Generally, essential thrombocythemia (ET) doesn't shorten life expectancy. However, medical treatment is important to prevent or treat complications.
It's important that your doctor is experienced in treating myeloproliferative neoplasms (MPNs) or works in consultation with a hematologist oncologist who has experience treating MPN patients.
ET is a chronic disease: It's not curable, but it can usually be managed effectively for long periods. Careful medical supervision and therapy to prevent or treat thrombosis, a serious complication that can affect vital organs such as the brain or the heart, are important. Also, for untreated pregnant patients with ET, there's a risk to the fetus' survival.
Patients with low risk for clotting are usually observed without any therapy; low-dose aspirin can be considered. Patients with high risk for clotting require medical therapy to decrease platelets to normal levels, and are given low-dose aspirin to prevent clotting.
A risk factor for bleeding can include a very elevated platelet count (over 2 million platelets per microliter of blood). Therefore, in a young patient with low risk for clotting but with an extremely high platelet count, one should be aware of the increased risk of bleeding. In this case, use of medications to lower an extremely high platelet count should be considered, but aspirin should be avoided as it may contribute to bleeding risk (at least until the number of platelets has been decreased).
Types of ET Treatment
Doctors use the following approaches to treat ET:
- Chemotherapy and drug therapy
Your doctor may suggest that you participate in a clinical trial. Clinical trials can involve therapy with new drugs and new drug combinations.
ET therapies are aimed at:
- Lowering the hematocrit and hemoglobin concentration to normal or near-normal levels
- Lowering the platelet count if it's high or it becomes high over time
Finding the Best Treatment Approach
The treatment your doctor recommends is based on your risk of clotting or bleeding complications. The complication risk may be low for some patients with no signs of the disease other than an increased platelet count. Patients with low risk, especially young people with no other cardiovascular risk factors, may need only periodic medical checkups. On the other hand, in patients with previous bleeding or clotting episodes, or in patients who are at high risk for such complications, doctors may use medications to reduce high platelets.
Risks for clotting complications (thrombosis) include:
- A history of a clot
- Advanced age (over 60 years)
- Cardiovascular risk factors, such as high cholesterol, diabetes, smoking, obesity or hypertension, are considered by many doctors as additional risk factors for thrombosis.
The goals of ET treatment are to:
- Reduce high platelet counts
- Reduce the risk of complications
As you develop a treatment plan with your doctor, be sure to discuss:
- The results you can expect from treatment
- Potential side effects
- The possibility of participating in a clinical trial, where you'll have access to advanced medical treatment that may be more beneficial to you than standard treatment
You may find it helpful to bring a loved one with you to your doctor's visits for support and to take notes and ask follow-up questions. It's a good idea to prepare questions you'd like to ask when you visit your doctor. You can also record your conversations with your doctor and listen more closely when you get home.
Click here to download lists of suggested questions to ask your healthcare providers.