Adult Langerhans Cell Histiocytosis: A Rare Etiology of Spinal Cord Compression


Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. Clinical presentation is variable, ranging from a single location in the bone to severe multivisceral involvement. Moreover, spinal involvement causing myelopathy is even rare and unusual. We report a rare case of adult Langerhans cell histiocytosis in the dorsal spine causing a spinal cord compression associated with a pulmonary process treated by surgery, radiotherapy and systemic therapy with good evolution.

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Kouhen, F. , Benhmiddou, N. , Afif, M. , Rais, F. , Mahdi, Y. , Khannoussi, B. , Bellahamou, K. , Ghissassi, I. , Boussouni, K. , Elkacemi, H. , Elmajjaoui, S. , Kebdani, T. and Benjaafar, N. (2015) Adult Langerhans Cell Histiocytosis: A Rare Etiology of Spinal Cord Compression. Open Journal of Internal Medicine, 5, 21-28. doi: 10.4236/ojim.2015.53005.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] Khung, S., Budzik, J.F., Amzallag-Bellenger, E., Lambilliote, A., Soto Ares, G., Cotten, A., et al. (2013) Skeletal Involvement in Langerhans Cell Histiocytosis. Insights Imaging, 4, 569-579.
[2] Girschikofsky, M., Arico, M., Castillo, D., Chu, A., Doberauer, C., Fichter, J., et al. (2013) Management of Adult Patients with Langerhans Cell Histiocytosis: Recommendations from an Expert Panel on Behalf of Euro-Histio-Net. Orphanet Journal of Rare Diseases, 8, 72.
[3] Broadbent, V., Egeler, R.M. and Nesbit Jr., M.E. (1994) Langerhans Cell Histiocytosis: Clinical and Epidemiological Aspects. Br J Cancer Suppl, 23, S11-S6.
[4] Langerhans, P. (1868) Uber die nerven der menschlichen haut. Archiv für pathologische Anatomie und Physiologie und für klinische Medicin, 44, 325-327.
[5] Robitaille, N. and Hume, H.A. (2006) Blood Components and Fractionated Plasma Products: Preparations, Indications, and Administration. Pediatric Hematology, 3, 693-708.
[6] Andersson By, U., Tani, E., Andersson, U. and Henter, J.I. (2004) Tumor Necrosis Factor, Interleukin 11, and Leukemia Inhibitory Factor Produced by Langerhans Cells in Langerhans Cell Histiocytosis. Journal of Pediatric Hematology/Oncology, 26, 706-711.
[7] Kawakubo, Y., Kishimoto, H., Sato, Y., Yanagimoto, K., Tsuruta, T., Ogawa, Y., et al. (1999) Human Cytomegalovirus Infection in Foci of Langerhans Cell Histiocytosis. Virchows Archiv, 434, 109-115.
[8] Csire, M., Mikala, G., Jako, J., Masszi, T., Janosi, J. and Dolgos, J. (2007) Persistent Long-Term Human Herpesvirus 6 (HHV-6) Infection in a Patient with Langerhans Cell Histiocytosis. Pathology and Oncology Research, 13, 157-160.
[9] Satter, E.K. and High, W.A. (2008) Langerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte Society. Pediatric Dermatology, 25, 291-295.
[10] Beverley, P.C., Egeler, R.M., Arceci, R.J. and Pritchard, J. (2005) The Nikolas Symposia and Histiocytosis. Nature Reviews Cancer, 5, 488-494.
[11] Allen, C.E., Li, L., Peters, T.L., Leung, H.C., Yu, A., Man, T.K., et al. (2010) Cell-Specific Gene Expression in Lesions Has a Different Profile Compared with Normal Epidermal Langerhans Cells. The Journal of Immunology, 184, 4557-4567.
[12] McClain, K.L. and Allen, C.E. (2010) Inflammatory and Malignant Histiocytosis. McGraw Hill Medical, New York, 1047-1064.
[13] Ghorbel, I.B., Boukhris, I., Kchir, M.N., Abderrahmane, K., Khanfir, M., Lamloum, M., et al. (2008) Langerhans’ Cell Histiocytosis of the Dorsal Spine. A Rare Etiology of Spinal Cord Compression. La Revue de Médecine Interne, 29, 58-62.
[14] Baillet, A., Grange, L., Lafaix, P.A., Gaudin, P. and Juvin, R. (2007) Histiocytose de Langerhans et radiculalgie. Revue Du Rhumatisme, 74, 289-293.
[15] Simanski, C., Bouillon, B., Brockmann, M. and Tiling, T. (2004) The Langerhans’ cell Histiocytosis (Eosinophilic Granuloma) of the Cervical Spine: A Rare Diagnosis of Cervical Pain. Magnetic Resonance Imaging, 22,589-294.
[16] Lichtenstein, L. (1953) Histiocytosis X: Integration of Eosinophilic Granuloma of Bone, Letterer-Siwe Disease, and Schuller-Christian Disease as Related Manifestations of a Single Nosologic Entity. A.M.A. Archives of Surgery, 56, 84- 102.
[17] Hoover, K.B., Rosenthal, D.I. and Mankin, H. (2007) Langerhans Cell Histiocytosis. Skeletal Radiology, 36, 95-104.
[18] Numata, T., Okubo, Y., Uchiyama, M., Takeuchi, Y., Muro, M., et al. (2015) Adult Case of Langerhans Cell Histiocytosis. The Journal of Dermatology, 42,107-108.
[19] Tazi, A. (2006) Adult Pulmonary Langerhans’ Cell Histiocytosis. European Respiratory Journal, 27, 1272-1285.
[20] Vassallo, R., Ryu, J.H., Schroeder, D.R., Decker, P.A. and Limper, A.H. (2002) Clinical Outcomes of Pulmonary Lan- gerhans’-Cell Histiocytosis in Adults. The New England Journal of Medicine, 346, 484-490.
[21] Khung, S., Budzik, J.F., Amzallag-Bellenger, E., et al. (2013) Skeletal Involvement in Langerhans Cell Histiocytosis. Insights into Imaging, 4, 569-579.
[22] Vadivelu, S., Mangano, F.T., Miller, C.R. and Leonard, J.R. (2007) Multifocal Langerhans Cell Histiocytosis of the Pediatric Spine: A Case Report and Literature Review. Childs Nervous System, 23,127-131.
[23] Azouz, E.M., Saigal, G., Rodriguez, M.M. and Podda, A. (2005) Langerhans’ Cell Histiocytosis: Pathology, Imaging and Treatment of Skeletal Involvement. Pediatric Radiology, 35, 103-115.
[24] Sutton, D. (2011) Textbook of Radiology and Imaging. Churchill Livingstone, London, Vol. 7, 1340-1344.
[25] Yochum and Rowe’s (2005) Essential of Skeletal Radiology. Lippincott/Williams & Wilkins, Philadelphia, Vol. 3, 1533-1534.
[26] Guillerman, R.P. (2010) Imaging of Childhood Interstitial Lung Disease. Pediatric Allergy, Immunology and Pulmonology, 23, 43-68.
[27] Geissmann, F., Emile, J.F., Donadieu, J., Andry, P., Thomas, C. and Brousse, N. (1997) Aspects cliniques et physiopathologiques de l’histiocytose langerhansienne. Une proliferation clonale de cellules dendritiques de Langerhans. Hématologie, 3, 33-43.
[28] Windebank, K. and Nanduri, V. (2009) Langerhans Cell Histiocytosis. Archives of Disease in Childhood, 94, 904-908.
[29] Lin, M.W., Chang, Y.L., Lee, Y.C., Cheng, H.L., Chen, J.S. and Hsu, H.H. (2009) Pulmonary Langerhans Cell Histiocytosis. Lung, 187, 261-262.
[30] Thomas, C. and Donadieu, J. (2008) Histiocytose langerhansienne: Prise en charge thérapeutique. Archives de Pédiatrie, 15, 529-531.
[31] Sahin, F., Ozen, K.P., Vural1, F., Kamer, S., Argin, M., Anacak, Y., et al. (2007) Retrospective Analysis of Seven Patients with Adult-Onset Langerhans Cell Histiocytosis Syndromes: A Single Center experience. Turkish Journal of He- matology, 24, 75-79.
[32] Cassady, J.R. (1987) Current Role of Radiation Therapy in the Management of Histiocytosis-X. Hematology/Oncology Clinics of North America, 1, 123-129.
[33] Brady, L.W. (2008) Langerhans Cell Histiocytosis. Springer Verlag, Berlin, 397-423.
[34] Micke, O. and Seegenschmiedt, M.H. (2002) Consensus Guidelines for Radiation Therapy of Benign Diseases: A Multicenter Approach in Germany. International Journal of Radiation Oncology Biology Physics, 52, 496-513.

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