Introduction: Langerhans cell histiocytosis (LCH) is a rare condition primarily affecting children. It is characterised by clonal proliferation of Langerhans cells. Disease severity is dependent on the type and number of organs involved in addition to focality. Disease aetiology remains unclear. We aimed to record the incidence and characteristics of West of Scotland LCH patients with a focus on predictive factors for endocrine dysfunction.
Method: Consecutive diagnoses of LCH, at The Royal Hospital for Sick Children Glasgow, between January 1998 and December 2012 were identified. Patient notes were used to collect information on age, sex, postcode (Scottish index of deprivation-SIMD: one-most deprived, six-least deprived), ethnicity, systems involved, signs and symptoms of presentation, disease progression, treatment and current status.
Results: 23 patients were identified. Median age of diagnosis was 2.7 years (range: 1 month15 years). 52% (n=12) were female. Over half of patients 57% (n=13) have SIMD scores of 1/2. 82% of patients were Caucasian, 13% South Asian and 4% mixed origin. The head was the most common site of presentation. There was 1 mortality in the cohort. Common symptoms at presentation included skin rashes, lumps and musculoskeletal pain. All patients with high risk organ involvement (spleen, liver, hematopoietic system or lung) were girls. Endocrine dysfunction, with diabetes insipidus being most common, was seen in 35% (n=8). 62.5% (n=5) were diagnosed with diabetes insipidus alone. Patients with endocrine dysfunctions had a longer symptom interval compared to those without endocrine dysfunction; with 62.5% (n=5) diagnosed after 4 months compared to 13.3% (n=2) without endocrine dysfunction (P=0.052).
Conclusion: Females are more likely to develop high risk organ disease. Deprivation is associated with an increased incidence of LCH, which has not been previously reported. A third of paediatric LCH patients develop endocrine dysfunction and these patients have a longer symptom interval.