy: ITP occurs more commonly during pregnancy or postpartum period, usually resolving once delivery is complete.
Diagnosis and assessing severity
ITP is typically diagnosed based on evaluations of the clinical symptoms and signs coupled with blood tests. If blood platelet counts are found to be lower than normal range of 150,000-450,000 platelets per microliter of blood, along with no other apparent causes, a diagnosis of ITP is made. Severity of ITP is assessed based on platelet counts and risk of bleeding:
– Mild: Platelet counts above 30,000 and no bleeding.
– Moderate: Platelet counts between 30,000-10,000 with few petty bleeds.
– Severe: Platelet counts below 10,000 with serious internal bleeding risks.
First line Immune Thrombocytopenia Treatment options
For newly diagnosed mild cases of ITP, initial treatment may not always be required as spontaneous remission is possible. The patient is monitored closely and advised to avoid contact sports or other activities with bleeding risks. However, for moderate and severe ITP following options are considered:
Corticosteroids:
Drugs like prednisone and dexamethasone are effective first line treatments that work by suppressing the immune system. Complete remission is achieved in 60-80% of children and 30-50% of adults with short term use of steroids. However, relapses are common once steroids are tapered.
Intravenous immunoglobulin (IVIg):
IVIg injections contain antibodies from thousands of healthy blood donors. It works by blocking Fc receptors on macrophages and inhibiting the production of autoantibodies against platelets. IVIg effectively raises platelet counts within 3-5 days in 60-80% of ITP cases.
Anti-D immunoglobulin:
This treatment is reserved only for Rh+ve pregnant women with ITP to raise critically low platelet counts as it carries risk of hemolytic anemia in non-pregnant individuals. Anti-D works by binding to and clearing platelets coated with anti-platelet antibodies from circulation.
Second line Immune Thrombocytopenia Treatment options
For patients who do not respond sufficiently or frequently relapse after first line treatments, second line options include:
Rituximab:
A monoclonal antibody that targets and depletes B-cells responsible for producing anti-platelet antibodies. FDA approved for use in ITP, it induces remission in 60-80% cases even after other treatments fail.
Thrombopoietin receptor agonists (TPO-RAs):
Drugs like eltrombopag and romiplostim act by binding to c-mpl receptor on bone marrow cells and stimulating platelet production irrespective of immune response. They help raise platelet counts and reduce bleeding risks.
Splenectomy:
Surgical removal of the spleen, where antibody coated platelet destruction mainly occurs, is an option for patients who failed at least one line of treatment. It provides long term remission in around 60% post-splenectomy ITP cases.
Immunosuppressants:
Azathioprine, mycophenolate mofetil, cyclosporine A help dampen the immune response, but their role is limited due to more adverse effects compared to targeted therapies.
Evolving novel treatments
As the complex immune mechanisms in ITP are being better understood, new treatment approaches are evolving. These include B-cell targeting therapies beyond Rituximab like Bruton tyrosine kinase inhibitors, FDA approved for rheumatoid arthritis. Chimeric antigen receptor T-cell therapy is showing promise in targeting specific B-cell subsets producing antiplatelet antibodies in early phase trials. And stem cell transplants remain an option for select refractory ITP patients who fail multiple lines of standard care. Overall, with expanding treatment armamentarium today ITP prognosis has significantly improved.
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1. Source: Coherent Market Insights, Public sources, Desk research
2. We have leveraged AI tools to mine information and compile it