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Incidence and clinical features of langerhans cell histiocytosis in the UK and Ireland
  1. Jane A Salotti (jane.salotti{at}ncl.ac.uk)
  1. Newcastle University, United Kingdom
    1. Vasanta Nanduri (vasanta.nanduri{at}whht.nhs.uk)
    1. Watford General Hospital, United Kingdom
      1. Mark S Pearce (m.s.pearce{at}ncl.ac.uk)
      1. Newcastle University, United Kingdom
        1. Louise Parker (louise.parker{at}iwk.nshealth.ca)
        1. Canadian Cancer Society (Nova Scotia Division), Canada
          1. Richard M Lynn (richard.lynn{at}rcpch.ac.uk)
          1. Royal College of Paediatrics and Child Health, United Kingdom
            1. Kevin P Windebank (k.p.windebank{at}ncl.ac.uk)
            1. Newcastle University, United Kingdom

              Abstract

              Objectives: There are few published studies on epidemiology of Langerhans Cell Histiocytosis (LCH). We undertook a survey to ascertain all newly diagnosed cases aged 0-16 years in the UK and Republic of Ireland.

              Design: Three methods of ascertainment were used; the British Paediatric Surveillance Unit (BPSU) system, a survey by Newcastle University, and the Children’s Cancer and Leukaemia Group (CCLG) registry. Deaths data were obtained from the UK Office for National Statistics and the Central Statistics Office in Ireland. Clinicians who reported cases were sent a questionnaire to obtain demographic and clinical details.

              Results: Over the two-year period, 94 cases were identified. The age-standardised incidence rate of LCH in children aged 0-14 years was 4.1 per million per year. The sex ratio (M:F) was 1.5:1 and median age at diagnosis was 5.9 years. Single system disease (predominantly bony involvement) accounted for 73% cases and 27% had multi-system disease of whom 7% had involvement of “risk organs” (liver, lung, spleen, bone marrow). Three children died, two of whom were diagnosed post mortem.

              Conclusions: This is the first study of LCH to use an active surveillance method with additional sources of ascertainment. Our incidence is comparable with other national reports although it is likely to be an underestimate as each method may have missed some cases, either diagnosed or undiagnosed.

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