Alternative Names: Leukemic reticuloendotheliosis; HCL; Leukemia - hairy cell

Causes, incidence, and risk factors:

Hairy cell leukemia (HCL) is an uncommon cancer of the blood. It can be one of the causes of low numbers of normal blood cells. The disease is caused by the abnormal growth of B cells that can look "hairy" under the microscope because they have fine projections coming from their surface.

The cause of this disease is unknown. It affects men 5 times more often than women, and the average age of onset is 55. Hairy cell leukemia is rare and only accounts for about 2% of the leukemias diagnosed each year.

Symptoms:

• Weakness
• Fatigue
• Weight loss
• Easy brusing or bleeding
• Recurrent infections and fevers�
• Excessive sweating (especially at night)
• Swollen lymph glands
• Early satiety (feeling full after eating only a small amount)

Signs and tests:

• A physical examination can reveal an enlarged spleen or liver
• A bone marrow biopsy shows hairy cells
• A peripheral blood smear may show hairy cells
• A special test done on blood or bone marrow cells (tartrate-resistant acid phosphatase) can confirm the diagnosis
• An abdominal CT scan shows an enlarged spleen and liver
• A CBC shows low white blood cell count, low red cell count, and low platelets

Treatment:

Early in the course of the disease, no treatment may be necessary. Some patients may need an occasional blood transfusion.

If treatment is needed because of very low blood counts, a variety of chemotherapy drugs (cladribine, pentostatin) can be used. Interferon is also used. In the majority of cases these drugs can produce a remission (complete relief from the disease) that lasts for many years. It is unclear if chemotherapy will cure the disease, however, because most patients will relapse over time.

The removal of the spleen may improve blood counts, but is unlikely to cure the disease. Symptomatic treatment of infections with antibiotics or low blood counts with growth factors and transfusions may be required.

Support Groups:

Expectations (prognosis):

Newer treatments using chemotherapy have greatly improved the survival of patients with hairy cell leukemia. Most patients with hairy cell leukemia can expect to live 10 years or longer with the disease.

Complications:

The low blood counts caused by hairy cell leukemia can lead to infections, fatigue, and excessive bleeding.

Calling your health care provider:

Call your health care provider if significant bleeding occurs. Also call if signs of infection are present, including persistent fever, cough, or general ill feeling.

Prevention:

There is no known prevention.