Lymphocytic-plasmacytic lymphomas

Abstract

Lymphocytic lymphoma is frequently associated with chronic lymphocytic leukaemia and is regarded as the tissue phase of this disease in the Kiel classification (Lennert, 1981). Patients with chronic lymphocytic leukaemia frequently have some degree of generalized lymphadeno-pathy although there is rarely any clinical indication for biopsy of these nodes. Lymph node biopsy is more likely to be performed in those patients with lymphocytic lymphoma who present with substantial localized or generalized lymphadenopathy. Such patients may have blood and bone marrow disease at the time of biopsy or develop this after a variable period (Galton, 1964), although a number of patients have been recorded who have been followed for many years after the diagnosis of lymphocytic lymphoma and who have not developed leukaemia (Pangalis et al., 1977). A small proportion of patients with chronic lymphocytic leukaemia develop an aggressive lymphoma with pleomorphic cytology that may cause nodal or extranodal tumour masses (Richter’s syndrome) (Richter, 1928). There is increasing evidence from careful immunohistological studies that the pleomorphic lymphoma that characterizes Richter’s syndrome is of the same clonal origin as the small lymphocytes of the original lymphocytic lymphoma (Delsol et al., 1981; Harousseau et al., 1981). Lymphocytic lymphoma occurs most frequently in the sixth and seventh decades of life, is rare in young adults and is usually generalized at the time of diagnosis.