Egyptian experience in Langerhans cells histiocytosis: frequent multisystem affection and reactivation rates

• Published in: Pediatric hematology and oncology Vol. 37; no. 8; pp. 696 - 706
• Main Authors: Tantawy, Azza A. Gawad, Ragab, Iman A., Elsherif, Nayera H. K., Makkeyah, Sara M., AbdelRaheem, Heba G., Elsantiel, Hisham I. E.
• Format: Journal Article
• Language: English
• Published: England Taylor & Francis 16-11-2020
• Subjects: Child; Child, Preschool; Cladribine - administration & dosage; Cyclosporine - administration & dosage; Disease Progression; Egypt; Female; Histiocytosis, Langerhans-Cell - drug therapy; Histiocytosis, Langerhans-Cell - mortality; Histiocytosis, Langerhans-Cell - physiopathology; Humans; Infant; Langerhans cells; Langerhans Cells - pathology; Liver - drug effects; Liver - pathology; Lymph Nodes - drug effects; Lymph Nodes - pathology; Lymphohistiocytosis, Hemophagocytic - complications; Lymphohistiocytosis, Hemophagocytic - drug therapy; Lymphohistiocytosis, Hemophagocytic - mortality; Lymphohistiocytosis, Hemophagocytic - physiopathology; Male; reactivations; Recurrence; Retrospective Studies; Risk Factors; Survival Rate; survival rates; Treatment Outcome; Egypt; Langerhans cells; reactivations; survival rates
• ISSN: 0888-0018; 1521-0669
• DOI: 10.1080/08880018.2020.1790703

Background: Histiocytoses are unique disorders; their clinical presentations vary from self-healing lesions to life-threatening disseminated disease. Objectives: We aimed to evaluate the different clinical presentations, frequency of reactivations, and treatment outcome of Langerhans cell histiocytosis among Egyptian children. Methods: we restrospectively analyzed the data of 37 Langerhans cell histiocytosis patients (LCH) registered at Ain Shams University Children's Hospital for clinicopathological features, treatment modalities and their outcomes. Results: Twenty seven (73%) of the studied patients with LCH had multisystem disease (MS), 24 (88.9%) of them had risk organ involvement (MS RO+) and only 3 without risk organ (MS RO-). Most of the patients received LCH III protocols. Eleven patients (29.7%) had reactivations with median time till reactivation of 17 months (IQR 5-23).Reactivation rates were 40% and 50% in patients with no evidence of active disease (NAD) and those with active disease better (AD better) at week 6 evaluation respectively (p = 0.71).We report 9 deaths (all had MS RO+, two died after reactivation and 7 had progressive disease. The 5 years EFS and OS were 49.4% and 81.2% respectively. Risk stratification did not significantly affect the EFS or OS (p = 0.64 and p = 0.5 respectively). Conclusion: A high reactivation rate was encountered in children with LCH and MS-RO + irrespective of 6 weeks response to induction therapy. A high mortality in patients with progressive disease necessitates a possible earlier aggressive salvage in such group.