Langerhans cell histiocytosis (eosinophilic granuloma) of bone in children and adolescents
- Kenneth L McClain, MD, PhD
Kenneth L McClain, MD, PhD
- Professor of Pediatrics
- Texas Children's Cancer Center/Hematology Service
- Baylor College of Medicine
Langerhans cell histiocytosis (LCH) of the bone, also called eosinophilic granuloma of bone, was first described in 1940 [1-4]. Patients may present with a solitary lesion (monostotic) or multiple sites of involvement (polyostotic) .
In 1953, eosinophilic granuloma of bone was classified along with Hand-Schüller-Christian disease (with the classic triad of exophthalmos, diabetes insipidus, and skull lesions) and Letterer-Siwe disease (lymphadenopathy, skin rash, hepatosplenomegaly, fever, anemia, and thrombocytopenia) as Histiocytosis X . Subsequently, the Langerhans cell of the skin was proposed as the underlying shared pathologic feature among these three disorders .
However, gene expression array analysis of CD207+ (langerin) histiocytes from bone lesions compared with the Langerhans cells of skin proves that these are two distinctly different cell types . The pathologic cells in LCH are now known to be derived from circulating myeloid dendritic cells .
Under the direction of the Writing Group of the Histiocyte Society, LCH has been adopted as the appropriate clinicopathologic designation that encompasses and essentially replaces the previous historical terms used to classify this category of abnormal histiocyte proliferation . However, many authors continue to utilize the term eosinophilic granuloma of bone; thus, this latter terminology is still both historically and clinically relevant.
This topic review will discuss LCH of the bone in children. Discussions of LCH involving other organs in children and adults are presented separately. (See "Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis" and "Pulmonary Langerhans cell histiocytosis".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Lichtenstein L, Jeffe HL. Eosinophilic granuloma of bone: With report of a case. Am J Pathol 1940; 16:595.
- Ghanem I, Tolo VT, D'Ambra P, Malogalowkin MH. Langerhans cell histiocytosis of bone in children and adolescents. J Pediatr Orthop 2003; 23:124.
- Plasschaert F, Craig C, Bell R, et al. Eosinophilic granuloma. A different behaviour in children than in adults. J Bone Joint Surg Br 2002; 84:870.
- Yasko AW, Fanning CV, Ayala AG, et al. Percutaneous techniques for the diagnosis and treatment of localized Langerhans-cell histiocytosis (eosinophilic granuloma of bone). J Bone Joint Surg Am 1998; 80:219.
- Schmitz L, Favara BE. Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998; 12:221.
- LICHTENSTEIN L. Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schüller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol 1953; 56:84.
- Nezelof C, Basset F, Rousseau MF. Histiocytosis X histogenetic arguments for a Langerhans cell origin. Biomedicine 1973; 18:365.
- 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 2010; 184:4557.
- Berres ML, Lim KP, Peters T, et al. BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups. J Exp Med 2014; 211:669.
- Histiocytosis syndrome in children. Writing Group of the Histiocyte Society. Lancet 1987; 24:208.
- Guyot-Goubin A, Donadieu J, Barkaoui M, et al. Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000-2004. Pediatr Blood Cancer 2008; 51:71.
- Malpas JS. Langerhans cell histiocytosis in adults. Hematol Oncol Clin North Am 1998; 12:259.
- Cochrane LA, Prince M, Clarke K. Langerhans' cell histiocytosis in the paediatric population: presentation and treatment of head and neck manifestations. J Otolaryngol 2003; 32:33.
- Bertram C, Madert J, Eggers C. Eosinophilic granuloma of the cervical spine. Spine (Phila Pa 1976) 2002; 27:1408.
- Jubran RF, Marachelian A, Dorey F, Malogolowkin M. Predictors of outcome in children with Langerhans cell histiocytosis. Pediatr Blood Cancer 2005; 45:37.
- Islinger RB, Kuklo TR, Owens BD, et al. Langerhans' cell histiocytosis in patients older than 21 years. Clin Orthop Relat Res 2000; :231.
- Karagoz Guzey F, Bas NS, Emel E, et al. Polyostotic monosystemic calvarial and spinal langerhans' cell histiocytosis treated by surgery and chemotherapy. Pediatr Neurosurg 2003; 38:206.
- Meyer JS, De Camargo B. The role of radiology in the diagnosis and follow-up of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998; 12:307.
- Jiang L, Liu ZJ, Liu XG, et al. Langerhans cell histiocytosis of the cervical spine: a single Chinese institution experience with thirty cases. Spine (Phila Pa 1976) 2010; 35:E8.
- Baumgartner I, von Hochstetter A, Baumert B, et al. Langerhans'-cell histiocytosis in adults. Med Pediatr Oncol 1997; 28:9.
- Bayazit Y, Sirikci A, Bayaram M, et al. Eosinophilic granuloma of the temporal bone. Auris Nasus Larynx 2001; 28:99.
- Grois N, Pötschger U, Prosch H, et al. Risk factors for diabetes insipidus in langerhans cell histiocytosis. Pediatr Blood Cancer 2006; 46:228.
- Howarth DM, Mullan BP, Wiseman GA, et al. Bone scintigraphy evaluated in diagnosing and staging Langerhans' cell histiocytosis and related disorders. J Nucl Med 1996; 37:1456.
- Aricò M, Egeler RM. Clinical aspects of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998; 12:247.
- Azouz EM, Saigal G, Rodriguez MM, Podda A. Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol 2005; 35:103.
- Binkovitz LA, Olshefski RS, Adler BH. Coincidence FDG-PET in the evaluation of Langerhans' cell histiocytosis: preliminary findings. Pediatr Radiol 2003; 33:598.
- De Schepper AM, Ramon F, Van Marck E. MR imaging of eosinophilic granuloma: report of 11 cases. Skeletal Radiol 1993; 22:163.
- Phillips M, Allen C, Gerson P, McClain K. Comparison of FDG-PET scans to conventional radiography and bone scans in management of Langerhans cell histiocytosis. Pediatr Blood Cancer 2009; 52:97.
- Braier J, Chantada G, Rosso D, et al. Langerhans cell histiocytosis: retrospective evaluation of 123 patients at a single institution. Pediatr Hematol Oncol 1999; 16:377.
- Emile JF, Abla O, Fraitag S, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016; 127:2672.
- Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127:2375.
- Terracciano L, Kocher T, Cathomas G, et al. Langerhans cell histiocytosis of the stomach with atypical morphological features. Pathol Int 1999; 49:553.
- Oliveira M, Steinbok P, Wu J, et al. Spontaneous resolution of calvarial eosinophilic granuloma in children. Pediatr Neurosurg 2003; 38:247.
- Yanagawa T, Watanabe H, Shinozaki T, et al. The natural history of disappearing bone tumours and tumour-like conditions. Clin Radiol 2001; 56:877.
- Gadner H, Minkov M, Grois N, et al. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood 2013; 121:5006.
- www.histiocytesociety.org (Accessed on March 31, 2010).