Hairy cell leukemia may cause symptoms like extreme tiredness, infections and pain below your ribs. Healthcare providers treat this rare blood cancer with chemotherapy, immunotherapy and targeted therapy. Treatment often puts it into remission, where you don’t have symptoms and tests show no signs of cancer. But the condition often comes back.
Hairy cell leukemia is a rare type of blood cancer that happens when your bone marrow makes abnormal B-cells (B lymphocytes). These are a type of white blood cells that fight infections. The name of the condition comes from the tiny hair-like protrusions on the abnormal B-cells, which pathologists can see when they look at the cells under a microscope.
You have B lymphocytes in your bone marrow, lymph nodes and spleen. Hairy cell leukemia symptoms happen when your normal B lymphocytes change into abnormal ones. When that happens:
Hairy cell leukemia is a rare condition that affects 1 in 100,000 people in the U.S.
The condition affects men and people assigned male at birth (AMAB) more than women and people assigned female at birth (AFAB). It’s more common in people who are white than people in other ethnic groups.
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Hairy cell leukemia may not cause symptoms. But when it does, symptoms may include:
Experts don’t know the exact cause. Research shows that 90% of people with hairy cell leukemia have a genetic mutation (change) that affects the BRAF gene. This gene helps cell growth. When it changes, it creates abnormal cells that grow and multiply uncontrollably. This change happens after you’re born. Researchers aren’t sure what triggers the change, but there are risk factors that increase your chance of developing hairy cell leukemia.
Hairy cell leukemia risk factors include:
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This condition increases your risk of infections because you have very low levels of infection-fighting white blood cells. These include bacterial infections, viral infections or fungal infections that may be life-threatening.
There’s also a small chance that you’ll develop another type of cancer (secondary cancer) years after you receive your diagnosis and treatment.
For example, researchers following up on people with hairy cell leukemia found some people had cancerous tumors that appeared 10 to 20 years after successful treatment for the condition.
Healthcare providers will ask when your symptoms started and take a medical history, including whether you have infections that keep coming back. They’ll also do a physical examination. They may feel (palpitate) your lymph nodes, spleen and liver for signs of swelling.
They may refer you to a hematologist or medical oncologist for more tests that will lead to a diagnosis. These tests may include:
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You might need immediate treatment for an infection and then other treatments to manage symptoms of hairy cell leukemia. But the condition doesn’t always cause symptoms. In that case, your hematologist or oncologist may recommend active surveillance. If you have symptoms, they may recommend medication.
Hairy cell leukemia grows very slowly. You may have it for years before developing symptoms. Cancer treatment comes with side effects and potential complications. If you don’t have symptoms, your oncologist may recommend delaying treatment until you develop symptoms.
In active surveillance, you’ll see your oncologist every three to six months. During those checkups, they’ll:
It can be stressful to wait for symptoms to develop before starting treatment. Your oncologist will understand your feelings and will be glad to explain why they recommend active surveillance.
Chemotherapy is the most common initial (first-line) treatment for people who have symptoms with hairy cell leukemia. Cladribine (Leustatin®) or pentostatin (Nipent®) are common initial anticancer drug treatments for this condition.
You may have other treatments for hairy cell leukemia if chemotherapy isn’t effective or the condition comes back. Treatment options include:
All cancer treatments have side effects. In this case, the chemotherapy drugs that treat hairy cell leukemia increase your risk of infections that may be life-threatening. Your oncologist will explain what steps you can take to prevent infections. They’ll also tell you what changes in your body may be signs you have an infection.
There’s no cure for hairy cell leukemia. Research shows treatment, including chemotherapy, can put this condition into remission for 10 years or more. Remission means you don’t have symptoms anymore and tests don’t find any signs of the condition. One analysis of people’s experience with hairy cell leukemia suggests that, after treatment, they’ll live as long as people who don’t have the condition.
That said, hairy cell leukemia can come back after several years of initial treatment. But there are treatments to put the disease back into remission.
Most people need medical care and support for the rest of their lives so their providers can watch for recurrent hairy cell leukemia. If you have this disease but don’t have symptoms, you’ll have checkups and tests every three to six months after your diagnosis.
According to the Leukemia and Lymphoma Society, 90% of people treated with the chemotherapy drug cladribine were alive five years after treatment. But your prognosis (what may happen after treatment), depends on many factors. Your age, your overall health, your symptoms and your body’s response to treatment all play a part in your prognosis. If you have questions about your survival rate, ask your oncologist to explain how survival rate data applies to you.
Hairy cell leukemia increases the chance you’ll develop infections that can be life-threatening. You can strengthen your immune system and protect yourself from infection. Here are some suggestions:
Hairy cell leukemia and treatments increase the chance you’ll have serious and sometimes life-threatening infections. Contact your healthcare provider if you have the following symptoms:
Maybe you’ve felt more tired than usual, for seemingly no reason. Or maybe you’ve wondered about a respiratory infection that you just can’t seem to beat. Now tests show you have hairy cell leukemia, a rare blood cancer that treatment can’t cure. But there are treatments that put the condition into remission for years at a time. Long-term remission means your healthcare team will check on your overall health for the rest of your life. But researchers are investigating ways to treat hairy cell leukemia that comes back so that the condition goes back into remission. And there are things you can do to help yourself. For example, cancer treatment may increase your risk for infections. Ask your healthcare provider about vaccinations and other steps you can take to reduce infection risk.
Last reviewed on 07/26/2024.
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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy