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Prolymphocytic Leukemia Treatments

Prolymphocytic leukemia (PLL) is actually a rare form of chronic lymphocytic leukemia (CLL), a slowly progressive cancer of the lymphocytes (a type of white blood cell). PLL, a small-cell variant form of CLL, originates in immature white blood cells (prolymphocytes). The method of treatment for your PLL will depend on the type of prolymphocytes affected, T-cells or B-cells, as well as your age, health and the severity of the disease.

  1. T-Cell Prolymphocytic Leukemia

    • While T-cell prolymphocytic leukemia (T-PLL) can have a poor response to chemotherapy, it's still used as part of treatment. Fludarabine is by far the drug of choice, since it's often used with cancers affecting the blood, bone marrow and lymph nodes. However, your doctor may use pentostatin or an alkylating agent instead of, or in combination with fludarabine. Alkylating agents, a group of "anticancer" drugs, act on the rapidly-dividing cells characteristic of leukemia and other cancers. These drugs attach themselves to the DNA of the abnormal cells. This damages the DNA, impeding cell division and causing cell death. During chemotherapy treatment, many patients experience nausea, vomiting, fatigue, weakness and loss of appetite.

      If this treatment doesn't provide results, your doctor may introduce alemtuzumab into your treatment plan. This antibody activates your immune system to better kill cancerous cells. Previously, alemtuzumab was only used for B-PLL, but has now proven effective in treating T-PLL as well. An article in the Medical Science Monitor cites a greatly improved remission rate when alemtuzumab was used during the course of treatment.

      A favorable response to these treatments may be followed by a stem cell transplant (SCT). Stem cells are administered directly into your bloodstream, where they travel to the cavities of your bone marrow to generate new, healthy marrow. Since T-cells originate within bone marrow, SCT may serve to create healthy lymphocytes. Typically, SCT is given to patients who are young (under the age of 60) and in good health, as they have a better response to this form of treatment. Older patients are usually observed to determine whether or not another course of chemo and antibodies is necessary.

      T-PLL is an aggressive form of CLL, with a median survival rate of seven months. Early treatment improves the prognosis.

    B-Cell Prolymphocytic Leukemia

    • With B-cell prolymphocytic leukemia (B-PLL), as in other forms of CLL, treatment often begins with chemotherapy. As with T-PLL, doctors often turn to fludarabine. However, your doctor may decide to use it in conjunction with cladribine, a chemotherapy drug used for hairy-cell leukemia.

      If these medications don't prove effectual, your doctor will often introduce either alemtuzumab or rituximab into your treatment plan. Rituximab, similar to alemtuzumab, is an antibody that activates your immune system to better kill cancerous cells. During treatment, you may experience fevers, chills and run a greater risk of infections.

      After chemotherapy, SCT is necessary to create new marrow free of the disease, which may serve to create healthy lymphocytes. Older patients (over the age of 60) are usually observed to determine whether or not another course of chemo and antibodies is necessary.

      If your spleen becomes engorged during treatment, it may need to be removed through a splenectomy. Or radiation may be administered to return the organ to its normal health. Enlargement of the spleen is often an indication that leukemia cells are also growing in this organ, so targeted treatment is often necessary.

      B-PLL is more treatable than T-PLL so the prognosis is often better. The median survival rate is three years.

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