This slow-growing cancer doesn't always need immediate treatment. Learn how doctors decide to start chemotherapy and biological therapy for hairy cell leukemia.
Hairy cell leukemia is a rare, slow-growing cancer of the blood in which your bone marrow makes too many B cells (lymphocytes), a type of white blood cell that fights infection.
These excess B cells are abnormal and look "hairy" under a microscope. As the number of leukemia cells increases, fewer healthy white blood cells, red blood cells and platelets are produced.
Hairy cell leukemia affects more men than women, and it occurs most commonly in middle-aged or older adults.
Hairy cell leukemia is considered a chronic disease because it may never completely disappear, although treatment can lead to a remission for years.
Some people have no signs or symptoms of hairy cell leukemia, but a blood test for another disease or condition may inadvertently reveal hairy cell leukemia.
Other times people with hairy cell leukemia experience signs and symptoms common to a number of diseases and conditions, such as:
Make an appointment with your doctor if you have any persistent signs and symptoms that worry you.
It's not clear what causes hairy cell leukemia.
Doctors know that cancer occurs when cells develop errors (mutations) in their DNA. In the case of hairy cell leukemia, mutations in the DNA cause your bone marrow stem cells to create too many white blood cells that don't work properly. Doctors don't know what causes the DNA mutations that lead to hairy cell leukemia.
Certain factors may increase your risk of developing hairy cell leukemia. Not all research studies agree on what factors increase your risk of the disease.
Some research indicates that your risk of hairy cell leukemia increases based on your:
Hairy cell leukemia progresses very slowly and sometimes remains stable for many years. For this reason, few complications of the disease occur.
Untreated hairy cell leukemia that progresses may crowd out healthy blood cells in the bone marrow, leading to serious complications, such as:
Some studies have found that people with hairy cell leukemia may have an increased risk of developing a second type of cancer. It isn't clear whether this risk is due to hairy cell leukemia's effect on the body or if the risk comes from the medications used to treat hairy cell leukemia.
Second cancers found in people treated for hairy cell leukemia include non-Hodgkin's lymphoma, among others.
To diagnose hairy cell leukemia, your doctor may recommend:
Physical exam. By feeling your spleen — an oval-shaped organ on the left side of your upper abdomen — your doctor can determine if it's enlarged. An enlarged spleen may cause a sensation of fullness in your abdomen that makes it uncomfortable to eat.
Your doctor may also check for enlarged lymph nodes that may contain leukemia cells.
Blood tests. Your doctor uses blood tests, such as the complete blood count, to monitor the levels of blood cells in your blood.
People with hairy cell leukemia have low levels of all three types of blood cells — red blood cells, white blood cells and platelets. Another blood test called a peripheral blood smear looks for hairy cell leukemia cells in a sample of your blood.
Careful analysis of hairy cell leukemia cells in your blood and bone marrow samples may reveal certain genetic and chemical changes that give your doctor an idea of your prognosis and play a role in determining your treatment options.
It's not always necessary to start treatment for hairy cell leukemia immediately after the diagnosis is confirmed. Because this cancer progresses very slowly and sometimes doesn't progress at all, treatment can be delayed.
You'll have regular follow-up appointments with your doctor to monitor for progression of the hairy cell leukemia. If you experience signs and symptoms of the cancer, you may decide to undergo treatment. The majority of people with hairy cell leukemia eventually need treatment.
There is no cure for hairy cell leukemia. But treatments are effective at putting hairy cell leukemia in remission for years.
Doctors consider chemotherapy drugs the first line of treatment for hairy cell leukemia. A great majority of people will experience complete or partial remission through the use of chemotherapy.
Two chemotherapy drugs are used in hairy cell leukemia:
Cladribine. Treatment for hairy cell leukemia typically begins with cladribine. You may receive either a continuous infusion of the drug or daily injections into a vein over several days.
Most people who receive cladribine experience a complete remission that can last for several years. If your hairy cell leukemia returns, you can be treated with cladribine again. Side effects of cladribine may include infection and fever.
Biological therapy attempts to make cancer cells more recognizable to your immune system. Once your immune system identifies cancer cells as intruders, it can set about destroying your cancer.
Two types of biological treatments are used in hairy cell leukemia:
Rituximab. Rituximab (Rituxan) is a monoclonal antibody approved to treat non-Hodgkin's lymphoma and chronic lymphocytic leukemia, though it's sometimes used in hairy cell leukemia.
If chemotherapy drugs haven't worked for you or you can't take chemotherapy, your doctor might consider rituximab. You doctor may also combine cladribine and rituximab. Side effects of rituximab include fever and infection.
Interferon. Currently, the role of interferon in hairy cell leukemia treatment is limited. You might receive interferon if chemotherapy hasn't been effective or if you can't take chemotherapy.
Most people experience partial remission with interferon, which is taken for a year. Side effects include flu-like symptoms, such as fever and fatigue.
Other drugs that target the immune system may be recommended if your cancer returns or if it doesn't respond to standard treatments. Clinical trials are studying new biological therapies and targeted therapies for treating hairy cell leukemia.
Surgery to remove your spleen (splenectomy) might be an option if your spleen ruptures or if it's enlarged and causing pain. Though removing your spleen can't cure hairy cell leukemia, it can usually restore normal blood counts.
Splenectomy isn't commonly used to treat hairy cell leukemia, but it may be helpful in certain situations. Any surgery carries a risk of bleeding and infection.
No alternative medicines have been found to treat hairy cell leukemia. But alternative medicine may help you cope with the stress of a cancer diagnosis and the side effects of cancer treatment.
Talk to your doctor about your options, such as:
Doctors consider hairy cell leukemia a chronic form of cancer because it never completely goes away. Even if you achieve remission, you'll likely require follow-up visits with your doctor to monitor your cancer and your blood counts.
Knowing that your cancer could come back at any time can be stressful. To help you cope, you might consider trying to:
Find out enough to feel comfortable making decisions about your care. Learn about your hairy cell leukemia and its treatment so that you can feel more confident about making decisions about your treatment.
Having a better idea of what to expect from treatment and life after treatment can make you feel more in control of your cancer. Ask your doctor, nurse or other health care professional to recommend some reliable sources of information to get you started.
Connect with other cancer survivors. While friends and family provide an important support network during your cancer experience, they can't always understand what it's like to face cancer. Other cancer survivors provide a unique network of support.
Ask your doctor or another member of your health care team about support groups or organizations in your community that can connect you with other cancer survivors. Organizations such as the American Cancer Society and the Leukemia & Lymphoma Society offer online chat rooms and discussion boards.
Take care of yourself. You can't control whether your hairy cell leukemia comes back, but you can control other aspects of your health.
Take care of yourself by eating a balanced diet with plenty of fruits and vegetables and by exercising regularly. A healthy body can more easily fend off infections, and should you ever need to be treated for cancer again, you'll be better able to cope with the side effects of treatment.
You're likely to start by first seeing your family doctor. If your doctor suspects you may have hairy cell leukemia, you may be referred to a doctor who treats diseases of the blood and bone marrow (hematologist).
Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be prepared. Here's some information to help you get ready and know what to expect from your doctor.
Your time with your doctor is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For hairy cell leukemia, some basic questions to ask your doctor include the following:
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions during your appointment.
Your doctor is likely to ask you a number of questions. Being ready to answer them may allow more time later to cover other points you want to address. Your doctor may ask:
September 1st, 2021