Adult central nervous system leukemia: incidence and clinicopathologic features

Two hundred and seventy-two adults diagnosed between 1949 and 1971 as having acute leukemia were evaluated. Two hundred and fifty-seven patients had died and autopsies were obtained in 202 cases. Central nervous system (CNS) leukemia was demonstrated in 22 of 93 autopsies with acute nonlymphocytic leukemia (ANLL) during the period 1949 through 1966 and 8 of 47 during the period 1967 through 1971. Nine of 45 autopsies on acute lymphoblastic leukemia (ALL) patients diagnosed during 1949 through 1966 had CNS involvement, compared to 7 of 17 during 1967 through 1971. The median time from diagnosis of acute leukemia to CNS manifestations was two months for ANLL and six months for ALL. Headache, papilledema, and cranial nerve palsy were the common findings with meningeal leukemia. Early CNS involvement was observed in patients with high initial leukocyte/blast counts, low platelet counts, and early lymphadenopathy and hepatosplenomegaly. Ten of 13 patients treated between 1967 and 1971 with cranial irradiation and intrathecal chemotherapy responded; however, the duration of remission in ALL was short-lived with subsequent relapses at various intervals. In contrast, CNS recurrence in ANLL was rare. The value of CNS prophylactic and maintenance therapy is discussed.