Prevention
Because the cause is unknown, no effective preventive measures can be taken, but the U.S. Institute of Medicine permits an association to exposure to herbicides (atrazine). The U.S. Department of Veterans Affairs also considers it a service disability related to Agent Orange. Because the disease is rare, routine screening is not cost effective. Treatment
Several treatments are available, and successful control of the disease is common. Not everyone needs treatment immediately. Treatment is usually given when the symptoms of the disease interfere with the patients everyday life, or when white blood cell or platelet counts decline to dangerously low levels, such as an absolute neutrophil count below 1000 cells per microliter (1.0 K/μL). Not all patients need treatment immediately upon diagnosis. Treatment delays are less important than in solid tumors. Unlike most cancers, treatment success does not depend on treating the disease at an early stage. Because delays do not affect treatment success, no standards exist for how quickly a patient should receive treatment. Waiting too long, though, can cause its own problems, such as an infection that might have been avoided by proper treatment to restore immune-system function. Also, having a higher number of hairy cells at the time of treatment can make certain side effects somewhat worse, as some side effects are primarily caused by the bodys natural response to the dying hairy cells. This can result in the hospitalization of a patient whose treatment would otherwise be carried out entirely at the hematologists office. Single-drug treatment is typical.
Unlike most cancers, only one drug is normally given to a patient at a time. While monotherapy is normal, combination therapy—typically using one first-line therapy and one second-line therapy—is being studied in current clinical trials, and is used more frequently for refractory cases. Combining rituximab with cladribine or pentostatin may or may not produce any practical benefit to the patient. Combination therapy is almost never used with a new patient. Because the success rates with purine analog monotherapy are already so high, the additional benefit from immediate treatment with a second drug in a treatment-naïve patient is assumed to be very low. For example, one round of either cladribine or pentostatin gives the median first-time patient a decade-long remission; the addition of rituximab, which gives the median patient only three or four years, might provide no additional value for this easily treated patient. In a more difficult case, however, the benefit from the first drug may be substantially reduced, so a combination may provide some benefit. First-line therapy
Cladribine (2CDA) and pentostatin (DCF) are the two most common first-line therapies. They both belong to a class of medications called purine analogs, which have mild side effects compared to traditional chemotherapy regimens. Cladribine can be administered by injection under the skin, by infusion over a few hours into a vein (inravenous - IV), or by a pump worn by the patient that provides a slow drip into a vein, 24 hours a day for 7 days.