Clinical characteristics and outcome of pediatric patients diagnosed with Langerhans cell histiocytosis in pediatric hematology and oncology centers in Poland
Anna Raciborska,
Katarzyna Bilska,
Jadwiga Węcławek-Tompol,
Olga Gryniewicz-Kwiatkowska,
Małgorzata Hnatko-Kołacz,
Joanna Stefanowicz,
Anna Pieczonka,
Katarzyna Jankowska,
Filip Pierelejewski,
Tomasz Ociepa,
Grażyna Sobol-Milejska,
Katarzyna Muszyńska-Rosłan,
Olga Michoń,
Wanda Badowska,
Monika Radwańska,
Katarzyna Drabko
Affiliations
Anna Raciborska
Department of Oncology and Surgical Oncology for Children and Youth, Institute of Mother and Child
Katarzyna Bilska
Department of Oncology and Surgical Oncology for Children and Youth, Institute of Mother and Child
Jadwiga Węcławek-Tompol
Department and Clinic of Pediatric Oncology, Hematology and Bone Marrow Transplantation, Wroclaw Medical University
Olga Gryniewicz-Kwiatkowska
Department of Oncology, Children’s Memorial Health Institute
Małgorzata Hnatko-Kołacz
Department of Oncology and Hematology, University Children’s Hospital of Cracow
Joanna Stefanowicz
Department of Pediatric Hematology and Oncology, Medical University of Gdansk
Anna Pieczonka
Department of Pediatric Oncology, Hematology and Transplantology, Medical University of Poznan
Katarzyna Jankowska
Department of Pediatric Hematology and Oncology Collegium Medicum, Nicolaus Copernicus University
Filip Pierelejewski
Department of Pediatrics, Oncology and Hematology, Medical University of Lodz
Tomasz Ociepa
Department of Pediatrics, Hematology and Oncology, Medical University of Szczecin
Grażyna Sobol-Milejska
Unit of Pediatric Oncology, Hematology and Chemotherapy, Medical University of Silesia
Katarzyna Muszyńska-Rosłan
Department of Pediatric Oncology and Hematology, Medical University of Bialystok, Children’s Clinical Hospital of L. Zamenhof
Olga Michoń
Department of Pediatrics, Hematology and Oncology, Medical University of Zabrze
Wanda Badowska
Department of Pediatric Oncology, Hematology, Medical University of Olsztyn
Monika Radwańska
Department of Pediatric Oncology, Hematology, Medical University of Rzeszow
Katarzyna Drabko
Department of Pediatric Hematology, Oncology and Transplantology, Medical University of Lublin
Abstract Background Langerhans cell histiocytosis (LCH) affects 1–2 in 1,000,000 people. The disease is not associated with increased risk of treatment failure (especially among older children), but appropriate procedures implemented in advance can eliminate complications which might appear and significantly worsen the patients’ quality of life. Thus, we sought to evaluate the clinical features, management, and outcome of children with LCH treated in Polish pediatric hematology-oncology centers. Materials and methods One hundred eighty two patients with LCH were treated according to the Histiocytic Society Guidelines between 2010 and 2017. The participating centers were requested to provide the following data: demographic, clinical, as well as local or systemic treatment data and patients’ outcome. Overall survival (OS) and event free survival (EFS) were estimated by Kaplan-Meier methods and compared using the log-rank test. Results Sixty nine percent of children were classified as single system (SS). The patients with SS disease were significantly older as compared to the children with multisystem disease (MS), 6 vs. 2.3 years respectively (p 0.003). Bones were involved in 76% of patients. Systemic treatment was applied to 47% of children with SS disease and 98% with MS disease. Fourteen patients relapsed while two children died. OS and EFS in entire group were 0.99 and 0.91 respectively (with median follow-up 4.3 years). Conclusion The treatment of LCH in Polish centers was effective, however, new approaches, including mutation analyses and good inter-center cooperation, are needed to identify patients who might require modification or intensification of treatment.
