Langerhans Cell Histiocytosis in Children - a Disease with Many Faces. Recent Advances in Pathogenesis, Diagnostic Examinations and Treatment

Overview

Journal Postepy Dermatol Alergol

Publisher Termedia

Specialty Allergy & Immunology

Date 2018 Mar 31

PMID 29599667

Citations 49

Authors

Michalina Jezierska

Joanna Stefanowicz

Grzegorz Romanowicz

Wojciech Kosiak

Magdalena Lange

Affiliations

Soon will be listed here.

Abstract

Langerhans cell histiocytosis is a rare clonal disease characterized by the proliferation of CD1a-positive immature dendritic cells. The purpose of this article was to present an updated review of recent advances in the pathogenesis, clinical features, imaging and treatment of this disease. The discovery of oncogenic BRAF mutations and the presence of proinflammatory cytokines and chemokines confirmed the unusual characteristics of this disease. Currently, children with organ involvement who do not have a good response to chemotherapy and have neurodegeneration or diabetes insipidus are the most problematic patients. Further research is needed to improve the results of treatment.

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References

Lasek-Duriez A, Charkaluk M, Gosset P, Modiano P . [Blueberry Muffin Baby and Langerhans' congenital cell histiocytosis]. Ann Dermatol Venereol. 2014; 141(2):130-3. DOI: 10.1016/j.annder.2013.10.057. View

Wnorowski M, Prosch H, Prayer D, Janssen G, Gadner H, Grois N . Pattern and course of neurodegeneration in Langerhans cell histiocytosis. J Pediatr. 2008; 153(1):127-32. DOI: 10.1016/j.jpeds.2007.12.042. View

McClain K . Drug therapy for the treatment of Langerhans cell histiocytosis. Expert Opin Pharmacother. 2005; 6(14):2435-41. DOI: 10.1517/14656566.6.14.2435. View

Kaste S, Rodriguez-Galindo C, McCarville M, Shulkin B . PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 2007; 37(7):615-22. DOI: 10.1007/s00247-007-0467-4. View

Senechal B, Elain G, Jeziorski E, Grondin V, De Serre N, Jaubert F . Expansion of regulatory T cells in patients with Langerhans cell histiocytosis. PLoS Med. 2007; 4(8):e253. PMC: 1945037. DOI: 10.1371/journal.pmed.0040253. View

Davidson L, McComb J, Bowen I, Krieger M . Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution. J Neurosurg Pediatr. 2008; 1(3):187-95. DOI: 10.3171/PED/2008/1/3/187. View

Tweedie D, Sambamurti K, Greig N . TNF-alpha inhibition as a treatment strategy for neurodegenerative disorders: new drug candidates and targets. Curr Alzheimer Res. 2007; 4(4):378-85. DOI: 10.2174/156720507781788873. View

Morimoto A, Ishida Y, Suzuki N, Ohga S, Shioda Y, Okimoto Y . Nationwide survey of single-system single site Langerhans cell histiocytosis in Japan. Pediatr Blood Cancer. 2009; 54(1):98-102. DOI: 10.1002/pbc.22224. View

Sieni E, Barba C, Mortilla M, Savelli S, Grisotto L, Di Giacomo G . Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis. PLoS One. 2015; 10(7):e0131635. PMC: 4503531. DOI: 10.1371/journal.pone.0131635. View

10.

Favara B, Jaffe R, Egeler R . Macrophage activation and hemophagocytic syndrome in langerhans cell histiocytosis: report of 30 cases. Pediatr Dev Pathol. 2002; 5(2):130-40. DOI: 10.1007/s10024001-0159-2. View

11.

Lau L, Stuurman K, Weitzman S . Skeletal Langerhans cell histiocytosis in children: permanent consequences and health-related quality of life in long-term survivors. Pediatr Blood Cancer. 2007; 50(3):607-12. DOI: 10.1002/pbc.21322. View

12.

Laurencikas E, Gavhed D, Stalemark H, Vant Hooft I, Prayer D, Grois N . Incidence and pattern of radiological central nervous system Langerhans cell histiocytosis in children: a population based study. Pediatr Blood Cancer. 2010; 56(2):250-7. DOI: 10.1002/pbc.22791. View

13.

Sasaki Y, Guo Y, Arakawa F, Miyoshi H, Yoshida N, Koga Y . Analysis of the BRAFV600E mutation in 19 cases of Langerhans cell histiocytosis in Japan. Hematol Oncol. 2016; 35(3):329-334. DOI: 10.1002/hon.2293. View

14.

Willman C, Busque L, Griffith B, Favara B, McClain K, Duncan M . Langerhans'-cell histiocytosis (histiocytosis X)--a clonal proliferative disease. N Engl J Med. 1994; 331(3):154-60. DOI: 10.1056/NEJM199407213310303. View

15.

Braier J, Ciocca M, Latella A, de Davila M, Drajer M, Imventarza O . Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell Histiocytosis. Med Pediatr Oncol. 2002; 38(3):178-82. DOI: 10.1002/mpo.1306. View

16.

Lin C, Lin W, Chiang I, Ho Y, Peng C, Wu K . Langerhans cell histiocytosis with thyroid and lung involvement in a child: a case report. J Pediatr Hematol Oncol. 2010; 32(4):309-11. DOI: 10.1097/MPH.0b013e3181c4de1a. View

17.

Madrigal-Martinez-Pereda C, Guerrero-Rodriguez V, Guisado-Moya B, Meniz-Garcia C . Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations. Med Oral Patol Oral Cir Bucal. 2009; 14(5):E222-8. View

18.

Yadav S, Kharya G, Mohan N, Sehgal A, Bhat S, Jain S . Langerhans cell histiocytosis with digestive tract involvement. Pediatr Blood Cancer. 2010; 55(4):748-53. DOI: 10.1002/pbc.22663. View

19.

Badalian-Very G, Vergilio J, Degar B, Macconaill L, Brandner B, Calicchio M . Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010; 116(11):1919-23. PMC: 3173987. DOI: 10.1182/blood-2010-04-279083. View

20.

Battistella M, Fraitag S, Teillac D, Brousse N, de Prost Y, Bodemer C . Neonatal and early infantile cutaneous langerhans cell histiocytosis: comparison of self-regressive and non-self-regressive forms. Arch Dermatol. 2010; 146(2):149-56. DOI: 10.1001/archdermatol.2009.360. View