Causes of treatment failure for children with LCH in Morocco

Year 2014, Volume: 6 , - , 07.01.2014

Siham Cherkaoui Sarra Benmiloud Jamila El Houdzi Jamila Hachim Amina Kili Leila Hessissen Saadia Zafad

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

Keywords

Langerhans cell histiocytosis, Children, Therapy, Treatment failure

References

• Willman C, Busque L, Griffith BB, et al. Langerhans’ cell histiocytosis (histiocytosis X), a clonal proliferative disease. N Engl J Med 1994;331:154–160.
• Egeler M, D’Angio GJ. Langerhans cell histiocytosis. J Pediatr 1995;127:1-11. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer 1999;85:2278-90.
• Donadieu J, Guyot-Goubin A, Clavel J, Thomas C. Clinical presentation and epidemiology of childhood Langerhans cell Histiocytosis. Arch Pediatr. 2008;15:520-2.
• Salotti JA, Nanduri V, Pearce MS, et al. Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland. Arch Dis Child 2009; 94: 376-80.
• Donadieu J, The French Langerhans' Cell.Study Group A multicentre retrospective survey of Langerhans' cell histiocytosis: 348 cases observed between 1983 and 1993. Archives of Disease in Childhood 1996;75:17-24
• Goubin A, Donadieu J, Barkaoui M, et al. Descriptive epidemiological study of childhood langerhans cell histiocytosis in France from 2000 to 2004. Study of 258 cases. Pediatr Blood Cancer 2008,51:71-75
• Carstensen H, Ornvold K. The epidemiology of Langerhans cell histiocytosis in children in Denmark, 1975–1989. Med Pediatr Oncol 1993;21:387–388.
• Muller J, Garami M, Hauser P, et al. Hungarian experience with Langerhans cell histiocytosis in childhood. Pediatr Hematol Oncol 2006;23:135
• Karis J, Bernstrand C, Fadeel B, et al. The incidence of Langerhans cell histiocytosis in children in Stockholm county, Sweden, 1992– 200
• Alston RD, Tatevossian RG, McNally RJ, et al. Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998. Pediatr Blood Cancer 2007;48:555
• Minkov M. Multisystem Langerhans Cell Histiocytosis in Children. Pediatr Drugs 2011;13:75-86.
• Riccardo Haupt et al. Permanent Consequences in Langerhans Cell Histiocytosis Patients: A Pilot Study From the Histiocyte Society—Late Effects Study Group Pediatr Blood Cancer 2004;42:438– 444