Langerhans cell histiocytosis with multisystem involvement in an infant: A case report

Bi,Lintao; Sun,Butong; Lu,Zhenxia; Shi,Zhangzhen; Wang,Dan; Zhu,Zhenxing

doi:10.3892/etm.2015.2396

Langerhans cell histiocytosis with multisystem involvement in an infant: A case report

Authors:
- Lintao Bi
- Butong Sun
- Zhenxia Lu
- Zhangzhen Shi
- Dan Wang
- Zhenxing Zhu
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Affiliations: Department of Hematology and Oncology, China‑Japan Union Hospital of Jilin University, Changchun, Jilin 130033, P.R. China
Published online on: March 30, 2015 https://doi.org/10.3892/etm.2015.2396
Pages: 2137-2140

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Abstract

Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells, with a wide range of clinical presentations that vary from a solitary lesion to more severe multifocal or disseminated lesions. The disease can affect any age group; however, the peak incidence rate is in infants aged between 1 and 3 years‑old. Diagnosis of LCH should be based on the synthetical analysis of clinical presentations, in addition to features of imaging and histopathology. Although certain cases regress spontaneously, other patients require systemic chemotherapy together with the administration of steroids. The present study reports the case of an infant with LDH with multisystem involvement, including that of the bone, skin, orbit, spleen and lungs. The patient received chemotherapy and obtained rapid improvement in the involved systems. A total of 2.5 years after completion of the therapy, the patient still remains in follow‑up and no evidence of active disease has been noted.

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1	Satter EK and High WA: Langerhans cell histiocytosis: A review of the current recommendations of the Histiocyte Society. Pediatr Dermatol. 25:291–295. 2008. View Article : Google Scholar : PubMed/NCBI
2	Favara BE, McCarthy RC and Mierau GW: Histiocytosis X. Hum Pathol. 14:663–676. 1983. View Article : Google Scholar : PubMed/NCBI
3	Lieberman PH, Jones CR, Steinman RM, et al: Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol. 20:519–552. 1996. View Article : Google Scholar : PubMed/NCBI
4	Egeler RM and D'Angio GJ: Langerhans cell histiocytosis. J Pediatr. 127:1–11. 1995. View Article : Google Scholar : PubMed/NCBI
5	Schulze J, Kitz R, Gruttner HP, et al: Severe isolated pulmonary Langerhans cell histiocytosis in a 6-year-old girl. Eur J Pediatr. 163:320–322. 2004. View Article : Google Scholar : PubMed/NCBI
6	Lajolo C, Campisi G, Deli G, et al: Langerhans's cell histiocytosis in old subjects: Two rare case reports and review of the literature. Gerodontology. 29:e1207–e1214. 2012. View Article : Google Scholar : PubMed/NCBI
7	Minkov M, Prosch H, Steiner M, et al: Langerhans cell histiocytosis in neonates. Pediatr Blood Cancer. 45:802–807. 2005. View Article : Google Scholar : PubMed/NCBI
8	McClain KL: Langerhans cell histiocytosis: what is the orphan telling us? Hematology (Am Soc Hematol Educ Program). 84–87. 2004.
9	Isaacs H Jr: Fetal and neonatal histiocytoses. Pediatr Blood Cancer. 47:123–129. 2006. View Article : Google Scholar : PubMed/NCBI
10	Abla O, Egeler RM and Weitzman S: Langerhans cell histiocytosis: Current concepts and treatments. Cancer Treat Rev. 36:354–359. 2010. View Article : Google Scholar : PubMed/NCBI
11	Allen CE and McClain KL: Langerhans cell histiocytosis: a review of past, current and future therapies. Drugs Today (Barc). 43:627–643. 2007. View Article : Google Scholar : PubMed/NCBI
12	Lau EG, Stepenaskie S, Moran R, et al: ʻBlueberry muffinʼ rash and large right thigh mass: a unique presentation of Langerhans cell histiocytosis. Dermatol Online J. 19:185682013.PubMed/NCBI
13	Herwig MC, Wojno T, Zhang Q and Grossniklaus HE: Langerhans cell histiocytosis of the orbit: Five clinicopathologic cases and review of the literature. Surv Opthalmol. 58:330–340. 2013. View Article : Google Scholar
14	Kasper EM, Aguirre-Padilla DH, Alter RY, et al: Histiocytosis X: Characteristics, behavior and treatments as illustrated in a case series. Surg Neurol Int. 2:572011. View Article : Google Scholar : PubMed/NCBI
15	Kairouz S, Hashash J, Kabbara W, et al: Dendritic cell neoplasms: an overview. Am J Hematol. 82:924–928. 2007. View Article : Google Scholar : PubMed/NCBI
16	Takahashi K, Harada M, Kimoto M and Kondo F: Diagnostic confirmation of Langerhans cell histiocytosis of the jaws with CD1a immunostaining: a case report. J Oral Maxillofac Surg. 61:118–122. 2003. View Article : Google Scholar : PubMed/NCBI
17	Valladeau J, Dezutter-Dambuyant C and Saeland S: Langerin/CD207 sheds light on formation of birbeck granules and their possible function in Langerhans cells. Immunol Res. 28:93–107. 2003. View Article : Google Scholar : PubMed/NCBI
18	Rizzo FM, Cives M, Simone V, et al: New insights into the molecular pathogenesis of langerhans cell histiocytosis. Oncologist. 19:151–163. 2014. View Article : Google Scholar : PubMed/NCBI
19	Egeler RM, van Halteren AG, Hogendoorn PC, et al: Langerhans cell histiocytosis: fascinating dynamics of the dendritic cell-macrophage lineage. Immunol Rev. 234:213–232. 2010. View Article : Google Scholar : PubMed/NCBI
20	Allen CE, Li L, Peters TL, et al: Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. J Immunol. 184:4557–4567. 2010. View Article : Google Scholar : PubMed/NCBI
21	Annels NE, Da Costa CE, Prins F, et al: Aberrant chemokine receptor expression and chemokine production by Langerhans cells underlies the pathogenesis of Langerhans cell histiocytosis. J Exp Med. 197:1385–1390. 2003. View Article : Google Scholar : PubMed/NCBI
22	Hicks J and Flaitz CM: Langerhans cell histiocytosis: current insights in a molecular age with emphasis on clinical oral and maxillofacial pathology practice. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 100:(2 Suppl). 42–66. 2005. View Article : Google Scholar
23	Bechan GI, Egeler RM and Arceci RJ: Biology of Langerhans cells and Langerhans cell histiocytosis. Int Rev Cytol. 254:1–43. 2006. View Article : Google Scholar : PubMed/NCBI
24	De Filippi P, Badulli C, Cuccia M, et al: Specific polymorphisms of cytokine genes are associated with different risks to develop single-system or multi-system childhood Langerhans cell histiocytosis. Br J Haematol. 132:784–787. 2006. View Article : Google Scholar : PubMed/NCBI
25	Egeler RM, Annels NE and Hogendoorn PC: Langerhans cell histiocytosis: a pathologic combination of oncogenesis and immune dysregulation. Pediatr Blood Cancer. 42:401–403. 2004. View Article : Google Scholar : PubMed/NCBI
26	Chikwava KR, Hunt JL, Mantha GS, et al: Analysis of loss of heterozygosity in single-system and multisystem Langerhans' cell histiocytosis. Pediatr Dev Pathol. 10:18–24. 2007. View Article : Google Scholar : PubMed/NCBI
27	Badalian-Very G, Vergilio JA, Degar BA, et al: Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 116:1919–1923. 2010. View Article : Google Scholar : PubMed/NCBI
28	Sahm F, Capper D, Preusser M, et al: BRAFV600E mutant protein is expressed in cells of variable maturation in Langerhans cell histiocytosis. Blood. 120:e28–e34. 2012. View Article : Google Scholar : PubMed/NCBI
29	Haroche J, Cohen-Aubart F, Emile JF, et al: Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation. Blood. 121:1495–1500. 2013. View Article : Google Scholar : PubMed/NCBI
30	Minkov M: Multisystem Langerhans cell histiocytosis in children: current treatment and future directions. Paediatr Drugs. 13:75–86. 2011. View Article : Google Scholar : PubMed/NCBI
31	Behrman RE, Kliegman RM and Jenson HB: Class 1 histiocytosesNelson Textbook of Pediatrics. 16th. W.B. Saunders; Philadelphia, PA: pp. 1570–1572. 2000
32	Jordan MB, McClain KL, Yan X, Hicks J and Jaffe R: Anti-CD52 antibody, alemtuzumab, binds to Langerhans cells in Langerhans cell histiocytosis. Pediatr Blood Cancer. 44:251–254. 2005. View Article : Google Scholar : PubMed/NCBI