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Although chronic lymphocytic leukemia CLL remains incurable, over the past decade there have been major advances in understanding the pathophysiology of CLL and in the treatment of this disease. This has led to greatly increased response rates and durations of response but not yet improved survival. Questions remain, including, what is the optimal first-line treatment and its timing and is there any role of maintenance therapy or stem cell transplantation in this disease?
CLL is a disease of the elderly and not all patients are eligible for aggressive up-front chemoimmunotherapy regimens, so what is the optimal treatment approach for more frail elderly patients? It is highly likely that our treatment approaches will continue to evolve as the results of ongoing clinical trials are released and that further improvements in the outcome of this disease will result from identification of therapies that target the underlying pathophysiology of CLL.
It is estimated that 15 people men and women will be diagnosed with chronic lymphocytic leukemia CLL in the United States in The age-adjusted incidence rate is 4.
The disease is twice as common in males as females, more common in white than black Americans, rarer in Hispanics and Native Americans, and much rarer in the Asian population. Among the strongest risk factors for the development of CLL is a family history of this or other lymphoid malignancies. Several familial clusters of CLL have been reported, 2 and there is genetic anticipation, the process whereby the median age at onset in a child of a multigeneration family with malignancy is younger than that of the parent generations.
In a report from the National Cancer Institute Familial Registry, the mean age at diagnosis among familial cases was 58 years, 14 years younger than that of sporadic cases. Apart from the difference in age at presentation, familial CLL is essentially indistinguishable from sporadic CLL, favoring a genetic basis to disease development in general rather than a simple environmental etiology.