ABSTRACT
I. INTRODUCTION Chronic lymphocytic leukemia (CLL) is the most common of all leukemias in Western countries. The National Cancer Institute’s Surveillance Epidemiology and End Results (SEER) program data for the 15-year period from 1973 to 1987 indicates that CLL ac counts for 31% of all leukemias in the United States (1). There is a higher incidence rate among Caucasians compared with the African-American population (1). It is estimated that more than 7000 persons are newly diagnosed with CLL in the United States every year. CLL is considered to be mainly a disease of the elderly (median age, 70 years), but it is not unusual today to make this diagnosis even in younger age groups (i.e., 30-39 years). The incidence increases rapidly after age 55. There are no clearly discernible occu pational or environmental risk factors that predispose to CLL. Although there have been reports of excess risk of CLL among farmers (2-4) with benzene and heavy solvent expo sure (2,3,5) and among rubber-manufacturing workers (6,7), these associations have not been proven to be causal (8). CLL and other lymphoid, hematological, and solid tumors occur with higher than expected frequency among first-degree relatives, but there is no available proof of genetic transmission of CLL. It is a generally held belief that CLL is an indolent disease associated with a prolonged chronic course and that the eventual cause of death may be unrelated to CLL for less than 30% of all cases. The natural history is heterogeneous in most patients. Some patients die rapidly, within 2 to 3 years of diagnosis and from a complication or cause directly related to CLL. Many patients live 5 to 10 years with an initial course that is relatively benign but almost always followed by a terminal phase lasting 1 to 2 years, during which there is considerable morbidity, both from the
disease itself and from complications of therapy. The most frequent causes of death are severe systemic infections (e.g., pneumonia, septicemia), bleeding, and inanition with cachexia. In a small minority of patients, a diffuse large-cell immunoblastic lymphoma supervenes terminally (Reichter’s transformation) associated with a rapidly progressive course, refractoriness to all chemotherapy, and death within 6 months. Patients with CLL are considered to be at higher risk of other cancers developing; usually lung or gastrointes tinal cancers occur with considerably greater frequency in CLL patients than among the general population, but these do not result in higher mortality. Advances in the treatment of the hematological malignancies have led to longer survival of many patients and cure of more. Interest in how these patients cope with the newer and more technological treat ment and their quality of life has increased. The psychological issues are outlined in the following, as they relate to diagnosis, treatment, and the adaptation of survivors.
