Erdheim-Chester Disease: Characteristics and Management |
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| Institution: | 1. Hematology-Oncology, Banner MD Anderson Cancer Center, Gilbert, AZ;2. Department of Investigational Cancer Therapeutics (Phase I Clinical Trials Program), University of Texas MD Anderson Cancer Center, Houston;3. Division of Dermatology, University of California, San Diego, San Diego;4. Center for Personalized Cancer Therapy, and Division of Hematology and Oncology, University of California San Diego Moores Cancer Center, San Diego;1. Vitreous Retina Macula Consultants of New York, New York, New York;2. LuEsther T. Mertz Retinal Research Center, Manhattan, Eye, Ear and Throat Hospital, New York, New York;3. Singapore National Eye Center/Singapore Eye Research Institute, Singapore;4. The Rotterdam Eye Hospital, Rotterdam, Netherlands;5. Retina Center of Hawaii, Honolulu, Hawaii;6. Memorial Sloan Kettering Cancer Center, New York, New York;7. Department of Pathology, Section Ophthalmic Pathology, Erasmus MC University Medical Center Rotterdam, Rotterdam, Netherlands;8. Departments of Internal Medicine & Immunology, Erasmus MC University Medical Center Rotterdam, Rotterdam, Netherlands;1. Service de Médecine Interne 2, Centre National de Référence Maladies Auto Immunes Systémiques Rares, Institut E3M, Hôpital Pitié-Salpêtrière, Paris, France;2. AP-HP, Service d''Anatomocytopathologie, Hôpital Pitié-Salpêtrière, Paris, France;3. AP-HP, Service de neurologie Mazarin, Hôpital Pitié-Salpêtrière, Paris, France;4. Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA, USA;5. AP-HP, Service d''Hématologie, Hôpital Trousseau, Paris, France;6. Nephrology Unit, University Hospital, Parma, Italy;7. Leukemia Service, Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center, New York, NY, USA;8. EA4340 and Pathology Department, Ambroise Paré Hospital AP-HP and Versailles University, Boulogne, France;1. Assistance publique–Hôpitaux de Paris, hôpital Pitié-Salpêtrière, université Pierre-et-Marie-Curie Paris 6, institut E3M, centre de référence des maladies rares auto-immunes et systémiques, service de médecine interne 2, Paris, France;2. Assistance publique–Hôpitaux de Paris, hôpital Pitié-Salpêtrière, université Pierre-et-Marie-Curie Paris 6, service d’anatomopathologie, Paris, France;3. Assistance publique–Hôpitaux de Paris, hôpital Pitié-Salpêtrière, université Pierre-et-Marie-Curie Paris 6, service de médecine nucléaire, Paris, France;4. Assistance publique–Hôpitaux de Paris, hôpital Pitié-Salpêtrière, université Pierre-et-Marie-Curie Paris 6, département de radiologie, Paris, France;5. Assistance publique–Hôpitaux de Paris, hôpital Ambroise-Paré, université Saint-Quentin-en-Yvelines, service d’anatomopathologie, Boulogne, France;1. Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY;2. Leukemia Service, Human Oncology and Pathogenesis Program, Memorial Sloan-Kettering Cancer Center, New York, NY;3. Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY;4. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY;5. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY;6. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY;7. Developmental Therapeutics Center, Memorial Sloan-Kettering Cancer Center, New York, NY;8. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY |
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| Abstract: | Erdheim-Chester disease is a rare CD68+, CD1a? non-Langerhans cell histiocytosis with multiorgan involvement. The etiology of Erdheim-Chester disease is unclear; there are no known associated infectious or hereditary genetic abnormalities. However, somatic BRAF mutations have recently been identified in these patients. Historically, the literature regarding the management of Erdheim-Chester disease consisted of case reports and small case series with anecdotal therapeutic responses to agents including, but not limited to, cytotoxic chemotherapy, bone marrow transplantation, cladribine, corticosteroids, IFN-α, the BCR-ABL/KIT inhibitor imatinib mesylate, the IL-1 receptor antagonist anakinra, the TNF-inhibitor infliximab, and recently the BRAF inhibitor vemurafenib. We performed a search of the literature using PubMed with the terms Erdheim Chester disease, without date limitations, including case reports, case series, original articles, and previous review articles. In the absence of large-scale studies, experience-based management prevails. The present review details our approach to the management of patients with Erdheim-Chester disease. |
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| Keywords: | CNS"} {"#name":"keyword" "$":{"id":"kwrd0015"} "$$":[{"#name":"text" "_":"central nervous system ECD"} {"#name":"keyword" "$":{"id":"kwrd0025"} "$$":[{"#name":"text" "_":"Erdheim-Chester disease PDGF"} {"#name":"keyword" "$":{"id":"kwrd0035"} "$$":[{"#name":"text" "_":"platelet-derived growth factor |
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