Management of malignant lymphoproliferative disorders of the nervous system

The frequency of involvement of the nervous system (NS) among 266 patients seen at the University College Hospital (UCH), Ibadan, with a diagnosis of malignant lymphoproliferative diseases (MLPD) was determined. Only one of these patients who had a solitary spinal-cord involvement by Hodgkin's disease (HD) was considered to have primary lymphoma of NS. In all other cases, NS was only secondarily involved by MLPD. This was most commonly observed in association with Burkitt's lymphoma and occurred in 49.3 and 67% of such patients who were previously untreated or previously treated, respectively. Nervous system (NS) involvement at presentation was much less commonly observed in non-Hodgkin's (NHL), HD and acute lymphoblastic leukaemia (ALL) at the rate of 6.7, 4.4 and 0% respectively of previously untreated patients. The NS was the site of relapse in 63.6, 43, 12.5 and 0% in BL, ALL, NHL and HD, respectively. Intra-cranial NS disease was identified as a poor prognostic feature as this was associated with a reduction of the probability of prolonged survival from 48% to less than 20% among patients with intra-cranial and/or extra-cranial disease in BL. Management of MLPD with high rate of NS involvement should include effective delivery of chemotherapy into CFS, both by direct injection intrathecally and through filtration across the blood-brain barrier following high-dose intravenous injection of agents like methotrexate or cytosine arabinoside.