Abstract
Background: Langerhans cell histiocytosis (LCH) is a poorly understood disease with heterogeneous
clinical presentation ranging from unifocal bony involvement to disseminated disease
with life-threatening complications. Materials and Methods: The clinical profile, laboratory findings, treatment, and long-term outcome were
retrieved from maintained medical records from January 2006 to January 2016 and were
retrospectively analyzed. The extent of the disease was classified as per the LCH-III
trial of “The Histiocyte Society.” The assessment and categorization of treatment
response followed LCH III trial definitions. Results: A total of 28 children with LCH were diagnosed. The age ranged between 5 months and
9 years, with a mean of 3½ years. The M: F ratio was 3:1. Single system, unifocal
and multifocal bone diseases were seen in nine (32.1%) and two (7.1%) cases, respectively.
Disseminated disease without risk organ involvement was seen in six (21.1%), whereas
disseminated disease with risk organ involvement was seen in 11 (39.3%) cases. The
most common presentation was bony involvement (19 [67.8%]), out of which 16 (88.8%)
had skull involvement. During follow-up, 17 (60.7%) were in complete remission though
five (17.8%) of them relapsed, but achieved second remission. Two (7.1%) were lost
to follow-up. Six (21.4%) had progressive disease of which four expired and two abandoned
treatment. Two (10.7%) refused the initiation of treatment. Conclusion: A better understanding of the disease, early suspicion, and diagnosis can improve
the outcome of patients with LCH.
Key words
Histiocytosis - Langerhans cell - outcome - risk organ - single system - survival
