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When does hairy cell leukemia (HCL) need to be treated?

Treatment may be advised for people with HCL if they have low blood cell counts, recurrent infections, an enlarged spleen or lymph nodes, or other bothersome symptoms.

Treatment doesn't cure HCL, but it can help with symptoms, stop the HCL from progressing, and help people live longer.

Which treatments are used for hairy cell leukemia (HCL)?

The first treatment for HCL is most often chemotherapy (chemo), with either cladribine (2-CdA) or pentostatin. Sometimes the monoclonal antibody rituximab is given after the chemo.

Most often HCL responds well to these drugs, and the responses often last for many years.

If the leukemia comes back at some point, it can often be treated with the same drug again, especially if the HCL stayed in remission for a long time (typically at least 2 years). If one chemo drug doesn't work, another can be tried.

In rare cases where HCL doesn't respond to chemo, or if the response to treatment doesn't last for long, other types of medicines might be tried. For example, targeted drugs known as BRAF inhibitors, such as vemurafenib (sometimes with rituximab) or dabrafenib (plus trametinib) might be options. BTK inhibitors might also be an option, as might rituximab alone or peginterferon-alfa, a type of immunotherapy.

If a person is uncomfortable because of an enlarged spleen, surgery to remove the spleen (splenectomy) can often help relieve pain.

Treatment of other problems caused by hairy cell leukemia (HCL)

Like chronic lymphocytic leukemia (CLL), HCL can cause low blood counts and infections. Treatment of these problems is discussed in Supportive or Palliative Care for Chronic Lymphocytic Leukemia.