OUTCOME OF TREATING PEDIATRIC LANGERHANS HISTIOCYTOSIS WITH LCH III IN
TERTIARY CENTER, SAUDI ARABIA.
Abstract
Langerhans cell histiocytosis (LCH) is a rare disease affects any age
and any organ; its presentations and outcome vary from self-healing
lesions to life-threatening disseminated disease. This study evaluates
the outcome of treating children diagnosed with Langerhans cell
histiocytosis (LCH) at Oncology Center, Saudi Arabia. Procedure: Through
a retrospective study design, the researchers reviewed the medical
records and electronic files of all children (aged from 0-≤ 14 years)
who had been diagnosed and treated for LCH at Princess Norah Oncology
Center (PNOC), King Abdelaziz Medical City, Saudi Arabia, in the period
from January 2000 to December 2019 (n=33). Results Males constituted
(66.7%), with remarkable dominance of Saudis (93.9%). The median age
at diagnosis was 28 months (IQR=49 months); (42.4%) were diagnosed
before reaching their second birthday. Fourteen patients (42.4%) had
multisystem (MS-LCH) involvement, of which 13 patients with risk organ
(RO) (+) and one patient without risk organ (RO) (-). Most of the
patients received LCH III protocols. Reactivation occurred in 11
patients (33.3%), and two deaths (6.1%) occurred in cases with MS (RO)
(+) progressive disease. The overall survival was 93.9%; with no
statistically significant difference in event free survival observed
between patients with multisystem compared to single system involvement.
Conclusion Excellent outcome of LCH is associated with single system
involvement and worse outcome (reactivations, or morality) is determined
by multi-organ involvement especially at younger age less than 24
months. Understanding of pathophysiology and genetic molecular
background could lead a novel therapy that warrants a prospective
clinical trial.