Meningeal involvement in patients with acute nonlymphocytic leukemia incidence, management, and predictive factors

Abstract

Between 1977 and 1983, symptomatic meningeal leukemia was diagnosed in 9 of 93 adult patients with acute nonlymphocytic leukemia (10%). All cases occurred after complete remission had been achieved (46 patients), either as the only site of relapse (3 patients), or together with a first bone marrow relapse (3 patients), or after a bone marrow relapse (3 patients). Extramedullary involvement at initial diagnosis was the only independent predictive factor (P = 0.005), the number of patients with initial hyperleukocytosis being too small (three) for evaluation. Remission of the meningeal leukemia was obtained after treatment in four of eight patients. The presence of meningeal leukemia and the response to therapy had no influence on survival. However, the morbidity and therapy related toxicity of meningeal leukemia were impressive. Some recommendations for prophylactic treatment are suggested.

Leukemia and the nervous system

“…The clinical presentation of LM in patients with leukemia is similar to that seen in patients with LM from solid tumors [19,22,23,31]. However, patients with hematologic malignancies present with a higher frequency of cranial nerve signs (eg, trigeminal mental or optic neuropathies) as initial manifestations of neoplastic meningitis [20].…”

“…Late-delayed radiation complication (occurring years after radiotherapy) has two major forms: mineralizing ateriopathy and a necrotizing leukoencephalopathy. The former is reflected as dystrophic calcification in small blood vessels and is commonly seen in the basal ganglia, dentate nuclei, thalami, and subcortical white [18][19][20][21][22][23]. The presence or absence of LM always needs to be ascertained, and if LM is diagnosed, prognosis is profoundly affected.…”

“…This is also true for Burkitt's lymphoma and lymphoblastic lymphoma (2% to 3% of all adult nonHodgkin's lymphoma), two subtypes of what is now considered ALL [6]. Although AML infrequently results in LM, an unusual subtype, patients with acute myelomonocytic leukemia (AMML) are at high risk (estimated at 20%) for the development of LM [5,19]. Before CNS prophylaxis was practiced, 70% of autopsied patients with ALL had postmortem evidence of LM.…”

“…However, patients with hematologic malignancies present with a higher frequency of cranial nerve signs (eg, trigeminal mental or optic neuropathies) as initial manifestations of neoplastic meningitis [20]. LM is pleomorphic in its clinical presentation because it affects all levels of the CNS [19,20,22,23,31]. In general, three domains of neurologic disturbance are affected by LM: 1) the cerebral hemispheres; 2) the cranial nerves; and 3) the spinal cord and roots.…”

“…The single most useful laboratory test in diagnosing LM is an examination of the CSF, which is usually obtained by lumbar puncture [19,20,22,23,31,32]. In nearly all patients with LM, the CSF is abnormal regardless of the results of CSF cytology.…”