Hairy cell Leukemia

Hairy cell leukemia (HCL) is a rare type of chronic leukemia that affects the B lymphocytes. It accounts for approximately 2% of all leukemia cases.

It is called “hairy cell” because of the characteristic appearance of the leukemic lymphocytes which have tiny projections, or “hairs,” on their surface. Other names for HCL include leukemic reticuloendotheliosis, reticulosis, aleukemic reticuloendotheliosis, and reticulum cell leukemia.

As with many types of cancer, the exact cause of HCL is not known. Researchers are investigating to see if there is a link between exposure to radiation, viruses and certain chemicals with the development of HCL. So far, these associations are not conclusive.

Although doctors do not know what causes HCL, some groups do seem to be at a higher risk of developing the disease. These include:

• Men
• Middle-aged people (around 55)
• Caucasians
• People of Jewish descent

It is important to note that many people with risk factors never develop the disease, and many people with HCL have no risk factors.

Many of the early signs and symptoms of HCL are related to the leukemia cells crowding out the normal cells in the marrow. This accumulation of hairy cells can cause low red blood cells (anemia), low platelets (thrombocytopenia), and low white blood cells (neutropenia). As a result, you may notice increased infections, bruising and bleeding easily, and weakness or shortness of breath.

Hairy cells can also often gather in your liver and spleen, causing enlargement of these organs. In more than 90% of HCL patients, an enlarged spleen, or splenomegaly, is noted. Liver enlargement (hepatomegaly) can be found in 40-60% of patients. Enlargement of these organs can cause a feeling of “fullness” or discomfort in your upper abdomen and unexplained weight loss.

HCL can be identified with a standard CBC blood test and bone marrow biopsy and aspirate.

Blood tests may be drawn at a laboratory or in the hospital. Blood samples may be taken by a nurse or technician, usually from a vein in your arm, using a needle and syringe. You may feel a burning or stinging sensation when the needle is poking through your skin, but the discomfort should only last a few seconds.

Once the blood samples are drawn, the technician will send them off for microscopic analysis. Hairy cells may be difficult to find in the blood.

Bone marrow aspiration and biopsy will be necessary to confirm the disease. Aspiration and biopsy are done at the same time, and may be done in your doctor’s clinic or at the cancer center.

During bone marrow testing, you will be asked to lie on your side. Your doctor will use an antiseptic to cleanse the biopsy area, which is usually your hipbone but may also be your breastbone. After the area is cleansed, the doctor will use a small needle to inject some anesthetic to numb the site. You may feel a stinging sensation while the medication is injected.

Then, he or she will use a longer needle with a syringe to go right into the bone and withdraw some liquid marrow. This is called the aspiration. In patients with HCL, it may be difficult for the doctor to obtain the aspiration because of changes that the disease causes in the marrow.

After withdrawing the aspiration sample, the doctor will then insert a slightly larger, hollow needle into the site. With this needle, they will remove a small piece of your bone with some marrow attached to it. This is called the biopsy. You may feel a dull pressure when the needles are inserted into your bone.

The aspiration and biopsy samples are then sent to the laboratory to be analyzed.

When the procedure is complete, your doctor or nurse will cover the area with a bandage. While the anesthetic is still active, you will not have any discomfort at the biopsy site, but you may need a mild analgesic later on in the day.

Treatment of Hairy Cell Leukemia

Although you may be anxious to begin therapy and fight your cancer, treatment does have possible serious complications and it may be more beneficial for you to wait.

HCL is a very treatable form of leukemia. Remission rates as high as 85-91% are reported with just a single round of chemotherapy treatment.

The most common and effective medication used to treat HCL is Leustatin (cladribine). It is given for seven days through your vein. Common side effects from Leustatin include fevers and bone marrow damage resulting in low white cells, platelets and red blood cells.

If HCL does not respond to Leustatin, or you have a relapse in your disease following a remission, Nipent (pentostatin) is another drug that may be ordered. It is also given through your vein, and is administered every other week for three to six months.

Taking Care of Yourself

Although HCL is highly treatable, it is still cancer! It is okay for you to feel scared, anxious, lonely, or exhausted! Make sure that while you are worrying about your disease, your home life, your job, and all those other things, you try to take some time AWAY from worrying. Easy to say, but not so easy to do!

Find a way to take some time to do things that make you happy and relaxed. What about tea with a friend that always makes you laugh? Or a silly animated movie? Maybe pick up that knitting project that has been sitting by your sofa since last Christmas - it’s hard to worry and count at the same time!

Anything you can do to raise your spirits, even if just for a short time, will help to rejuvenate you and give you a boost to fight that cancer!

_Sources:

_{Becker, Susan. "The Disease With Hope: Hairy Cell Leukemia" Clinical Journal of Oncology Nursing October 2007 11:731-735.}

_{Grever, Michael. "How I Treat Hairy Cell Leukemia" Blood January 2010 115:21-28.}

_{Sheldon, Jessica, Wisenor, Debra, and Roberts, George. "Hairy Cell Leukemia" Continuing Education Topics and Issues April 2005 62-65.}