Background: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes
encompasses a wide clinical spectrum, ranging from a benign localized disease to acute
generalized disease with fatal outcome.
Objectives: to retrospectively evaluate clinical characteristics at diagnosis and outcome of
patients with Langerhans cell histiocytosis.
Patients and methods: A retrospective analysis of data on 21 children with Langerhans cell
histiocytosis followed at Oncology unit, Children Welfare Teaching Hospital, Medical City,
Baghdad, between 1999 and 2006.
Results : The age at time of diagnosis of LCH ranged from 3 months to 9 years, with a median of
22 months, and male to female ratio was 1:1.1. The duration of the onset of the disease before
diagnosis ranged from 1month to1 year. Bone lesions, skin lesions and LAP were the common
presenting features. Skull was the major site of lytic lesions 10(47.6%) patients. Tissue biopsy
and/or aspiration were the main diagnostic procedures. Twenty patients treated by different
combinations of chemotherapy. Ten patients survived (50%), and the mean time of follow up
was 28 months.
Conclusion : The study showed a relatively high incidence of advanced (III and IV) stages of
disease 12 patients (57.1 %) with subsequent poor outcome and survival.
Recommendation : Long term follow-up by a multidisciplinary care team is required