Leukemia - B-cell Prolymphocytic Leukemia and Hairy Cell Leukemia

ON THIS PAGE : You will find out more about the factors that increase the chance of developing PLL or HCL. Use the menu to see other pages.

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. Knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

In general, leukemia can sometimes be caused by a genetic mutation or change. These may be changes that are passed from generation to generation within a family. Or, environmental factors may cause these changes, such as smoking or exposure to certain chemicals or radiation. However, most often the cause of leukemia is not known.

The following factors may raise a person’s risk of developing PLL or HCL. Learn more about the risk factors for CLL .

• Age. HCL occurs most often in people between ages 40 and 70.

• Gender. Men are 5 times more likely to develop HCL than women.

• Race/Ethnicity. HCL is more common in white people in general and Ashkenazi Jewish men specifically. PLL and HCL are rare in people of Japanese or Southeast Asian descent.

The next section in this guide is Symptoms and Signs . It explains what body changes or medical problems this disease can cause. You may use the menu to choose a different section to read in the guide.

ON THIS PAGE : You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. Use the menu to see other pages.

Doctors use many blood and bone marrow tests to diagnose HCL and PLL. They also use these tests to find out how much the disease has spread. Doctors may also do tests to learn which treatments could work best.

Your doctor may suspect that you have HCL or PLL based on your symptoms. Blood and bone marrow tests are the only sure way for the doctor to know if you have HCL or PLL.

This list describes options for diagnosing HCL and PLL. Not all tests listed below will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

• The type of leukemia suspected

• Your signs and symptoms

• Your age and medical condition

• The results of earlier medical tests

The following tests may be used to diagnose PLL and HCL:

• Blood tests. The diagnosis of PLL or HCL begins with a blood test, called a complete blood count (CBC) . A CBC measures the numbers of different types of cells in a person's blood. If the blood contains many white blood cells, a type of B-cell leukemia may be suspected. However, patients with HCL often have very low levels of white blood cells.

• Bone marrow aspiration and biopsy. These 2 procedures are similar and often done at the same time to examine the bone marrow. Bone marrow has both a solid and a liquid part. A bone marrow aspiration removes a sample of fluid with a needle. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle.

A pathologist then analyzes the sample(s) to determine the number and type of abnormal cells. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. A common site for the bone marrow aspiration and biopsy is the pelvic bone, which is located in the lower back by the hip. The skin in that area is usually numbed with medication beforehand. Other types of anesthesia (medication to block the awareness of pain) may also be used.

• Molecular testing. Your doctor may recommend running laboratory tests on a bone marrow sample to identify specific genes, proteins, and other factors unique to the leukemia.

• Immunophenotyping is the examination of antigens, a specific type of protein, on the surface of the leukemia cells. Immunophenotyping allows the doctor to confirm the exact type of leukemia.

• Cytogenetics is the examination of the leukemia cells for abnormalities in the long strands of genes called chromosomes. It helps the doctor confirm the diagnosis and may help to determine the person’s chance of recovery.

Results of these tests will also help your doctor decide if your treatment options include targeted therapy (see Treatment Options ).

• Computed tomography (CT or CAT) scan. A CT scan creates a 3-dimensional picture of the inside of the body using x-rays taken from different angles. A computer combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan shows enlarged lymph nodes or a swollen spleen and can be used to measure the size of a swollen lymph node. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill to swallow.

• Positron emission tomography (PET) or PET-CT scan. A PET scan is usually combined with a CT scan (see above), called a PET-CT scan . However, you may hear your doctor refer to this procedure just as a PET scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body. A PET scan is sometimes used to help diagnose and evaluate PLL and HCL.

After diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is HCL or PLL, these results also help the doctor describe the leukemia.

The next section in this guide is Stages . It explains the system doctors use to describe the extent of the disease. You may use the menu to choose a different section to read in the guide.

ON THIS PAGE : You will learn about the different treatments doctors use for people with HCL or PLL. Use the menu to see other pages.

This section tells you the treatments that are the standard of care for HCL and PLL. “Standard of care” means the best treatments known. When making treatment plan decisions, patients are encouraged to consider clinical trials as an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn whether the new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Your doctor can help you consider all your treatment options. To learn more about clinical trials, see the About Clinical Trials and Latest Research sections.

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team . Cancer care teams include a variety of other health care professionals, such as physician assistants, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

The goal of treatment for HCL and PLL is remission. Remission is the absence of leukemia symptoms and is also called “no evidence of disease” or NED.

Descriptions of the most common treatment options for HCL and PLL are listed below. Treatment options and recommendations depend on several factors, including whether you have HCL or PLL, possible side effects, and your preferences and overall health. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. Learn more about making treatment decisions .

Watch and wait/active surveillance

Many patients may not need treatment for years after diagnosis. For these patients, it is recommended that the disease be monitored carefully without active treatment. This is called “watch and wait” or active surveillance. This approach might be hard to understand, which can be stressful. However, patients may find that their concerns about this approach lessen over time. Talk with your health care team if you have questions or concerns.

During this time, if the leukemia shows signs of worsening, active treatment would then begin. Learn more about this approach for CLL .

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by ending the leukemia cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Or it is given by a hematologist, a doctor who specializes in treating blood disorders.

Systemic chemotherapy gets into the bloodstream to reach leukemia cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or combinations of different drugs given at the same time.

Drugs that are used to treat HCL include cladribine (Leustatin) and pentostatin (Nipent). These are the standard initial treatment for HCL. For most people with HCL, both of these drugs can bring a remission that lasts for many years.

PLL is treated with a combination of drugs, and several different regimens can be used. A specific regimen is recommended for each patient. It is based on the type of chemotherapy the patient may have received previously and the possible side effects.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away after treatment is finished.

Learn more about the basics of chemotherapy and preparing for treatment . The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases .

Immunotherapy

Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. Immunotherapy for HCL includes recombinant interferon alpha (Alferon, Intron A, Roferon-A).

Different types of immunotherapy can cause different side effects. Talk with your doctor about possible side effects for the immunotherapy recommended for you. Learn more about the basics of immunotherapy .

Targeted therapy

Targeted therapy is a treatment that targets the leukemia’s specific genes, proteins, or the tissue environment that contributes to growth and survival. This type of treatment blocks the growth and spread of leukemia cells while limiting damage to healthy cells.

Recent studies show that not all cancers have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors involved in your leukemia. This helps doctors can better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them. Learn more about the basics of targeted treatments .

A monoclonal antibody is a type of targeted therapy. It recognizes and attaches to a specific protein in the leukemia cells, and it does not affect cells that don’t have that protein. Rituximab (Rituxan) is an antibody directed against the surface protein CD20. BL22 immunotoxin is an antibody that is designed to attach to the surface protein CD22 and deliver a toxin to the leukemia cell. Both rituximab and BL22 have been used to treat HCL that did not go into remission after the initial treatment. However, BL22 is not approved by the U.S. Food and Drug Administration (FDA) for the treatment of PLL and is only available in clinical trials . Alemtuzumab (Campath) is an antibody that targets the surface protein CD52 and has been used to treat PLL. Talk with your doctor about possible side effects for a specific medication and how they can be managed.

Surgery

The spleen helps make the body’s white blood cells. Surgery to remove the spleen is called a splenectomy. This may be recommended for some patients. A surgical oncologist is a doctor who specializes in treating cancer using surgery.

Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have. Learn more about the basics of surgery .

Stem cell transplantation/bone marrow transplantation

A stem cell transplant is a medical procedure in which bone marrow that contains leukemia is replaced by highly specialized cells, called hematopoietic stem cells, that develop into healthy bone marrow. Hematopoietic stem cells are blood-forming cells found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because it is the stem cells in the blood that are typically being transplanted, not the actual bone marrow tissue.

Before recommending transplantation, doctors will talk with the patient about the risks of this treatment and consider several other factors, such as the type of leukemia, results of any previous treatment, and patient’s age and general health. Transplantation is a higher-risk procedure. It is not used often for people with HCL and PLL because it is not always an effective therapy for these diseases and because many patients with HCL or PLL are older and the risks of the procedure are higher.

There are 2 types of stem cell transplantation depending on the source of the replacement blood stem cells: allogeneic (ALLO) and autologous (AUTO). ALLO uses donated stem cells, while AUTO uses the patient’s own stem cells. ALLO transplantation is sometimes considered for younger patients with PLL when chemotherapy was not effective. However, AUTO transplantation is rarely considered for people with PLL or HCL.

In both types, the goal is to destroy all of the leukemia cells in the marrow, blood, and other parts of the body using high doses of chemotherapy and/or radiation therapy and then allow replacement blood stem cells to create healthy bone marrow.

Side effects depend on the type of transplant, your general health, and other factors. Learn more about the basics of stem cell and bone marrow transplantation .

Getting care for symptoms and side effects

HCL and PLL, as well as the treatments for these types of leukemia, often cause side effects. In addition to treatments intended to slow, stop, or eliminate the disease, an important part of care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the treatment process. People often receive treatment for the leukemia at the same time that they receive treatment to ease side effects. In fact, patients who receive both at the same time often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional support, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the leukemia, such as chemotherapy, surgery, or radiation therapy. Talk with your doctor about the goals of each treatment in your treatment plan.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and palliative care options. During and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care .

Refractory HCL and PLL

If HCL or PLL worsen during treatment, doctors call it refractory or resistant leukemia. If this happens, it is a good idea to talk with doctors who have experience in treating this type of HCL or PLL. Doctors can have different opinions about the best standard treatment plan. Also, clinical trials might be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your chosen treatment plan.

For HCL, your treatment plan may include rituximab and/or BL22 immunotoxin (see Targeted therapy, above). Palliative care will also be important to help relieve symptoms and side effects. In addition, it has recently been shown that almost all patients with HCL have a mutation in the leukemia cells in a gene called BRAF . A drug that stops a mutated BRAF gene from helping the leukemia grow and spread called vemurafenib (Zelboraf) has treated melanoma. A few patients with refractory HCL have also received this drug. Talk with your doctor about your treatment options.

For most patients, a diagnosis of refractory HCL or PLL is very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.

Remission and the chance of recurrence

A remission is when HCL or PLL cannot be detected in the body and there are no symptoms. A remission may be temporary or permanent. This uncertainty causes many people to worry that the leukemia will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the leukemia returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the disease does return. Learn more about coping with the fear of recurrence .

If HCL or PLL does return after the original treatment, it is called recurrent HCL or PLL. When this occurs, a new cycle of testing will begin again to learn as much as possible about the recurrence. After this testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the treatments described above such as chemotherapy, immunotherapy, targeted therapy, and stem cell transplantation, but they may be used in a different combination or given at a different pace. For instance, cladribine and pentostatin can work again to treat HCL if it recurs after a long period of remission. Your doctor may suggest clinical trials that are studying new ways to treat recurrent HCL or PLL. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

People with recurrent HCL or PLL often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with a recurrence .

If treatment doesn’t work

Recovery from HCL and PLL is not always possible. If the leukemia cannot be cured or controlled, the disease may be called advanced or terminal.

This diagnosis is stressful, and for many people, advanced leukemia is difficult to discuss. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Patients who have advanced HCL or PLL and who are expected to live less than 6 months may want to consider a type of palliative care called hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Learn more about advanced cancer care planning .

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss .

The next section in this guide is About Clinical Trials . It offers more information about research studies that are focused on finding better ways to care for people with cancer. You may use the menu to choose a different section to read in the guide.

ON THIS PAGE : You will read about the scientific research being done now to learn more about HCL and PLL and how to treat these types of leukemia. Use the menu to see other pages.

Doctors are working to learn more about HCL and PLL, ways to prevent these types of leukemia, how to best treat each disease, and how to provide the best care to people diagnosed with HCL or PLL. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the best diagnostic and treatment options for you.

• Targeted therapy. New treatments are being tested in clinical trials, including those that use angiogenesis inhibitors and monoclonal antibodies. Angiogenesis inhibitors are focused on stopping angiogenesis, which is the process of making new blood vessels. As discussed in the Treatment Options section, BL22 and LMB-2 are monoclonal antibodies linked to toxins that are designed to attach to the surface proteins of leukemia cells. Doctors are using these and other monoclonal antibodies in clinical trials for people with HCL when other treatments no longer work. In addition, drugs that target the BRAF gene are being studied for refractory HCL. In the future, finding out whether this gene is mutated also may help diagnose HCL.

Ibrutinib (Imbruvica) is a new drug targeting an enzyme called Bruton’s tyrosine kinase, which helps B-cell leukemia. It is being studied for several B-cell disorders, and clinical trials in PLL and HCL will be done as well.

In addition, drugs that target B-cells are also being researched for CLL. This includes drugs that target PI3K, a protein that can help CLL grow, among others.

• Immunotherapy. Researchers are looking at a new therapy called chimeric antigen receptor T-cell therapy or CAR T-cell therapy. This treatment uses the patient’s T-cells to attack the leukemia cells.

• Palliative care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current treatments to improve patients’ comfort and quality of life.

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding leukemia, explore these related items that take you outside of this guide:

• To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now .

• Visit the website of Conquer Cancer, the ASCO Foundation , to find out how to help support cancer research. Please note that this link takes you to a separate ASCO website.

The next section in this guide is Coping with Treatment . It offers some guidance in how to cope with the physical, emotional, and social changes that leukemia and its treatment can bring. You may use the menu to choose a different section to read in the guide.

ON THIS PAGE : You will read about your medical care after treatment for leukemia is completed, and why this follow-up care is important. Use the menu to see other pages.

Care for people diagnosed with leukemia doesn’t end when active treatment has finished. Your health care team will continue to check to make sure the disease has not returned, manage any side effects, and monitor your overall health. This is called follow-up care.

Your follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead. For HCL and PLL, this may include blood tests, bone marrow biopsies, and possibly imaging tests.

Learn more about the importance of follow-up care .

Watching for recurrence

One goal of follow-up care is to check for a recurrence. Leukemia recurs because small areas of leukemia cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will ask specific questions about your health. Some people may have blood tests or imaging tests done as part of regular follow-up care, but testing recommendations depend on several factors including the type and stage of cancer originally diagnosed and the types of treatment given.

The anticipation before having a follow-up test or waiting for test results can add stress to you or a family member. This is sometimes called “scan-xiety.” Learn more about how to cope with this type of stress .

Managing long-term and late side effects

Most people expect to experience side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. Other side effects called late effects may develop months or even years afterwards. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on the type of leukemia, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may have certain physical examinations, scans, or blood tests to help find and manage them.

Several long-term side effects or late effects from treatments for HCL and PLL may occur:

• Lung damage from certain drugs or radiation therapy to the lungs

• A weakened heart muscle from higher doses of doxorubicin (Adriamycin) or radiation therapy to the chest

• Infertility (inability to have a child) or premature menopause from high-dose cyclophosphamide (Neosar) or other chemotherapy

• A secondary leukemia or other type of cancer are those that develop because of the treatment for the first type of cancer. They are more common after treatment with chemotherapy. Specifically, people treated for CLL are at a higher risk for developing skin cancer and should protect their skin from the sun . They also have a higher risk of lung cancer , and smoking is strongly discouraged.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to discuss any concerns you have about your future physical or emotional health. ASCO offers forms to help create a treatment summary to keep track of the treatment you received and develop a survivorship care plan when treatment is completed.

This is also a good time to decide who will lead your follow-up care. Some survivors continue to see their oncologist or hematologist, while others transition back to the care of their family doctor or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your care will lead your follow-up care, be sure to share your cancer treatment summary and survivorship care plan forms with him or her and with all future health care providers. Details about your cancer treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship . It describes how to cope with challenges in everyday life after a leukemia diagnosis. You may use the menu to choose a different section to read in the guide.

ON THIS PAGE : You will find some questions to ask your doctor or other members of your health care team, to help you better understand your diagnosis, treatment plan, and overall care. Use the menu to see other pages.

Talking often with your health care team is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for a digital list and other interactive tools to manage your care.

Questions to ask after getting a diagnosis

• What is the exact type of leukemia that I have?

• Can you explain my pathology report (laboratory test results) to me?

• What is the progression or development of this disease? Is it possible to stop or slow the growth of the cancer? Is it curable?

Questions to ask about choosing a treatment and managing side effects

• How can my symptoms be controlled? For how long?

• What are my treatment options?

• What clinical trials are available for me? Where are they located, and how do I find out more about them?

• What treatment plan do you recommend? Why?

• What is the goal of each treatment? Is it to eliminate the leukemia, help relieve my symptoms, or both?

• Who will be part of my health care team, and what does each member do?

• Who will be leading my overall treatment?

• What are the possible side effects of each treatment, both in the short term and the long term?

• How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?

• Could this treatment affect my sex life? If so, how and for how long?

• Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before treatment begins?

• If I’m worried about managing the costs of medical care, who can help me?

• How can I keep myself as healthy as possible during and after treatment?

• What support services are available to me? To my family?

• Is there concern that other members of my family could get this disease? Is genetic testing available?

• Whom should I call with questions and problems?

• Is there anything else I should be asking?

Questions to ask about having chemotherapy, immunotherapy, or targeted therapy

• What type of treatment is recommended?

• What is the goal of this treatment?

• How long will it take to give this treatment?

• What side effects can I expect during treatment?

• What are the possible long-term effects of having this treatment?

• What can be done to relieve the side effects?

Questions to ask about having surgery

• What type of surgery will I have?

• How long will the operation take?

• How long will I be in the hospital?

• Can you describe what my recovery from surgery will be like?

• What are the possible long-term effects of having this surgery?

Questions to ask about planning follow-up care

• What is the chance that the leukemia will come back? Should I watch for specific signs or symptoms?

• What long-term side effects or late effects are possible based on the treatment I received?

• What follow-up tests will I need, and how often will I need them?

• How do I get a treatment summary and survivorship care plan to keep in my personal records?

• Who will be leading my follow-up care?

• What survivorship support services are available to me? To my family?
  

The next section in this guide is Additional Resources . It offers some more resources on this website beyond this guide that may be helpful to you. You may use the menu to choose a different section to read in the guide.