title: Childhood lymphomatoid granulomatosis: a report of 2 cases and review of the literature.
abstract: Lymphomatoid granulomatosis (LG) is a B-cell type lymphoproliferative disease. It mainly affects the lungs but may have extrapulmonary manifestations, especially in the central nervous system. The purpose of this study was to review the pediatric cases in the literature and add 2 new cases to the existing literature. A review of the literature was performed on children (0 to 18 years of age at diagnosis) with pathologically proven LG. We found 47 case reports, which, together with 2 new cases, were systematically analyzed. The median age was 12 years. The main symptoms were general, pulmonary, and neurological. Approximately one third of the patients were immunocompromised. High mortality rate was observed. Pediatric LG is a rare disease, which appears to be more frequently seen in immunocompromised patients, especially patients with leukemia. The disease has a high mortality rate; therefore, aggressive therapy according to a high-grade B-cell lymphoma protocol is justified.
authors.affiliation: *Division of Pediatric Oncology/Hematology Departments of †Child Neurology §Pathology, VU University Medical Center, Amsterdam ‡Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Zwanique C A
authors.initials: ZC
authors.lastname: Tacke
authors.forename: M Judith
authors.initials: MJ
authors.lastname: Eikelenboom
authors.forename: R Jeroen
authors.initials: RJ
authors.lastname: Vermeulen
authors.forename: Marjo S
authors.initials: MS
authors.lastname: van der Knaap
authors.forename: Anne M
authors.initials: AM
authors.lastname: Euser
authors.forename: Paul
authors.initials: P
authors.lastname: van der Valk
authors.forename: Gertjan J L
authors.initials: GJ
authors.lastname: Kaspers
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016867:Immunocompromised Host; D007223:Infant; D007231:Infant, Newborn; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis* / Q000188:drug therapy*; D008230:Lymphomatoid Granulomatosis / Q000175:diagnosis* / Q000188:drug therapy*; D008297:Male
chemical_list: D000970:Antineoplastic Agents