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Studies have shown that patients with CLL are also at increased risk for developing a second type of cancer. It has been estimated that up to 11% of CLL patients develop a second cancer that may include:

• Myelodysplastic syndromes (MDS)
• Acute myelogenous leukemia (AML)
• Malignant melanoma
• Brain cancer
• Bladder cancer
• Stomach cancer
• Kaposi's sarcoma

It has been suggested that impairment of the immune system in patients with CLL may be a primary factor in their susceptibility to developing secondary cancers. Lacking a normal, healthy immune system, some CLL patients are more vulnerable to developing cancer in other areas of the body. It is, therefore, important for all CLL patients to be monitored carefully by their health care provider to the occurrence of secondary cancers. This recommendation includes periodic follow-up with a dermatologist for a skin examination to detect any signs of skin cancer. CLL patients are also urged to avoid excessive exposure to sunlight which can cause skin cancer and to avoid exposure to tobacco smoke (including secondhand smoke) that has been linked as a risk factor for a variety of cancers.

Disease Transformation

Approximately 5% to 8% of patients with B-cell chronic lymphocytic leukemia develop a high-grade (aggressive) form of non-Hodgkin's lymphoma called a large B-cell lymphoma. This transformation of CLL to non-Hodgkin's lymphoma is known as Richter's syndrome or Richter's transformation. This disease transformation is often accompanied by symptoms such as fever, night sweats, weight loss, and enlargement of the lymph nodes, spleen, and liver. Richter's syndrome is usually treated with aggressive combination chemotherapy with regimens such as CHOP (cyclophosphamide plus doxorubicin plus vincristine plus prednisone). Unfortunately, despite aggressive treatment, transformation of CLL to Richter's syndrome usually carries a poor prognosis with median survival of less than 6 months after disease transformation.

In rare cases, transformation of CLL to other types of cancers has also been observed including:

• Prolymphocytic leukemia (PLL)
• Acute lymphocytic leukemia (ALL)
• Hodgkin disease
• Multiple myeloma