title: Primary immunodeficiencies in the Netherlands: national patient data demonstrate the increased risk of malignancy.
abstract: OBJECTIVE
  To analyze the data of the national registry of all Dutch primary immune deficiency (PID) patients, according to the European Society for Immunodeficiencies (ESID) definitions.
  
  
  RESULTS
  In the Netherlands, 745 patients had been registered between 2009 and 2012. An overall prevalence of 4.0 per 100,000 inhabitants was calculated. The most prevalent PID was 'predominantly antibody disorder (PAD)' (60.4%). In total, 118 transplantations were reported, mostly hematopoietic stem cell transplantations (HSCT). Almost 10% of the PID patients suffered from a malignancy, in particular 'lymphoma' and 'skin cancer'. Compared to the general Dutch population, the relative risk of developing any malignancy was 2.3-fold increased, with a >10-fold increase for some solid tumors (thymus, endocrine organs) and hematological disease (lymphoma, leukemia), varying per disease category.
  
  
  CONCLUSIONS
  The incidence rate and characteristics of PID in the Netherlands are similar to those in other European countries. Compared to the general population, PID patients carry an increased risk to develop a malignancy.
authors.affiliation: Emma Children's Hospital, Academic Medical Center (AMC), Dept. of Pediatric Hematology, Immunology and Infectious Diseases, University of Amsterdam (UvA), Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. Electronic address: bmjonkman@gmail.com.
authors.forename: B M
authors.initials: BM
authors.lastname: Jonkman-Berk
authors.affiliation: Emma Children's Hospital, Academic Medical Center (AMC), Dept. of Pediatric Hematology, Immunology and Infectious Diseases, University of Amsterdam (UvA), Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. Electronic address: j.m.vandenberg@amc.uva.nl.
authors.forename: J M
authors.initials: JM
authors.lastname: van den Berg
authors.affiliation: Academic Medical Center (AMC), Dept. of Internal Medicine, UvA, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. Electronic address: r.j.tenberge@amc.uva.nl.
authors.forename: I J M
authors.initials: IJ
authors.lastname: Ten Berge
authors.affiliation: Leiden University Medical Center (LUMC), Dept. of Pediatrics, PO box 9600, 2300 RC Leiden, The Netherlands. Electronic address: R.G.M.Bredius@lumc.nl.
authors.forename: R G M
authors.initials: RG
authors.lastname: Bredius
authors.affiliation: Erasmus Medical Center (ErasmusMC), Sophia Children's Hospital, Dept. of Pediatric Infectious Diseases and Immunology, PO box 2030, 3000 CB Rotterdam, The Netherlands. Electronic address: g.driessen@erasmusmc.nl.
authors.forename: G J
authors.initials: GJ
authors.lastname: Driessen
authors.affiliation: EMC, Dept. of Clinical Immunology, PO box 2040, 3000 CA Rotterdam, The Netherlands. Electronic address: v.dalm@erasmusmc.nl.
authors.forename: V A S H
authors.initials: VA
authors.lastname: Dalm
authors.affiliation: LUMC, Dept. of Infectious Diseases, PO box 9600, 2300 RC Leiden, The Netherlands. Electronic address: J.T.van_Dissel@lumc.nl.
authors.forename: J T
authors.initials: JT
authors.lastname: van Dissel
authors.affiliation: Radboud University Medical Center, Dept. of Internal Medicine, PO box 9101, 6500 HB Nijmegen, The Netherlands; Radboudumc Institute of Molecular Life Sciences (RIMLS), PO box 9101, 6500 HB Nijmegen, The Netherlands. Electronic address: Marcel.vanDeuren@radboudumc.nl.
authors.forename: M
authors.initials: M
authors.lastname: van Deuren
authors.affiliation: University Medical Center Utrecht, Dept. of Infectious Diseases, PO box 85500, 3509 GA Utrecht, The Netherlands. Electronic address: P.Ellerbroek@umcutrecht.nl.
authors.forename: P M
authors.initials: PM
authors.lastname: Ellerbroek
authors.affiliation: Radboud University Medical Center, Dept. of Pediatric Infectious Diseases and Immunology, PO Box 9101, 6500 HB Nijmegen, The Netherlands; Radboudumc Institute of Molecular Life Sciences (RIMLS), PO box 9101, 6500 HB Nijmegen, The Netherlands. Electronic address: Michiel.vanderFlier@radboudumc.nl.
authors.forename: M
authors.initials: M
authors.lastname: van der Flier
authors.affiliation: EMC, Dept. of Clinical Immunology, PO box 2040, 3000 CA Rotterdam, The Netherlands. Electronic address: p.m.vanhagen@erasmusmc.nl.
authors.forename: P M
authors.initials: PM
authors.lastname: van Hagen
authors.affiliation: University Medical Center Utrecht, Wilhelmina Children's Hospital, Dept. of Pediatric Immunology and Infectious Diseases, PO box 85090, 3508 AB Utrecht, The Netherlands. Electronic address: J.vanMontfrans@umcutrecht.nl.
authors.forename: J M
authors.initials: JM
authors.lastname: van Montfrans
authors.affiliation: University Medical Center Groningen (UMCG), Dept. of Rheumatology and Clinical Immunology, PO box 30.001, 9700 RB Groningen, The Netherlands. Electronic address: a.rutgers@umcg.nl.
authors.forename: A
authors.initials: A
authors.lastname: Rutgers
authors.affiliation: University Medical Center Groningen/Beatrix Children's Hospital, Dept. of Pediatric Immunology and Infectious Diseases, PO box 30.001, 9700 RB Groningen, The Netherlands. Electronic address: e.h.scholvinck@umcg.nl.
authors.forename: E H
authors.initials: EH
authors.lastname: Schölvinck
authors.affiliation: Jeroen Bosch Hospital, Dept. of Pediatrics, PO box 90153, 5200 ME 's-Hertogenbosch, The Netherlands. Electronic address: E.d.Vries@jbz.nl.
authors.forename: E
authors.initials: E
authors.lastname: de Vries
authors.affiliation: Sanquin Blood Supply Foundation, Medical Department, PO box 9190, 1006 AD Amsterdam, The Netherlands. Electronic address: R.vanbeem@sanquin.nl.
authors.forename: R T
authors.initials: RT
authors.lastname: van Beem
authors.affiliation: Emma Children's Hospital, Academic Medical Center (AMC), Dept. of Pediatric Hematology, Immunology and Infectious Diseases, University of Amsterdam (UvA), Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. Electronic address: t.w.kuijpers@amc.nl.
authors.forename: T W
authors.initials: TW
authors.lastname: Kuijpers
mesh_terms: D017677:Age Distribution; D005060:Europe / Q000453:epidemiology; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology*; D008297:Male; D009369:Neoplasms / Q000453:epidemiology*; D009426:Netherlands / Q000453:epidemiology; D015995:Prevalence; D012042:Registries / Q000706:statistics & numerical data; D012306:Risk; D017678:Sex Distribution
keywords: Infection; Malignancy; Primary immunodeficiencies; Transplantation