Currently, there is no cure for patients who suffer from chronic lymphocytic leukemia, so most individuals in the early stages of the disease are not treated as it tends to progress slowly.Many individuals lead normal and active lives for many years – even decades. Early chronic lymphocytic leukemia intervention doesn’t improve survival time or quality of life. Many specialists feel that it is best to plan treatment in relation to the stage of the disease. This enables a rational decision process, taking into account the survival rate since diagnosed and the progression of the disease.

Most patients with low-risk chronic lymphocytic leukemia have a 10-year average survival rate and most don’t die from leukemia. The issue of when treatment should be initiated has shown that with low-risk chronic lymphocytic leukemia (Rai stage 0) patients there is no advantage in beginning treatment at time of diagnosis. These patients are best observed until their disease enters a more aggressive state (Rai stage I or II)—when lymph nodes, liver, and spleen are enlarged and when the body inability to produce healthy number of marrow cells (red, white, and platelets) is present. When in a chronic aggressive stage, bleeding problems and serious infection issues may manifest.

For patients with intermediate-risk chronic lymphocytic leukemia (Rai stages I and II) the average survival rate is 5 years. The critical issue is the tumor burden because of the enlarged lymph nodes and liver. Few and modest symptoms due to the disease may not be immediately treated, similar to low-risk chronic lymphocytic leukemia. This is in contrast to patients with moderately severe to severe symptoms with high tumor burden (large lymph nodes, spleen or liver or with very high lymphocyte count) who often require immediate therapy. When treatment is required, most specialists use chlorambucil ( taken orally what?) which is relatively free of side-effects, in addition some specialist may combine chlorambucil with prednisolone (steroid), which has been known to be an effective combination.

Fludarabine is also a chemotherapy drug used in the treatment of chronic lymphocytic leukemia producing higher response rates than chlorambucil alone. It does have more side-effects than chlorambucil, particularly in bone marrow suppression. In rare instances, it may cause interstitial pneumonia (a diffuse lung infection), haemolytic anemia—a condition where the red blood cell precursors are prematurely destroyed. Occasionally it mayalso cause hair loss. (this is a run-on sentence)

Patients with high risk CLL (Rai stages III and IV) have an average survival rate of 3-4 years, and should be treated immediately at the time of diagnosis. There is a high probability of developing progressively advancing diseases, some causing death from leukemia. These patients benefit from symptomatictreatment with chlorambucil, fludarabine, and chemotherapy, or a combination of the three. Radiation therapy is occasionally used to relieve symptoms produced by enlarged lymph nodes or spleen.

Hairy cell leukemia is a sub-type of chronic lymphoid leukemia and represents about 2% of all cases of leukemia. Originally known as histiocytic leukemia, malignant reiculosis, or lymphoid myelofibrosis, this disease’s name is derived from the appearance of the cell under a microscope.

Most patients with hairy cell leukemia require treatment. Splenectomy was the initial choice and with the most benefit for patient—at least for a few months. Unfortunately most patients develop progressive diseases and require more aggressive therapy. The first line of therapy would be cladribine (2CDA) and pentostatin (DCF) which are purine analogs, both taken through IV. During the weeks following treatment, the patient’s immune system is severely weakened, but their bone marrow will usually begin to produce normal blood cells. Treatment often results in long-term remissions. There is an 85% success rate of patients to achieve complete response from either drug. If the cancer cells return, the treatment may be repeated and should result in remission. Remission rates vary from 1 to 20 years, with a median of being treatment-free in about 10 years.

If a patient is resistant to first-line therapy, then second-line therapy is pursued. Monoclonal antibodies are the most common treatment that destroys cancerous B cells—Rituximab being the most common taken by IV. Rituximab’s major side-effect is serum sickness, commonly known as an “allergic reaction”, especially from the first infusion. Other B cell-destroying monoclonal antibodies such as Alemtuzumab, Ibritumomab tiuxetan and I-131 Tositumomab may be considered for refractory cases.

Interfernon-alpha is another immune system hormone that is very helpful for most patients. In about 65% of patients the drug helps stabilize the disease. A majority of patients enjoy long periods of remission, but many relapse once the interferon therapy is discontinued. Interferon-alpha works by sensitizing the hairy cell to the killing effect of the immune system hormone TNF-alpha.