Biology and treatment of small non-cleaved cell lymphoma

Small non-cleaved cell lymphoma occurs predominantly in the first two decades of life, and in this age group can be divided into two major types, both of which are associated with non-random chromosomal translocations. The endemic form occurs at high frequency in equatorial Africa, and the sporadic form occurs at low frequency throughout the world. Treatment consists primarily of chemotherapy; surgery has an important role in resectable abdominal disease, but radiation is of limited value. Prophylactic treatment of the central nervous system is recommended in nearly all patients. The most effective regimens result in cure in almost all patients who have limited disease, and in a high proportion of patients with extensive disease. Patients with bone marrow or central nervous system involvement have a poor prognosis with most regimens. Therapy for recurrent disease continues to be a challenge, and remains largely experimental.