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Submitted by: P Nagpal Nagpal

Hairy Cell Leukemia

Overview

Hairy cell leukemia (HCL) is a rare cancer of the blood. It affects B cells, a type of white blood cell (lymphocyte).

Causes

HCL is caused by the abnormal growth of B cells. The cells can look “hairy” under the microscope because they have fine projections coming from their surface.

HCL can lead to low numbers of normal blood cells.

The cause of this disease is unknown. It affects men more often than women. The average age of onset is 55. Hairy cell leukemia is rare.

Risk and Symptom

* Easy bruising or bleeding

* Excessive sweating (especially at night)

* Fatigue

* Feeling full after eating only a small amount

* Recurrent infections and fevers

* Swollen lymph glands

* Weakness

* Weight loss

* Exposure to radiation. People exposed to radiation, such as those who work around X-ray machines or those who received radiation treatment for cancer, may have a higher risk of developing hairy cell leukemia.

* Exposure to chemicals. Industrial and agricultural chemicals could play a role in hairy cell leukemia development. However, some studies have found this not to be the case.

* Exposure to sawdust. Some studies have found a link between working with wood and sawdust and an increased risk of hairy cell leukemia. But this connection hasn’t been proven conclusively.

Exams and Tests

During a physical exam, the doctor may be able to feel a swollen spleen or liver. An abdominal CT scan may be done to confirm this swelling.

A complete blood count shows low levels of white and red blood cells as well as platelets.

Blood tests and a bone marrow biopsy can detect hairy cells. Flow cytometry or a test called TRAP can confirm the cancer diagnosis.

Treatment

Treatment may not be needed for the early stages of this disease. Some patients may need an occasional blood transfusion.

If treatment is needed because of very low blood counts, a variety of chemotherapy drugs can be used. A drug called interferon is also used. In most cases, chemotherapy can relieve the symptoms of the disease for many years. (When the signs and symptoms go away, you are said to be in remission.) Interferon can relieve symptoms but is unlikely to lead to remission.

Removing the spleen may improve blood counts, but is unlikely to cure the disease. Antibiotics can be used to treat infections. People with low blood counts will receive growth factors and, possibly, transfusions.

Treatment isn’t always necessary for people with hairy cell leukemia. Because this cancer progresses very slowly and sometimes doesn’t progress at all, some people prefer to wait to treat their cancer only if it causes signs and symptoms. The majority of people with hairy cell leukemia eventually need treatment.

Though you may be eager to rid your body of cancer if you’ve been diagnosed with hairy cell leukemia, there’s no advantage to early treatment. Unlike some other types of cancer, hairy cell leukemia is quite treatable at all stages, meaning that waiting to treat your cancer won’t make remission any less likely.

If your hairy cell leukemia causes signs and symptoms, you may decide to undergo treatment. There is no cure for hairy cell leukemia. But treatments are effective at putting hairy cell leukemia in remission for years.

Chemotherapy

Doctors consider chemotherapy drugs the first line of treatment for hairy cell leukemia. The 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 (Leustatin). Treatment for hairy cell leukemia typically begins with cladribine. You receive a continuous infusion of the drug into a vein over seven 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.

* Pentostatin (Nipent). Pentostatin causes remission rates similar to cladribine, but it’s given on a different schedule. People who take pentostatin receive infusions every other week for three to six months. Side effects of pentostatin may include fever, infection and kidney problems.

Biological treatments

Biological therapy (immunotherapy) 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: –

* Interferon. 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.

* Rituximab (Rituxan). Rituximab is a monoclonal antibody approved to treat non-Hodgkin’s lymphoma, 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. Side effects of rituximab include fever and infection.

Surgery

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. All surgery carries a risk of bleeding and infection.

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