Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis characterized by multisystemic involvement. We report a case of a 51 - year - old male patient treated with pegylated IFN- α in a monthly schedule with low-dose prednisolone and achieved 34 months of control with minimal side effects.
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Jendro, M.C., Zeidler, H., Rosenthal, H., Haller, H. and Schwarz, A. (2004) Improvement of Erdheim-Chester Disease in Two Patients by Sequential Treatment with Vinblastine and Mycophenolate Mofetil. Clinical Rheumatology, 23, 52-56. https://doi.org/10.1007/s10067-003-0801-7
Yano, S., Kobayashi, K., Kato, K., Tokuda, Y., Ikeda, T. and Takeyama, H. (2007) A Case of Erdheim-Chester Disease Effectively Treated by Cyclophosphamide and Prednisolone. Nihon Kokyuki Gakkai Zasshi, 45, 43-48.
Gaspar, N., Boudou, P., Haroche, J., Wechsler, B., Van Den Neste, E., Hoang-Xuan K, Amoura, Z., Guillevin, R., Savatovski, J., Azar, N., Piette, J.C. and Leblond, V. (2006) High-Dose Chemotherapy Followed by Autologous Hematopoietic Stem Cell Transplantation for Adult Histiocytic Disorders with Central Nervous System Involvement. Haematologica, 91, 1121-1125.
Esmaeli, B., Ahmadi, A., Tang, R., Schiffman, J. and Kurzrock, R. (2001) Interferon Therapy for Orbital Infiltration Secondary to Erdheim-Chester Disease. American Journal of Ophthalmology, 132, 945-947. https://doi.org/10.1016/S0002-9394(01)01141-2
Suzuki, H.I., Hosoya, N., Miyagawa, K., Ota, S., Nakashima, H., Makita, N. and Kurokawa, M. (2010) Erdheim-Chester Disease: Multisystem Involvement and Management with Interferon-α. Leukemia Research, 34, e21-e24. https://doi.org/10.1016/j.leukres.2009.07.026
Haroche, J., Amoura, Z., Trad, S.G., Wechsler, B., Cluzel, P., Grenier, P.A. and Piette, J.C. (2006) Variability in the Efficacy of Interferon-α in Erdheim-Chester Disease by Patient and Site of Involvement: Results in Eight Patients. Arthritis & Rheumatology, 54, 3330-3336. https://doi.org/10.1002/art.22165
Hervier, B., Arnaud, L., Charlotte, F., Wechsler, B., Piette, J.C., Amoura, Z. and Haroche, J. (2012) Treatment of Erdheim-Chester Disease with Long-Term High-Dose Interferon-α. Seminars in Arthritis and Rheumatism, 41, 907-913. https://doi.org/10.1016/j.semarthrit.2011.11.004
Oneal, P.A., Kwitkowski, V., Luo, L., Shen, Y.L., Subramaniam, S., Shord, S., Goldberg, K.B., McKee, A.E., Kaminskas, E., Farrell, A. and Pazdur, R. (2018) FDA Approval Summary: Vemurafenib for the Treatment of Patients with Erdheim-Chester Disease with the BRAFV600 Mutation. The Oncologist, 23, 1520-1524. https://doi.org/10.1634/theoncologist.2018-0295
Cohen Aubart, F., Emile, J.F., Maksud, P., Galanaud, D., Cluzel, P., Benameur, N., Aumaitre, O., Amoura, Z. and Haroche, J. (2018) Efficacy of the MEK Inhibitor Cobimetinib for Wild-Type BRAF Erdheim-Chester Disease. British Journal of Haematology, 180, 150-153. https://doi.org/10.1111/bjh.14284
Arnaud, L., Gorochov, G., Charlotte, F., Lvovschi, V., Parizot, C., Larsen, M., Ghillani-Dalbin, P., Hervier, B., Kahn, J.E., Deback, C., Musset, L., Amoura, Z. and Haroche, J. (2011) Systemic Perturbation of Cytokine and Chemokine Networks in Erdheim-Chester Disease: A Single-Center Series of 37 Patients. Blood, 117, 2783-2790. https://doi.org/10.1182/blood-2010-10-313510
Diamond, E.L., Abdel-Wahab, O., Pentsova, E., Borsu, L., Chiu, A., Teruya-Feldstein, J., Hyman, D.M. and Rosenblum, M. (2013) Detection of an NRAS Mutation in Erdheim-Chester Disease. Blood, 122, 1089-1091. https://doi.org/10.1182/blood-2013-02-482984
Girschikofsky, M., Arico, M., Castillo, D., Chu, A., Doberauer, C., Fichter, J., Haroche, J., Kaltsas, G.A., Makras, P., Marzano, A.V., de Menthon, M., Micke, O., Passoni, E., Seegenschmiedt, H.M., Tazi, A. and McClain, K.L. (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, Article No. 72. https://doi.org/10.1186/1750-1172-8-72
Myra, C., Sloper, L., Tighe, P.J., McIntosh, R.S., Stevens, S.E., Gregson, R.H., Sokal, M., Haynes, A.P. and Powell, R.J. (2004) Treatment of Erdheim-Chester Disease with Cladribine: A Rational Approach. British Journal of Ophthalmology, 88, 844-847. https://doi.org/10.1136/bjo.2003.035584
Janku, F., Amin, H.M., Yang, D., Garrido-Laguna, I., Trent, J.C. and Kurzrock, R. (2010) Response of Histiocytoses to Imatinib Mesylate: Fire to Ashes. Journal of Clinical Oncology, 28, e633-e636. https://doi.org/10.1200/JCO.2010.29.9073
Dagna, L., Corti, A., Langheim S., Guglielmi, B., De Cobelli, F., Doglioni, C., Fragasso, G., Sabbadini, M.G. and Ferrarini, M. (2012) Tumor Necrosis Factor α as a Master Regulator of Inflammation in Erdheim-Chester Disease: Rationale for the Treatment of Patients with Infliximab. Journal of Clinical Oncology, 30, e286-e290. https://doi.org/10.1200/JCO.2012.41.9911