General Information About Hairy Cell Leukemia

Hairy cell leukemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).

Hairy cell leukemia is a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia because the leukemia cells look "hairy" when viewed under a microscope.

Leukemia may affect red blood cells, white blood cells, and platelets.

Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell.

A myeloid stem cell becomes one of three types of mature blood cells:

• Red blood cells that carry oxygen and other substances to all tissues of the body.
• Granulocytes, which are white blood cells that help fight infection and disease.
• Platelets that form blood clots to stop bleeding.

A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells):

• B lymphocytes that make antibodies to help fight infection.
• T lymphocytes that help B lymphocytes make antibodies to help fight infection.
• Natural killer cells that attack cancer cells and viruses.

In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. The abnormal lymphocytes are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell.

Signs and symptoms of hairy cell leukemia include infections, tiredness, and pain below the ribs.

These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions. Check with your doctor if you have any of the following:

• Weakness or feeling tired.
• Frequent infections.
• Easy bruising or bleeding.
• Shortness of breath.
• Pain or a feeling of fullness below the ribs.
• Painless lumps in the neck, underarm, stomach, or groin.

Tests that examine the blood and bone marrow are used to diagnose hairy cell leukemia.

In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures:

• Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • The number of red blood cells, white blood cells, and platelets.
  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
  • The portion of the sample made up of red blood cells.
  
• Peripheral blood smear: A procedure in which a sample of blood is checked for cells that look "hairy," the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells.
• Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
• Flow cytometry: A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of the cells, such as size, shape, and the presence of tumor (or other) markers on the cell surface. The cells from a sample of a patient’s blood, bone marrow, or other tissue are stained with a fluorescent dye, placed in a fluid, and then passed one at a time through a beam of light. The test results are based on how the cells that were stained with the fluorescent dye react to the beam of light. This test is used to help diagnose and manage certain types of cancers, such as leukemia and lymphoma.
• Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
• Immunophenotyping: A laboratory test that uses antibodies to identify cancer cells based on the types of antigens or markers on the surface of the cells. This test is used to help diagnose specific types of leukemia.
• Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a sample of blood or bone marrow are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in certain chromosomes may be a sign of cancer. Cytogenetic analysis is used to help diagnose cancer, plan treatment, or find out how well treatment is working.
• BRAF gene testing: A laboratory test in which a sample of blood or tissue is tested for certain changes in the BRAF gene. A BRAF gene mutation is often found in patients with hairy cell leukemia.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A CT scan of the abdomen may be done to check for swollen lymph nodes or a swollen spleen.

Certain factors affect treatment options and prognosis (chance of recovery).

The treatment options depend on the following:

• The number of hairy (leukemia) cells and healthy blood cells in the blood and bone marrow.
• Whether the spleen is swollen.
• Whether there are signs or symptoms of leukemia, such as infection.
• Whether the leukemia has recurred (come back) after previous treatment.

The prognosis depends on the following:

• Whether the hairy cell leukemia does not grow or grows so slowly it does not need treatment.
• Whether the hairy cell leukemia responds to treatment.

Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission.