Haematology

Langerhans Cell Histiocytosis (LCH)

The surveillance of Langerhans Cell Histiocytosis (LCH) took place from June 2003 to May 2005. There had been few large multi-centre studies of LCH, and there was poor documentation of its epidemiology. The study aimed to describe the epidemiology of LCH in the UK and Ireland as well as variations between ethnic groups and regions. It also aimed to assess the presenting features, the interval between LCH symptoms and diagnosis, referral patterns and outcome for the disease.

By bpsu · June 1, 2003

Lead investigator

Prof L Parker

About the study

Langerhans Cell Histiocytosis (LCH) is a multisystem disorder affecting children and adults. It may present with skin rash, bony lesions, hormone deficiencies or involvement of vital organs. If only one system had been affected, little or no treatment may have been required. Typically, the disease regressed with time, although this may have taken years.

However, children under two years who had the most serious form of LCH with lung, liver, bone marrow, or spleen involvement were managed using the UK Children’s Cancer Study Group (UKCCSG) international protocol. Children who survived severe LCH had long-term sequelae, which may have had a significant impact on their health and quality of life.

LCH can be presented at any age, ranging from the neonatal period to old age, with a peak in diagnosis in children aged 1 -3 years, with a predominance of boys. LCH was usually considered a non-familial disease. However, at least 1 % of LCH paediatric patients had another familial case which may involve siblings, cousins or two generations. There had been few large multi-centre studies of LCH. There was poor documentation of the epidemiology of LCH.

There were no well-accepted environmental risk factors for the disease. However, one US research study found an association between thyroid disease and infections from the neonatal period. There was also a protective correlation with childhood vaccinations before LCH. Reliable data on the annual incidence were difficult to gather. Only one national incidence estimate (5.4 per million per year) had been reported, for Denmark, during the 1980’s.

The study aimed to:

  • Describe the epidemiology of LCH in the UK and Ireland by age and gender.
  • Study variation in ethnic groups and regional differences.
  • Assess the presenting features, the interval between LCH symptoms and diagnosis, referral patterns and outcome for the disease.

Duration

June 2003 – May 2005

Published papers

Salotti JA, Nanduri V, Pearce MS, Parker L, Lynn R, Windebank KP. Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland. Arch Dis Child. 2009 May;94(5):376-80. doi: 10.1136/adc.2008.144527. Epub 2008 Dec 5. PMID: 19060008.

BPSU 20th Annual report 2005 -2006

Support group

Histio UK
Web: http://www.histiouk.org Email: info@hrtrust.org