title: High relapse rate in children with non-advanced nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL or nodular paragranuloma) treated with chemotherapy only.
abstract: Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) is a rare variant of Hodgkin's lymphoma (HL) in children. Since specific immunohistochemical staining has become available, NLPHL can be separated from classical Hodgkin's lymphoma (cHL) more accurately. Scarce information is available about pediatric NLPHL treated with chemotherapy only. Therefore, clinical characteristics, treatment, response and outcome of seven pediatric NLPHL patients, median age 9.2 years (range 7.5 - 14.2 years), diagnosed between 1986 - 2003 among 58 HL patients, uniformly treated in a single center with chemotherapy only, were evaluated. The median follow-up time was 4.2 years (range 2.1 - 10.2 years). NLPHL patients were stage I (n = 5), II (n = 2), whereas cHL, median age 11.4 years (range 3.3 - 15.9 years), were stage I (n = 8), II (n = 17), III (n = 9), IV (n = 1). Upfront treatment of NLPHL patients consisted of six courses of epirubicin, bleomycin, vinblastine and dacarbazine (EBVD) without radiotherapy, whereas cHL patients received six courses of EBVD (n = 14) or 4 - 6 courses of EBVD/MOPP (mitoxin, oncovin, procarbazine, prednison; n = 21). Chemotherapy was used as primary treatment thereby aiming to avoid radiotherapy with potential serious side effects to growing jaws and thyroid. All seven patients reached complete remission (CR). Four patients relapsed, of which three locally. These three were salvaged with second-line chemotherapy without radiation therapy (RT) and are in second CR. One patient relapsing with stage III disease was salvaged by EBVD/MOPP followed by autologous BMT and is also in second CR for 36 months. The event-free survival (EFS) is 43% and overall survival (OS) 100%. This study shows that apart from histology, immunohistochemistry (ICH) is required for diagnosing NLPHL. Moreover, it illustrates that although cure of pediatric NLPHL is feasible with chemotherapy only, high dosages of cytotoxic drugs are necessary as salvage treatment in a relatively high proportion of patients after relapse.
authors.affiliation: Department of Pediatric Oncology/Hematology, Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Martine
authors.initials: M
authors.lastname: van Grotel
authors.forename: King H
authors.initials: KH
authors.lastname: Lam
authors.forename: Rogier
authors.initials: R
authors.lastname: de Man
authors.forename: Auke
authors.initials: A
authors.lastname: Beishuizen
authors.forename: Rob
authors.initials: R
authors.lastname: Pieters
authors.forename: Marry M
authors.initials: MM
authors.lastname: van den Heuvel-Eibrink
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001761:Bleomycin / Q000008:administration & dosage; D002648:Child; D003606:Dacarbazine / Q000008:administration & dosage; D015251:Epirubicin / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006689:Hodgkin Disease / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality; D006801:Humans; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D012008:Recurrence; D012074:Remission Induction / Q000379:methods; D012189:Retrospective Studies; D016879:Salvage Therapy / Q000379:methods; D016019:Survival Analysis; D016896:Treatment Outcome; D014747:Vinblastine / Q000008:administration & dosage
chemical_list: D001761:Bleomycin; D015251:Epirubicin; D014747:Vinblastine; D003606:Dacarbazine