e-ISSN 2460-7991

Langerhans cell histiocytosis: Diagnosis and Management

Font Size:  Small  Medium  Large

Langerhans cell histiocytosis: Diagnosis and Management

Lusiana Lusiana, Hikari Ambara Sjakti, Githa Rahmayunita, Tjut Nurul Alam Jacoeb, Inge Ade Krisanti

Abstract


 

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal clonal proliferation of Langerhans dendritic cells. The incidence of LCH is 1 to 3 cases per 1 million children worldwide, and occurs most frequently in children of 1–4 years of age. The etiopathogenesis of LCH, whether it is neoplastic or reactive, is still controversial. Langerhans cell histiocytosis has a wide spectrum of clinical features, and dermatological abnormalities usually occur early. The most frequent lesions are elevated yellowish-red translucent papules about 1–2 mm in diameter and generally located in seborrheic areas. The most recent classification (the Histiocyte Society study 2017) categorized LCH into four groups; a single system with involvement of unifocal or multifocal organs, lung LCH, and multi-system LCH with either low- or high-risk multiorgan involvement. The definitive diagnosis of LCH are typical morphology along with Birbeck granules and/or positive results on CD1a antigen stain on cells found on lesions. Treatment of LCH is multimodal, determined based on age, extent of lesion, organ involvement, and organ location.

 

Keywords: histiocytosis, Langerhans cells, dendritic cells, children

 


Full Text: PDF

References


References

 

1.      Uppuluri R, Ramachandrakurup S, Subburaj D, Bakane A, Raj R. Excellent remission rates with limited toxicity in relapsed/refractory Langerhans cell histiocytosis with pulse dexamethasone and lenalidomide in children. Pediatr Blood Cancer. 2017;64(1):110-2.

2.      Gelmetti C. Langerhans Cell Histiocytosis. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffel JD, Wolff K, editor. Fitzpatrick’s dermatology in general medicine. 8th Edition. New York: McGraw-Hill. 2012:1782-95.

3.      Paller AS and Mancini AJ. Histiocytosis and Malignant Skin Diseases. In: Bonnett C, Gabbedy R, eds. Hurwitz Clinical Pediatric Dermatology. 4th Edition ed: Elsevier, 2011:219-23.

4.      Zinn DJ, Chakraborty R, Allen CE. Langerhans Cell Histiocytosis: Emerging Insights and Clinical Implications. Oncology (Williston Park) 2016;30:122-32, 39.

5.      Wollina U, Langner D, Hansel G, Schonlebe J. Cutaneous Langerhans cell histiocytosis : The spectrum of a rare cutaneous neoplasia.  Wien Med Wochenschr. 2016;168(9-10):243-7.

6.      Egeler RM, Katewa S, Leenen PJ, Beverley P, Collin M, Ginhoux F, et al. Langerhans cell histiocytosis is a neoplasm and consequently its recurrence is a relapse: In memory of Bob Arceci. Pediatr Blood Cancer 2016;63:1704-12.

7.      Minkov M, Grois N, McClain K, Nanduri V, Rodriguez-Galindo C, Simonitsch-Klupp I, et al. Langerhans Cell Histiocytosis Evaluation and Treatment Guidelines. In: Histiocytosis Society. 2009.

8.      Rigaud C, Barkaoui MA, Thomas C, Bertrand Y, Lambilliotte A, Miron J, et al. Langerhans cell histiocytosis: therapeutic strategy and outcome in a 30-year nationwide cohort of 1478 patients under 18 years of age. Br J Haematol 2016;174:887-98.

9.      Monsereenusorn C and Rodriguez-Galindo C. Clinical Characteristics and Treatment of Langerhans Cell Histiocytosis. Hematol Oncol Clin North Am 2015;29:853-73.

10.    1Morren MA, Vanden Broecke K, Vangeebergen L, Sillevis-Smitt JH, Van Den Berghe P, Hauben E, et al. Diverse Cutaneous Presentations of Langerhans Cell Histiocytosis in Children: A Retrospective Cohort Study. Pediatr Blood Cancer 2016;63:486-92.

11.    Diana IA. Histiositosis Sel Langerhans. In: Sugito TL, Lokanata MD, et al., eds. Update Dermatologi Pediatrik. Jakarta: PERDOSKI-Kelompok Studi Dermatologi Anak Indonesia, 2016:186-98.

12.    Chu AC. Histiocytosis. In: Burns T, Breathnach S, et al., eds. Rook's Textbook of Dermatology. 8th ed: Oxford: Blackwell Scientific, 2010:55.1-34.

13.    Orle J, Mosca AM, Sousa MA, Lima CM, Adriano AR, Rezende PM. Congenital self healing reticulohistiocytosis in a newborn (Hashimoto Pritzker). An Bras Dermatol 2011;86:785-8.

14.    Haupt R, Minkov M, Astigarraga I, Schafer E, Nanduri V, Jubran R, 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;60:175-84.

15.    Minkov M. Multisystem Langerhans cell histiocytosis in children: current treatment and future directions. Paediatr Drugs 2011;13:75-86.

16.    Emile, JF, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016;127: 2672-81.

17.    Shi Y, Qiao Z, Xia C, Gong Y, Yang H, Li G, et al. Hepatic involvement of Langerhans cell histiocytosis in children--imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography. Pediatr Radiol 2014;44:713-8.

18.    Lau SK, Chu PG, Weiss LM. Immunohistochemical expression of Langerin in Langerhans cell histiocytosis and non-Langerhans cell histiocytic disorders. Am J Surg Pathol 2008;32:615-9.

19.    Kusuma Kumary P, Priyakumari T, Chellam VG, James FV, Nair MK. Langerhans cell histiocytosis in children less than 2 years of age. Indian Pediatr 1999;36:29-36.

20.    Aihara M, Hasegawa T, Okuyama Y, Hiruma M, Ikeda S. Langerhans cell histiocytosis treated with narrow-band ultraviolet B. J Dermatol 2011;38:151-4.

21.    Karkashan E, Alsharif S, Alali A, Dahlan M. Successful Treatment of Cutaneous Langerhans Cell Histiocytosis with Intralesional Steroids: Case Report and Literature Review. Am J Dermatol Venerol 2017;6:1-5.

22.    Steen AE, Steen KH, Bauer R, Bieber T. Successful treatment of cutaneous Langerhans cell histiocytosis with low-dose methotrexate. Br J Dermatol 2001;145:137-40.

23.    Kwon OS, Cho KH, Song KY. Primary cutaneous Langerhans cell histiocytosis treated with photochemotherapy. J Dermatol 1997;24:54-6.

24.    Do JE, Lee JY, Kim YC. Successful treatment of cutaneous Langerhans' cell histiocytosis with targeted narrowband ultraviolet B phototherapy in an infant. Clin Exp Dermatol 2009;34:e280-1.

25.    Ness MJ, Lowe GC, Davis DMR, Hand JL. Successful Treatment of Cutaneous Langerhans Cell Histiocytosis with Intralesional Steroids: Case Report and Literature Review. Pediatr Dermatol 2014;31:e10–e2.

26.    Ladisch S, Gadner H, Arico M, Broadbent V, Grois N, Jacobson A, et al. LCH-I: a randomized trial of etoposide vs. vinblastine in disseminated Langerhans cell histiocytosis. The Histiocyte Society. Med Pediatr Oncol 1994;23:107-10.

27.    Gadner H, Grois N, Arico M, Broadbent V, Ceci A, Jakobson A, et al. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr 2001;138:728-34.

28.    Gadner H, Grois N, Potschger U, Minkov M, Arico M, Braier J, et al. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood 2008;111:2556-62.

29.    Gadner H, Minkov M, Grois N, Potschger U, Thiem E, Arico M, et al. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood 2013;121:5006-14.

30.    Minkov M, Grois N, Heitger A, Potschger U, Westermeier T, Gadner H. Treatment of multisystem Langerhans cell histiocytosis. Results of the DAL-HX 83 and DAL-HX 90 studies. DAL-HX Study Group. Klin Padiatr 2000;212:139-44.

31.    Podjasek JO, Loftus CG, Smyrk TC, Wieland CN. Adult-onset systemic Langerhans cell histiocytosis mimicking inflammatory bowel disease: the value of skin biopsy and review of cases of Langerhans cell histiocytosis with cutaneous involvement seen at the Mayo Clinic. Int J Dermatol 2014;53:305-11.

32.    Picarsic J, Egeler RM, Chikwava K, Patterson K, Jaffe R. Histologic patterns of thymic involvement in Langerhans cell proliferations: a clinicopathologic study and review of the literature. Pediatr Dev Pathol 2015;18:127-38.

33.    Herwig MC, Wojno T, Zhang Q, Grossniklaus HE. Langerhans cell histiocytosis of the orbit: five clinicopathologic cases and review of the literature. Surv Ophthalmol 2013;58:330-40.

34.    Pagnoux C, Hayem G, Roux F, Palazzo E, Meyer O. [Sclerosing cholangitis as a complication of Langerhans'cell histiocytosis]. Rev Med Interne 2003;24:324-7.

35.    Sabib M, Ettair S, Erreimi N, Mouane N. [Sclerosing cholangitis revealing Langerhans cell histiocytosis in a 15-month-old child]. Arch Pediatr 2011;18:974-8.

36.    Donadieu J, Rolon MA, Thomas C, Brugieres L, Plantaz D, Emile JF, et al. Endocrine involvement in pediatric-onset Langerhans' cell histiocytosis: a population-based study. J Pediatr 2004;144:344-50.

37.    The French Langerhans' Cell Histiocytosis Study Group. A multicentre retrospective survey of Langerhans' cell histiocytosis: 348 cases observed between 1983 and 1993. Arch Dis Child 1996;75:17-24.

38.    Ng SS, Koh MJ, Tay YK. Cutaneous Langerhans cell histiocytosis: study of Asian children shows good overall prognosis. Acta Paediatr 2013;102:e514-8.