title: Immunodeficiency and Hodgkin's disease: treatment and outcome in the DAL HD78-90 and GPOH HD95 studies.
abstract: BACKGROUND
  Excellent treatment results have been obtained for children with Hodgkin's disease (HD). Children with immunodeficiencies who present with HD do not have such a favourable prognosis.
  
  
  METHODS
  A systematic literature search using MEDLINE and a search for immunodeficiencies in the database of the trials DAL HD78-HD90 and GPOH HD95 (n = 2263) were carried out. Age, sex, type of immunodeficiency, disease stage, treatment and outcome of all HD cases with known immunodeficiency were recorded.
  
  
  RESULTS
  28 published cases and 13 children in the DAL/GPOH trials were identified. 19/28 and 6/13 patients have immunodeficiencies with increased DNA breakage (24/25 ataxia teleangiectasia, 1/25 Nijmegen breakage syndrome) who present largely with stage III - IV HD. Among the published cases with increased DNA breakage there is only one child who is surviving 16 months after diagnosis, while there are 6/9 survivors in the group of immunodeficiencies without increased DNA breakage. Similarly, only 1/6 children survives in the group of children reported to the DAL/GPOH trials suffering from HD and immunodeficiency with increased DNA breakage, while the outcome in children suffering from immunodeficiency without increased DNA breakage is much better with 5/7 survivors.
  
  
  CONCLUSIONS
  The literature review and data analysis of the DAL/GPOH studies show that treatment outcome is almost invariably fatal in children with HD and immunodeficiency with increased DNA breakage. Thus we propose to treat children with or without increased DNA breakage differently to improve the outcome of Hodgkin's disease in the subgroup of children with immunodeficiency.
authors.affiliation: Klinik für Kinder-Onkologie, -Hämatologie und -Immunologie, Universitätskinderklinik Düsseldorf, Germany. niehues@uni-duesseldorf.de
authors.forename: T
authors.initials: T
authors.lastname: Niehues
authors.forename: G
authors.initials: G
authors.lastname: Schellong
authors.forename: W
authors.initials: W
authors.lastname: Dörffel
authors.forename: P
authors.initials: P
authors.lastname: Bucsky
authors.forename: G
authors.initials: G
authors.lastname: Mann
authors.forename: D
authors.initials: D
authors.lastname: Körholz
authors.forename: U
authors.initials: U
authors.lastname: Göbel
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001260:Ataxia Telangiectasia / Q000150:complications / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D019457:Chromosome Breakage; D002986:Clinical Trials as Topic; D003131:Combined Modality Therapy; D005260:Female; D006689:Hodgkin Disease / Q000150:complications* / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy / Q000628:therapy*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000401:mortality; D008297:Male; D011379:Prognosis; D011879:Radiotherapy Dosage; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome