High-grade, B-lineage lymphoproliferative disorders can have a leukemic (acute lymphocytic leukemia [ALL] French-American-British [FAB] stage L-3), a lymphomatous (Burkitt's or small noncleaved [SNC]), or often a mixed clinical presentation in adults. Manifestations of these diseases in both adults and children include a propensity for abdominal and CNS involvement and a high incidence of metabolic abnormalities due to rapid cell growth and turnover, which are acutely exacerbated by treatment. Numerous studies show that FAB L-3, Burkitt's lymphoma (BL), and SNC are among the most curable of the multiple leukemia/lymphoma subtypes if treated appropriately. At least 50% of adults with these disorders are cured with the use of short-term intensive chemotherapeutic regimens, with treatment failure a consequence of both drug resistance and an inability of older adults to tolerate the side effects of therapy. Published studies have included very heterogeneous groups of patients, some characterized only by morphology, and others studied with cytogenetics but not necessarily having the classic mutations involving c-myc. Future studies should focus on better characterizing the patient populations to help identify the patients most likely to benefit from these aggressive regimens, as well as those who might require high-dose therapy with stem-cell transplantation.
Copyright 2001 by W.B. Saunders Company.