Causes of treatment failure for children with LCH in Morocco

Abstract

Abstract Background : Langerhans Cell Histiocytosis (LCH) is a rare disease with high survival rate. We noted poor outcomes in Morocco and thus this study was conducted to identify causes of preventable treatment failure in children. Patients & Methods : This study includes cases of children LCH followed in six Pediatric Oncology Units part of the Moroccan LCH Study Group (Groupe Marocain d'Etude de l'Histiocytose) between 2000 and 2009. Patients were categorized into risk group or low risk group as per the International Histiocyte Society classification. Results: Forty-two patients were included in the present study. The median age at diagnosis was 20.5 months. The sex-ratio M/F was 5. The onset symptom was a skin lesion in 20% of the cases and bone involvement in 17%. Seventeen patients had unifocal LCH (40%). The most common site of unifocal LCH was bone (65%) and the mean duration of the first episode treatment was 3 months. Complete or partial remission was achieved in 58% of the cases. Among 20 patients who had risk organ involvement, 11 died from progressive disease and 2 died from septic shock. The overall survival rate was 40% at 5 years. Conclusion : The most common cause of treatment failure was refractory disease in 26%. However, toxic death and abandonment caused an additional 24% of patients to fail treatment. Reducing this preventable failure requires carefully designed interventions. Thus, the Moroccan LCH Study Group was established in March 2010 and set as main objective the improvement of the therapeutic management