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Published on: June 22, 2016
Endocrine features of Langerhans cell histiocytosis in paediatric patients: A 30-year review
Ashley Alexander1, Margaret Zacharin1,2,3
1Department of Endocrinology and Diabetes, Royal Children's Hospital, Melbourne, Victoria, Australia.
Abstract:
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder characterised as an inflammatory myeloid neoplasia. Endocrine manifestations of LCH, particularly central diabetes insipidus (CDI), have been described from the 1940s, through case studies and small cohort analyses. There are limited Australian paediatric data described in recent literature.
Aim:
To document the incidence of endocrine features in paediatric patients with LCH, treated at a tertiary paediatric centre in Victoria, Australia.
Methods:
Retrospective chart review of electronic medical records and oncology database of patients with LCH managed at a tertiary paediatric centre. Patients were excluded if a biopsy did not suggest LCH or if records were incomplete.
Results:
One hundred seventy-one patients were identified and 141 records of patients diagnosed with LCH over the last 30 years were assessed for endocrinopathies, from diagnosis to last documented follow-up. Mean age at diagnosis was 5 years 8 months. Of these, 15% (n = 21) had CDI, 7% had growth hormone deficiency (GHD) (n = 10) and 8% (n = 11) had more than one endocrinopathy noted during follow-up. Forty percent (n = 57) were pre-pubertal at the time of audit or upon discharge from tertiary services.
Conclusions:
Ongoing pituitary assessment, in addition to CDI, is required to detect evolving deficiencies of GHD and gonadotropins as these can be subtle, late or missed. Close follow-up of growth and progression through puberty, even if discharged from tertiary care, is essential.
