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 patient's 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<unk>  cells at the time of treatment can make certain side effects somewhat worse, as some side effects are primarily caused by the body's natural response to the dying<unk>  cells. This can result in the hospitalization of a patient whose treatment would otherwise be carried out entirely at the hematologist's 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. Most patients receive cladribine by IV infusion once a day for five to seven days, but more patients are being given the option of taking this drug once a week for six weeks. The different dosing schedules used with cladribine are roughly equally effective and safe. Relatively few patients have significant side effects other than fatigue and a high fever caused by the cancer cells dying, although complications such as infection and acute kidney failure have been seen. Pentostatin is chemically similar to cladribine, and has a similar success rate and side effect profile, but it is always given over a much longer period of time, usually one dose by IV infusion every two weeks for 3-6 months. During the weeks following treatment, the patients' immune systems are severely weakened, but their bone marrow will begin to produce normal blood cells again. Treatment often results in long-term remission. About 85% of patients achieve a complete response from treatment with either cladribine or pentostatin, and another 10% receive some benefit from these drugs, although no permanent cure for this disease is known. If the cancer cells return, the treatment may be repeated and should again result in remission, although the odds of success decline with repeated treatment. Remission lengths vary significantly, from one year to more than twenty years. The median patient can expect a treatment-free interval of about ten years. 2-Chlorodeoxyadenosine (Cladribine) induced complete responses in patients with<unk>  resistant to DCF , suggesting a lack of cross-resistance. Also, 2-CdA is not prohibitively toxic in patients intolerant of DCF (Pentostatin). Second-line therapy If a patient is resistant to either cladribine or pentostatin, then second-line therapy is pursued. * Monoclonal antibodies: The most common treatment for cladribine-resistant disease is infusing monoclonal antibodies that destroy cancerous B cells. Rituximab is by far the most commonly used. Most patients receive one IV infusion over several hours each week for 4-8 weeks. Two partial and 10 complete responses resulted fromf 15 patients with relapsed disease, for a total of 80% responding. The median patient (including nonresponders) did not require further treatment for more than 3 years. This eight-dose study had a higher response rate than a four-dose study at Scripps, which achieved only 25% response rate. Rituximab has successfully induced a complete response in<unk>  Cell-Variant. Rituximab's major side effect is serum sickness, commonly described as an "allergic reaction", which can be severe, especially on the first infusion. Serum sickness is primarily caused by the antibodies clumping during infusion and triggering the complement cascade. Although most patients find that side effects are adequately controlled by antiallergy drugs, some severe, and even fatal, reactions have occurred. Consequently, the first dose is always given in a hospital setting, although subsequent infusions may be given in a physician's office. Remissions are usually shorter than with the preferred first-line drugs, but hematologic remissions of several years' duration are not uncommon. Other B cell-destroying monoclonal antibodies such as alemtuzumab, ibritumomab tiuxetan and I-131 tositumomab may be considered for refractory cases. * Interferon-alpha is an immune system hormone that is very helpful to a relatively small number of patients, and somewhat helpful to most patients. In about 65% of patients, the drug helps stabilize the disease or produce a slow, minor improvement for a partial response. The typical dosing schedule injects at least 3 million units of interferon-alpha (not pegylated versions) three times a week, although the original protocol began with 6 months of daily injections. Some patients tolerate IFN-alpha very well after the first fw weeks, while others find that its characteristic flu-like symptoms persist. About 10% of patients develop a level of depression. By maintaining a steadier level of the hormone in the body, daily injections might cause fewer side effects in selected patients. Drinking at least 2 liters of water each day, while avoiding caffeine and alcohol, can reduce many of the side effects. A drop in blood counts is usually seen during the first 1-2 months of treatment. Most patients find that their blood counts get worse for a few weeks immediately after starting treatment, although some patients find their blood counts begin to improve within just 2 weeks. Typically, 6 months are neededto figure out whether this therapy is useful. Common criteria for treatment success include: * Normalization of hemoglobin levels (above 12.0 g/dL), * A normal or somewhat low platelet count (above 100 K/µL), and * A normal or somewhat low absolute neutrophil count (above 1.5 K/µL). If it is well tolerated, patients usually take the hormone for 12 to 18 months. An attempt may be made then to end the treatment, but most patients discover that they need to continue taking the drug for it to be successful. These patients often continue taking this drug indefinitely, until either the disease becomes resistant to this hormone, or the body produces an immune system response that limits the drug's ability to function. A few patients are able to achieve a sustained clinical remission after taking this drug for six months to one year. This may be more likely when IFN-alpha has been initiated shortly after another therapy. IFN-alpha is considered the drug of choice for pregnant women with active<unk> , although it carries some risks, such as the potential for decreased blood flow to the placenta. IFN-alpha works by sensitizing the<unk>  cells to the killing effect of the immune-system hormone TNF-alpha, whose production it promotes. IFN-alpha works best on classic<unk>  cells that are not protectively adhered to vitronectin or fibronectin, which suggests that patients who encounter less fibrous tissue in their bone-marrow biopsies may be more likely to respond to IFN-alpha therapy. It also explains why unadhered<unk>  cells, such as those in the bloodstream, disappear during IFN-alpha treatment well before reductions are seen in adhered<unk>  cells, such as those in the bone marrow and spleen. Other treatments Splenectomy can produce long-term remissions in patients whose spleens seem to be heavily involved, but its success rate is noticeably lower than cladribine or pentostatin. Splenectomies are also performed for patients whose persistently enlarged spleens cause significant discomfort or in patients whose persistently low platelet counts suggest idiopathic thrombocytopenic purpura. Bone marrow transplants are usually shunned in this highly treatable disease because of the inherent risks in the procedure. They may be considered for refractory cases in
Hairy cell leukemia
Chronic lymphocytic leukemia
Prolymphocytic leukemia
B-cell leukemia
T-lymphoblastic leukemia/lymphoma
T-cell leukemia
Large granular lymphocytic leukemia
Leukemoid reaction