Central nervous system involvement in non-Hodgkin's lymphoma

Fifty-eight Hong Kong Chinese patients with CNS lymphoma were reviewed (primary seven, secondary 51). The incidence of secondary CNS lymphoma in patients with non-Hodgkin's lymphoma was estimated to be 9.4%. The Working Formulation separated subtypes which had a special propensity to involve the CNS. Significant proportions of our patients with secondary CNS lymphoma had other features which were known to be associated with a high risk of CNS disease including stage IV ( 48 51 , 91.4%), bone marrow ( 26 51 , 50.9%), peripheral blood (7.51, 13.7%), nasal ( 7 51 , 13.7%), orbital ( 3 51 , 5.9%), testicular ( 2 51 , 3.9%) and bulky retroperitoneal ( 6 51 , 11.8%) disease. 82% of patients with secondary CNS lymphoma had concurrent systemic disease and a further 12% had systemic relapse shortly afterward. CNS lymphoma is associated with poor prognosis and only 29% and 14% of the patients with primary and secondary CNS lymphoma respectively survived beyond 1 year. Patients responding to therapy had significantly better survival. 69.9% of the deaths were related to progressive systemic disease.