Hairy Cell Leukemia

1 What is Hairy Cell Leukemia?

Hairy cell leukemia is a rare, slow-growing cancer of the blood. It is characterized by increased number of B- lymphocytes, a type of white blood cell that fights infection, in bone marrow.

These excess B cells are abnormal and look "hairy" under a microscope. Increased number of leukemia cells suppresses the formation of white blood cells, red blood cells and platelets.

Hairy cell leukemia is more common in men compared to women and is diagnosed more often in middle-aged or elderly.

This disease is considered a chronic as it may never disappear completely. However, treatment can lead to a remission for years.

2 Symptoms

Prominent signs or symptoms of hairy cell leukemia can be revealed upon a blood test.

Sign and symptoms are more or less similar to a other diseases. Some early signs are as follows:

  • Fullness in abdomen
  • Fatigue
  • Easy bruising
  • Recurring infections
  • Weakness
  • Weight loss

Make an appointment with your doctor if you have any persistent signs and symptoms that makes you suspicious.

3 Causes

It's not clear what causes hairy cell leukemia.

Doctors know that cancer occurs when cells develop errors 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.

4 Making a Diagnosis

When suspected for hairy cell leukemia, your doctor may refer you to specialists who treats diseases of the blood and bone marrow (hematologist) to receive a diagnosis.

Be prepared before visiting a doctor. Follow these tips to make the conversation clear, precise and fruitful:

What to expect from your doctor? 

Note down symptoms you are experiencing and list out all medications, vitamins or supplements that you're taking currently. 

Write down key personal information, including any major stresses or recent life changes. 

Make a list of all medications, vitamins or supplements that you're taking. Consider taking a family member or friend along.

Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.

Make a list of questions you wish to ask your doctor. Few important questions might be as follows: 

  • What kinds of tests do I need?
  • Will I require treatment for my hairy cell leukemia?
  • If I don't have treatment, will my leukemia worsen? If I require treatment, what are my options?
  • Will treatment cure my hairy cell leukemia?
  • What are the side effects of each treatment option? Is there one treatment you feel is best for me?
  • How will cancer treatment affect my daily life?
  • I have these other health conditions. How can I best manage them together?
  • Are there any restrictions that I need to follow?
  • Should I see a specialist?
  • What will that cost and will my insurance cover it?
  • Are there brochures or other printed material that I can take with me? What websites do you recommend?

What your doctor may ask?

  • When was the first time you experienced symptoms?
  • Are your symptoms severe?
  • Is there any factor that improves or worsens your symptoms?
  • Do you have history of cancer in your family?

To diagnose hairy cell leukemia, doctor may recommend tests and procedures that include:

  • Physical exam: It involves checking the spleen if it has enlarged. An enlarged spleen may cause a sensation of fullness in your abdomen that makes it uncomfortable to eat. Similarly, lymph nodes enlargement and presence of leukemia cells in lymph nodes can be tested.
  • Blood tests: Blood tests, such as the complete blood count can be carried out to monitor the levels of blood cells in your blood. Significant drop in number of red blood cells, white blood cells and platelets can signal towards hairy cell leukemia. Similarly, other blood test known as a peripheral blood smear directly searches for hairy cell leukemia cells in a sample of your blood.
  • Bone marrow biopsy: During a bone marrow biopsy, a small amount of bone marrow is removed from hip region and both leukemia cells and healthy cells are monitored in the sample.
  • Computerized tomography (CT) scan: CT scan is generally used to detect enlargement of spleen and lymph nodes.

5 Treatment

The progression of hairy cell leukemia might be very slow and it may not always require immediate treatment. However, intense pain and severe symptoms should be treated as soon as possible.

Absolute cure for the disease is absent so treatments are effective at putting hairy cell leukemia in remission for years.

Chemotherapy: It is considered as the first line of treatment for hairy cell leukemia. However, it only expected to reduce the sign and symptoms for years.

Two most commonly used drugs for the treatment of hairy cell leukemia are as follows:

  • Cladribine: It is the primary drug infused into a vein over several days. People administering cladribine experience a complete remission that can last for several years. Relapse cases can again be treated with same drugs.
  • Pentostatin: Pentostatin is as effective as cladribine but it is given on a different schedule. Infusions are administered every other week for three to six months.

Side effects of both drugs include infection and fever.

Biological treatments: Biological therapy helps your immune system to react fast against cancer cells and destroy them. Two types of biological treatments used in hairy cell leukemia are as follows:

  • Interferon: Interferons are reserved for the cases when chemotherapy is not effective or for the patients who cannot undergo chemotherapy a tall. Most people experience partial remission with interferon which can be considered for a year. Side effects like flu-like symptoms, fever and fatigue can be expected.
  • Rituximab: Rituximab is a monoclonal antibody generally considered as second line drug for the treatment of hairy cell leukemia. If Interferons do not works on you then rituximab is the drug of choice. Side effects of rituximab include fever and infection.

Surgery (splenectomy): Surgery to remove your spleen may be essential for ruptured spleen or if symptoms like severe pain is associated. Surgical procedures only restores normal blood counts rather than completely curing the disease and it carries a risk of bleeding and infection.

6 Alternative and Homeopathic Remedies

Complementary and alternative remedies can't cure your hairy cell leukemia but it may offer helpful ways to cope during and after treatment.

Few of them could be as follows:

  • Acupuncture: A practitioner inserts tiny needles into your skin at precise points during an acupuncture session. Acupuncture may help relieve nausea and vomiting caused by chemotherapy. However, it is not safe if you have low blood counts or if you are taking blood thinners.
  • Aromatherapy: Oils such as lavender can be massaged into your skin, added to bath water or heated to release their scents. It may help to improve your mood and relieve stress. It is safe but make sure that you are not allergic to incorporated ingredients.
  • Massage: A massage therapist uses his or her hands to knead your muscles and soft tissues. Massage may help relieve anxiety and fatigue. People with cancer shouldn't have a massage if their blood counts are low. Deep pressure during massage should be avoided. Mind-body therapies like meditation may help you relax and reduce the pain.

7 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with hairy cell leukemia.

Hairy cell leukemia is considered as a chronic form of cancer and requires continuous monitoring even after remission.

Consider yourself to know better about the disease to cope with treatment and lifestyle adjustment.

Seek information from health care professional ask them to recommend some reliable sources to get you started.

Apart from family and friends, getting connected to other cancer survivors provide a unique network of support.

Focus on balanced diet, regular exercises and sanitation. Avoiding the relapse of disease is not under your control so stay positive and stay fit to deal with the situation.

8 Risks and Complications

There are several risks and complications associated with hairy cell leukemia.

Certain factors may increase your risk of developing hairy cell leukemia which are as follows:

  • Ethnicity. Hairy cell leukemia is more common in men of Ashkenazi Jewish ancestry than men of other ethnic groups.
  • Exposure to radiation: Long term exposure to the sources of X-rays may have a higher risk of developing hairy cell leukemia.
  • Exposure to chemicals: Few studies suggest that industrial and agricultural chemicals could play a role in hairy cell leukemia development
  • Exposure to sawdust. Some studies have found a link between working with wood and sawdust and an increased risk of hairy cell leukemia.

Progressive and untreated hairy cell leukemia may crowd out healthy blood cells leading to serious complications. Low white blood cell counts may put you at risk of infections.

Similarly, low platelet counts retards the cessation of blood after injuries whereas, low red blood cell count leads to anemia.

Some studies have found that people with hairy cell leukemia may have an increased risk of developing a second type of cancer. However it is still unclear that 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 lymphoma.

9 Related Clinical Trials