title: Central nervous system involvement at presentation in childhood acute lymphoblastic leukemia: management experience and lessons.
abstract: This study was designed to analyze the spectrum of central nervous system (CNS) disease at diagnosis, traumatic lumbar puncture (TLP), role of cranial irradiation, prognostic parameters, and survival outcome in patients with CNS involvement amongst 747 patients with acute lymphoblastic leukemia managed at our center. Twenty-five and six patients had CNS disease and TLP, respectively. Patients with CNS involvement had significantly higher mean presenting leukocyte count (p = 0.021) and incidence of hyperleukocytosis (p = 0.01) compared to those without it. The outcome was poor with three patients in continuous complete-remission, nine relapsers, eight deaths, and eight therapy defaulters. Three patients did not opt for therapy. CNS involvement was significantly associated with inferior survival by log-rank (p = 0.03) analysis but not by Cox-multivariate (p = 0.145) analysis. CNS involvement is a high-risk indicator. Poor outcome in our cohort indicates the need for the revaluation of our treatment protocols with the inclusion of risk-stratified systemic therapy, categorization of CNS involvement into CNS1/CNS2/CNS3, and appropriate use of intrathecal therapy.
authors.affiliation: Division of Pediatric Hematology-Oncology, Advanced Pediatric Center, PGIMER, Chandigarh, India. rammarwaha1@rediffmail.com
authors.forename: Ram K
authors.initials: RK
authors.lastname: Marwaha
authors.forename: Ketan P
authors.initials: KP
authors.lastname: Kulkarni
authors.forename: Deepak
authors.initials: D
authors.lastname: Bansal
authors.forename: Amita
authors.initials: A
authors.lastname: Trehan
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002490:Central Nervous System / Q000187:drug effects* / Q000473:pathology; D002493:Central Nervous System Diseases / Q000150:complications / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D012008:Recurrence; D012074:Remission Induction; D016019:Survival Analysis; D016896:Treatment Outcome