Langerhans-Cell Histiocytosis In Children: A Bicentric Study From Tunisia

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Published Jul 12, 2026
https://doi.org/10.62438/tunismed.v104i07.6763
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Vol. 104 No. 07 (2026): Tunis Med Jul 2026

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Samia Rekaia
Pediatric Hematology Department at the Bone Marrow Center Tunis , Faculty of medicine, University of Tunis El Manar ,Tunisia
Manel Gouja
Pediatric Hematology Department at the Bone Marrow Center Tunis, Tunisia
Feten Fedhila
Children's Medicine Department A at the Bechir Hamza Children's Hospital, Tunis, Faculty of medicine of Tunis, University of Tunis El Manar, Tunisia
Ilhem Ben Fradj
Pediatric Hematology Department at the Bone Marrow Center Tunis , Faculty of medicine, University of Tunis El Manar ,Tunisia
Samar Rhayem
Children's Medicine Department A at the Bechir Hamza Children's Hospital, Tunis, Faculty of medicine of Tunis, University of Tunis El Manar, Tunisia
Ghofrane Bedoui
Pediatric Hematology Department at the Bone Marrow Center Tunis, Tunisia
Aicha Bettaieb
Pediatric Hematology Department at the Bone Marrow Center Tunis , Faculty of medicine, University of Tunis El Manar ,Tunisia
Ameni Merdassi
Pediatric Hematology Department at the Bone Marrow Center Tunis , Faculty of medicine, University of Tunis El Manar ,Tunisia
Hajer Ben Belgacem
Pediatric Hematology Department at the Bone Marrow Center Tunis , Faculty of medicine, University of Tunis El Manar ,Tunisia
Fethi Mellouli
Pediatric Hematology Department at the Bone Marrow Center Tunis , Faculty of medicine, University of Tunis El Manar ,Tunisia
Mohamed Bejaoui
Pediatric Hematology Department at the Bone Marrow Center Tunis , Faculty of medicine, University of Tunis El Manar ,Tunisia
Monia Khemiri
Children's Medicine Department A at the Bechir Hamza Children's Hospital, Tunis, Faculty of medicine of Tunis, University of Tunis El Manar, Tunisia
Monia Ben Khaled
Pediatric Hematology Department at the Bone Marrow Center Tunis , Faculty of medicine, University of Tunis El Manar ,Tunisia
Monia Ouederni
Pediatric Hematology Department at the Bone Marrow Center Tunis , Faculty of medicine, University of Tunis El Manar ,Tunisia

Abstract

Langerhans cell histiocytosis (LCH) is the most common form of histiocytosis in children, characterized by clonal proliferation of CD1a+ and CD207+ dendritic cells. This retrospective study aimed to evaluate the clinical characteristics, treatment response, and progression of LCH in children diagnosed between January 2010 and July 2023 at two Tunisian referral centers. Forty-three patients were enrolled with a median age at diagnosis of 3 years. Multisystem LCH with risk organs involvement was the most common group (44%), followed by single-system LCH (37%). Bone, skin, and liver were the most frequently affected organs. Central nervous system tumorous lesions were observed in five patients. The BRAF-V600E mutation was identified in eight of nine tested cases. Thirty-four patients received a vinblastine-based first-line therapy; six patients (18%) showed no response after two courses of vinblastine induction. Two patients received a combination of Cytarabine and Cladribine, with a favorable outcome in one case. BRAF inhibitors were used in four cases, with a favorable response in two. Reactivations occurred in 28% of patients, and 23% experienced at least one permanent consequence. Overall survival (OS) and disease-free survival (DFS) rates were 93% and 63%, respectively. Risk organs involvement and poor response to vinblastine induction were associated with worse outcomes.

Keywords:

Pediatric, Langerhans cell histiocytosis, overall survival, disease-free survival, LCH reactivation

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