title: Clinical features and treatment results in children with anaplastic large cell lymphoma.
abstract: Anaplastic large cell lymphoma (ALCL) tends to have frequent relapse and good response to salvage chemotherapy. The frequency of ALCL among 1486 Non-Hodgkin's lymphoma (NHL) cases followed-up since 1972 was 1.5%, however, the percentage was 9.3% in cases diagnosed after 2000. Event-free survival (EFS) and overall survival (OS) rates for 23 children were 32.2% and 72.8% at 3 years, respectively. Disseminated diseases, no response to first line treatment, anaplastic lymphoma kinase (ALK) negativity were found as significant predictors on survival of ALCL. The proper diagnosis and early referral is essential in these children for a better survival rate. The children with ALK negative status should be monitored carefully because of the poor prognostic factors, and treated differently. The survival rates in this study are need of further improvement since the survival rates with current protocols are achievable at a level more than 80%. This is mainly related with late referral of those children with advanced disease.
authors.affiliation: Division of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara Turkey. tezerkutluk@gmail.com.
authors.forename: Erman
authors.initials: E
authors.lastname: Ataş
authors.forename: M Tezer
authors.initials: MT
authors.lastname: Kutluk
authors.forename: Canan
authors.initials: C
authors.lastname: Akyüz
authors.forename: Gülsev
authors.initials: G
authors.lastname: Kale
authors.forename: Ali
authors.initials: A
authors.lastname: Varan
authors.forename: Bilgehan
authors.initials: B
authors.lastname: Yalçın
authors.forename: Burça
authors.initials: B
authors.lastname: Aydın
authors.forename: Münevver
authors.initials: M
authors.lastname: Büyükpamukçu
mesh_terms: D000293:Adolescent; D000077548:Anaplastic Lymphoma Kinase; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017728:Lymphoma, Large-Cell, Anaplastic / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D009364:Neoplasm Recurrence, Local; D020794:Receptor Protein-Tyrosine Kinases; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases