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Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland
Lookup NU author(s)
Dr Jane Salotti
Dr Mark Pearce
Professor Louise Parker
Dr Kevin Windebank
Salotti J, Nanduri V, Pearce MS, Parker L, Lynn RM, Windebank KP
Archives of Disease in Childhood
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There are few published studies on the 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.
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.
Over the 2-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
the median age at diagnosis was 5.9 years. Single system disease
(predominantly bony involvement) accounted for 73% of cases
and 27% had multisystem disease of whom 7% had involvement of
"risk organs" (liver, lung, spleen and bone marrow). Three children
died, two of whom were diagnosed after death.
This is the first study of LCH to use an active surveillance
method with additional sources of ascertainment. Our incidence
is comparable with those in 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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