Beyond Platelets
Immune Thrombocytopenia (ITP), which was formerly known as Idiopathic Thrombocytopenic Purpura, is a rare blood disease that impacts roughly 9.5 people out of every 100,000 in the United States. There is both an acute and chronic form of this condition, with acute ITP being more common in children under 10 and the chronic condition more common in adults over 60. Over the years there has been an evolution in ITP care guidelines. This article will outline what ITP is, the signs and symptoms to watch for and current ITP adult care guidelines.
What is Idiopathic Thrombocytopenic Purpura?
Immune Thrombocytopenia (ITP) is a blood disease characterized by low levels of the platelets that help blood clot. Individuals with ITP often have issues with bleeding and bruising, due to low levels of these platelets. Generally, this condition occurs when the immune system makes a mistake and begins to attack and destroy the platelets that help blood clot. For ITP adults, the condition may be triggered by infections or bacteria, while children may develop it after a bout of the flu or the mumps.
While not everyone who has ITP will have symptoms, some may experience the following:
- Petechiae: little reddish-purple spots that indicate bleeding under the skin. They are most often located on the lower legs and may resemble a rash.
- Skin that is easily bruised.
- Larger spots of bleeding under the skin, called purpura (they are larger than petechiae).
- Excessively heavy menstrual bleeding.
- Nosebleeds or bleeding gums.
- Bloody urine or stool.
ITP tends to be more common in those who have concurrent immune system diseases such as rheumatoid arthritis or lupus.
What are the Care Guidelines for ITP?
The first step in managing ITP is a proper diagnosis. To diagnose ITP, other conditions must first be ruled out. Doctors will typically look at your platelet count and other metrics to come to a diagnosis since there is not one specific test that can be used to diagnose. The American Society of Hematology has updated their ITP care guidelines recently to provide the best possible care for newly diagnosed individuals.
Generally, individuals with a mild form of ITP will only need to be monitored and have their platelet count checked to ensure it is within a safe level. If there is no bleeding and the platelet count isn’t too low then they may not need treatment. However, since the condition is chronic it is likely that adults will need some form of treatment at some point in time.
These treatments can range from medications that increase platelet count to a splenectomy (removing the spleen). Working with your doctor will help you decide on the best treatment options based on your severity and symptoms.
The new care guidelines from The American Society of Hematology found that aggressive immunosuppressive treatment of newly diagnosed adults increases the likelihood of “curing” the disease. They suggest taking this approach before the disease is well-established and before trying other treatments.
Currently, they are testing one to four cycles of high-dose dexamethasone for four days, which demonstrates a durable response rate of 60 to 80%! This new approach to treatment could mitigate the need for other medications and even surgery.
Some Treatment Options for ITP
Depending on whether you are newly diagnosed or where you live, this treatment may not be available. If this is the case, some common medications that may be recommended to treat ITP include:
- Steroid medications, including oral steroids, may be recommended in the short term to increase platelet count. They are not recommended for long-term use since they can have side effects.
- Immune globulins are typically used for acute bleeding or to boost platelet count before surgery.
- Platelet-increasing drugs such as avatrombopag (Doptelet), romiplostim (Nplate) and eltrombopag (Promacta) can help stimulate the bone marrow to produce more platelets.
- Drugs that suppress immune system responses, such as Rituximab (Rituxan, Ruxience, Truxima), may be recommended in some cases, though there are risks that they can impact vaccine efficacy.
If these medications do not work, your doctor may recommend a splenectomy. Removing the spleen tends to help eliminate the immune attacks on platelets. People without a spleen may be at a higher risk of illness or of developing serious infections and the surgery does not always work.
The American Society of Hematology does recommend TPO-R agonist drugs for people with refractory ITP or who had failed spleen removal. They have seen positive results from using these new drugs, which include romiplostim (AMG 531) and eltrombopag. Your doctor will help you decide the best approach to treating ITP depending on the severity, platelet count and response to other drugs.
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