Hairy Cell Leukemia

 

 

OVERVIEW

Leukemia is a cancer that begins in the hematopoietic (blood-forming) cells of the bone marrow, the soft, spongy inner part of such bones as the skull, pelvis, and backbones. The hematopoietic cells produce three types of blood cells: red, white, and platelets. Red cells carry oxygen to the muscles and provide the body with energy. Platelets are the clotting cells that prevent bleeding. White blood cells are the cells of the immune system. Leukemia occurs when abnormal blood cells reproduce and crowd out normal cells in the bone marrow. Most leukemias are cancers of the white blood cells. Abnormal leukemic cells can spread the cancer to other parts of the body including the lymph nodes, liver, spleen, skin, and central nervous system. (When cancer starts elsewhere and spreads to the bone marrow, it is not leukemia.)

Leukemia can be acute or chronic (such as hairy cell leukemia), depending on whether the abnormal cells are mature or immature. In chronic leukemia, bone marrow cells mature but they do not function properly and do not fight infections as would normal white blood cells. Because chronic leukemic cells can live much longer than normal white blood cells, chronic leukemia is a slower-forming disease that usually worsens gradually.

Hairy cell leukemia is a slowly progressing cancer of the lymphocytes (white blood cells), but it is treated as a separate disease from chronic lymphocytic leukemia because it has different symptoms and treatments. About 2 percent of leukemias are hairy cell leukemia.

At The Cancer Center, our chief of the Division of Leukemia, Stuart L. Goldberg, M.D., is one of the nation's foremost authorities and researchers of all types of leukemia. He also serves as the medical advisor to the Northern NJ Chapter of the Leukemia and Lymphoma Society. He leads an active leukemia research team that is conducting about 30 clinical trials for new treatments for leukemia. Almost all patients with leukemia at The Cancer Center are enrolled in a clinical trial. National Cancer Institute research groups, pharmaceutical companies, and independent research agencies frequently turn to Dr. Goldberg to collaborate on clinical research studies because of his successful accruals (enrollments) and his ability to attract new patients to The Cancer Center.

A hematologist/oncologist, Dr. Goldberg also specializes in the use of stem cell transplantation to treat patients with leukemia. He and his colleagues at The Cancer Center's Adult Blood and Marrow Stem Cell Transplantation Program pioneered the use of reduced-intensity "mini" transplants for older patients and those who are too frail to undergo the rigors of a standard stem cell transplant. Our stem cell transplantation program is one of the nation's 10 largest; each year more than 200 stem cell transplants are performed here.

A crucial component to the accurate diagnosis of leukemia is The Cancer Center's Special Diagnostic Immunology Laboratory, one of only several sites in New Jersey where comprehensive tests are available to detect cancer at the molecular level and to stage and classify it.

 

DIAGNOSTIC SERVICES

A diagnosis of leukemia is made after several tests have been completed. Samples of cells are taken from a patient's blood and bone marrow. Other tissue and cell samples (possibly from the lymph nodes and cerebrospinal fluid) may be taken in order to guide treatment. A crucial component to the accurate diagnosis of leukemia is The Cancer Center's Special Diagnostic Immunology Laboratory, one of only several sites in New Jersey where comprehensive tests are available to detect cancer at the molecular level and to stage and classify it.

Radiologic tests used to diagnose leukemia may include a chest X-ray, bone scan, CT scan, MRI, and/or ultrasound.

 

RISK FACTORS

Smoking is directly related to leukemia because cancer-causing substances in tobacco smoke are absorbed through the lungs and then the blood stream. Environmental risk factors include cancer-causing industrial chemicals, such as benzene, and high-dose radiation exposure. Secondary (post-treatment) leukemias can result from chemotherapy and radiation therapy administered for other cancers.

 

SYMPTOMS

The onset of hairy cell leukemia occurs gradually, but there may be sudden bruising, severe anemia, and an enlarged spleen. The disease occurs six times more often in men than women and usually in middle age. Some other common symptoms of chronic leukemias include:

  • fever, chills, and other flu-like symptoms
  • frequent infections
  • weakness and fatigue (due to anemia)
  • loss of appetite and/or weight loss
  • swollen or tender lymph nodes, liver, or spleen
  • easy bruising or bleeding
  • tiny red spots (called petechiae) under the skin
  • swollen or bleeding gums
  • sweating, especially at night
  • bone or joint pain

 

HAIRY CELL LEUKEMIA TREATMENT OPTIONS

If you are not having symptoms, no treatment will be given but your hematologist/oncologist will want to see you every few months. Your doctor may suggest a splenectomy (removal of the spleen) if you have an enlarged spleen. He/she may also advise chemotherapy if you have a low blood count. The decision to receive treatments can often be quite difficult but may be based on the patient's subtype of leukemia, general health, age, and overall desires. Shortly after diagnosis a comprehensive review of the type of leukemia is performed to determine the aggressiveness of the cancer and to provide prognostic information that may be helpful in chosing a treatment course.

Chemotherapy

Chemotherapy is used to destroy leukemic cells and to help control symptoms and side effects of the cancer. Cladribine and Pentostatin are chemotherapy agents that have shown remarkable effectiveness in this rare disease. A one week course of outpatient cladribine yield significant remissions in most patients. A more complete discussion of the risks and benefits of chemotherapy will be provided during the extensive consultation process with members of the Division of Leukemia. Additional updates on current treatment approaches are available from the Leukemia and Lymphoma Society (www.LLS.org) and the National Cancer Institute (www.cancer.gov).

Antibody Therapies

One of the major advances in the past decade for hairy cell leukemia has been the use of antibodies which specifically target the cancer cells. Rituximab (Rituxan) has been used in the outpatient Cancer Center to treat patients with recurrent leukemia and elderly patients deemed to frail for chemotherapy. Rituximab attacks a target (CD20) on the surface of most hairy cells and early reports have been very encouraging.

Surgery

The only type of surgery used to treat leukemia is a splenectomy (removal of the spleen) if the organ is enlarged and pressing against other internal organs and causing pain.

Supportive Treatment

Whole blood transfusions or transfusions of platelets, white blood cells, and red blood cells may be given as supportive treatment.