Purpose or Case Report: Langerhans Cell Histiocytosis (LCH) is a rare histiocytic disorder, classically of the paediatric population but which may also affect adults. LCH affects up to 4.1 per million children each year in the UK and Ireland. Our goal in this presentation is to illustrate typical and atypical imaging features of LCH and discuss how these are relevant to further management.
Methods & Materials: Our hospital is the major tertiary referral centre for the evaluation and management of LCH in Ireland. Since 2005, sixty cases of LCH have presented or have been referred to OLCHC for further management. We reviewed all the imaging the patients had over the duration. We determined the age at presentation, type and number of body systems involved, biopsy proven or not etc
Results: The average age of presentation was 4.9yrs old, with a range from 1 month to 14.5 years old, and a very slight male predilection. The majority of cases (80%, n=48) were unisystem, with approximately half of these cases (25%, n=15) were multifocal bone. The vast (90%, n=54)of all cases were biopsy proven. One fifth of all patients(n=12) presented with multisystem disease, with a younger average age (1.4 years old) than the overall cohort.
Conclusions: LCH is a rare histiocytic multisystem disorder predominantly affecting the paediatric population. Its typical and atypical features, with particular emphasis on the most clinically relevant radiological findings, are discussed.