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Langerhans histiocytosis in children


Authors: Z. Křenová 1,2;  J. Šenkyřík 2,3
Authors‘ workplace: Klinika dětské onkologie, LF MU a FN Brno 1;  LF MU, Brno 2;  Klinika dětské radiologie, LF MU a FN Brno 3
Published in: Čes-slov Pediat 2021; 76 (8): 440-446.
Category: Original Papers

Overview

Langerhans cell histiocytosis (LCH) is the most common of the histiocytic disorders and occurs when the body accumulates too many immature Langerhans cells, a subset of the larger family of cells known as histiocytes. Scientific discussions on the definition of LCH used to be debated in terms of its classification as either an immune dysfunction or a rare cancer (neoplastic and malignant or not malignant). According to most experts, Langerhans cell histiocytosis (LCH) is currently classified as a myeloid neoplasm.

Keywords:

Langerhans histiocytosis – myeloid neoplasm – BRAFV600E mutation – LCH-associated neurodegeneration of brain


Sources

1. Rodriguez-Galindo C, et al. First line treatment of pediatric Langerhans cell histiocytosis. In: Abla Ousama, Janka Gritta (eds). Histiocytosis Disorders. Springer, 2018.

2. Haupt R, Minkov M, Astigarraga I, et al. Langerhans cell histiocytosis (LCH): Guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer 2013; 60c (2): 175–184.

3. Chellapandian D, Shaikh F, van den Bos C, et al. Management and outcome of patients with Langerhans cell histiocytosis and single-bone CNS-risk lesions: A multi-institutional retrospective study. Pediatr Blood Cancer 2015; 62 (12): 2162–2166.

4. Héritier S, Barkaoui MA, Miron J, et al. Incidence and risk factors for clinical neurodegenerative Langerhans cell histiocytosis: a longitudinal cohort study. Br J Haematol 2018; 183 (4): 608–617.

5. Eckstein OS, Visser J, Rodriguez-Galindo C, et al. Clinical responses and persistent BRAF V600E + blood cells in children with LCH treated with MAPK pathway inhibition. Blood 2019; 133 (15): 1691–1694.

6. Wnorowski M, Prosch H, Prayer D, et al. Pattern and course of neurodegeneration in Langerhans cell histiocytosis. J Pediatr 2008; 153 (1): 127–132.

7. Allen CE, Ladisch S, McClain KL. How I treat Langerhans cell histiocytosis. Blood 2015; 126 (1): 26–33.

8. McClain KL, Picarsic J, Chakraborty R, et al. CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions. Cancer 2018; 124 (12): 2607–2620.

9. Henter JI, Kvedaraite E, Martín Muñoz D, et al. Response to mitogen- activated protein kinase inhibition of neurodegeneration in Langerhans cell histiocytosis monitored by cerebrospinal fluid neurofilament light as a biomarker: a pilot study. Br J Haematol 2021. doi: 10.1111/bjh.17781.

10. Krenova Z, Sterba J. Langerhans cell histiocytosis: The time has come to consider a CNS-directed prophylactic approach. J Pediatr Hematol Oncol 2016; 38 (6): 493–495.

Labels
Neonatology Paediatrics General practitioner for children and adolescents
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