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ECD Literature Search (Found with This List Is an Attempt at Capturing Abstracts for Published Papers Regarding Erdheim-Chester Disease

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ECD Literature Search (Found with This List Is an Attempt at Capturing Abstracts for Published Papers Regarding Erdheim-Chester Disease ECD Literature Search (found with www.PubMed.gov) This list is an attempt at capturing abstracts for published papers regarding Erdheim-Chester Disease. It is meant for awareness purposes only. It is updated periodically. The last update date appears at the bottom of each page. (Where no PMID is noted, the article was not found on the www.pubmed.gov website.) Publ Publication Title Author(s) Author Contact Editted Abstract PMID Date 2011 Blood CNS involvement Arnaud L, Dr Julien Haroche Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis, 21239701 Jan 14 and treatment with Hervier B, Néel Service de Médecine with non-codified therapeutic management and high-mortality. No treatment has interferon-alpha are A, Hamidou MA, Interne 2. yet been shown to improve survival in these patients. We conducted a multicenter independent Kahn JE, Groupe Hospitalier Pitié- prospective observational cohort study to assess whether extraskeletal prognostic factors Wechsler B, Salpêtrière, manifestations and interferon-alpha treatment would influence survival in a large in Erdheim-Chester Pérez-Pastor G, 47-83 bd de l’Hôpital, cohort of ECD patients. To achieve this goal, we thoroughly analyzed the clinical disease: a Blomberg B, 75013, Paris, France. presentation of 53 patients with biopsy-proven ECD, and performed a survival multicenter survival Fuzibet JG, Phone number: (33) 1 analysis using Cox proportional hazard model. Fifty-three patients (39 men and 14 analysis of 53 Dubourguet F, 42 17 80 40 women) with biopsy-proven ECD were followed-up between Nov. 1981 and Nov. patients Marinho A, Fax number: (33) 1 42 2010. Forty-six patients (87%) received interferon-alpha and/or PEGylated Magnette C, 17 80 44 interferon-alpha. Multivariate survival analysis using Cox proportional hazard model Noel V, Pavic M, Email : revealed that CNS involvement was an independent predictor of death (Hazard Casper J, [email protected] Ratio, HR: 2.51, CI95%: 1.28-5.52; p=0.006) in our cohort. Conversely, treatment Beucher AB, .fr with interferon-alpha was identified as an independent predictor of survival (HR: Costedoat- 0.32, CI95%: 0.14-0.70; p=0.006). Although definitive confirmation would require a Chalumeau N, randomized controlled trial, these results suggest that interferon-alpha improves Aaron L, survival in ECD patients. This may be seen as a significant advance, as it is the Salvatierra J, first time a treatment is shown to improve survival in this multisystemic disease with Graux C, high mortality. Cacoub P, Delcey V, Dechant C, Bindi P, Herbaut C, Graziani G, Amoura Z, Haroche J. 2011 Blood Systemic Arnaud L, Departments of Internal Immunopathogenesis of Erdheim-Chester disease (ECD), a rare non-Langerhans 21205927 Jan 4 perturbation of Gorochov G, Medicine, Pathology, cell histiocytosis, is poorly known. In previous studies, various cytokines were cytokine and Charlotte F, Immunochemistry & detected in ECD lesions, presumably orchestrating lesional histiocyte recruitment. chemokine Lvovschi V, Virology, Hopital Pitie- Since ECD lesions are frequently associated with systemic symptoms, we networks in Parizot C, Salpetriere, AP-HP, postulated that underlying global immune perturbations might also be revealed. We Erdheim-Chester Larsen M, Paris, France quantitatively analyzed 23 cytokines in serum samples obtained from a large disease: a single- Ghillani-Dalbin single-center cohort of 37 ECD patients, and studied the impact of treatment over center series of 37 P, Hervier B, cytokine production. Interleukin (IL)-6, IL-12, interferon-alpha (IFN-α) and monocyte patients. Kahn JE, chemotactic protein-1 (MCP-1) levels were significantly higher in untreated patients Deback C, than in controls, while interferon-γ inducible protein-10 (IP-10), IL-12, MCP-1, IL-1 Musset L, receptor antagonist (IL1-RA) were found significantly increased in interferon-alpha Amoura Z, treated patients. A biomathematical approach was used to rationalize Haroche J. multiparameter data, in order to generate new hypotheses and identify global control pathways. Interestingly, cytokine profiles proved to be particularly stable at the individual level, and an "ECD signature" further distinguished patients from controls, based on their production of IFN-α, IL-12, MCP-1, IL-4 & IL-7. Altogether, our data underline the systemic immune Th-1 oriented perturbation associated with this condition, and provide clues for the choice of more focused therapeutic agents in this rare disease with non-codified therapeutic management. Pg 1 of 79 1/21/2011 Publ Publication Title Author(s) Author Contact Editted Abstract PMID Date 2010 Ann Dermatol Erdheim-chester Kim MS, Kim Mi-Woo Lee, M.D., Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis of 21165216 Nov disease. CH, Choi SJ, Department of unknown etiology, characterized by multi-organ involvement. ECD is usually Won CH, Chang Dermatology, Asan diagnosed on the basis of characteristic radiologic and histopathological findings. SE, Lee MW, Medical Center, Lesions may be skeletal and/or extraskeletal in location, and may include the skin, Choi JH, Moon University of Ulsan lung, heart, and central nervous system. We describe here a 68-year-old man with KC. College of Medicine, 86 multiple yellowish plaques and a pinkish nodule on his face and scalp. He had Asanbyeonwon-gil, been previously diagnosed with diabetes insipidus, and recently complained of Songpa-gu, Seoul 138- coughing and dyspnea. Imaging studies showed multiple osteosclerotic lesions of 736, Korea. Tel: 82-2- the bones, a moderate amount of pericardial effusion, and multifocal infiltrative 3010-3460, Fax: 82-2- lesions in the perirenal space. Histopathological examination of the skin lesions 486-7831, Email: revealed dermal infiltration of foamy histiocytes with multinuclear giant cells. [email protected] Moreover, laparoscopic biopsy of the perirenal tissue revealed fibrosis with infiltrating foamy histiocytes being CD68-positive and S100-negative. Based on these findings, he was diagnosed with ECD with extraskeletal manifestations, and treated with interferon-alpha. 2010 J Cutan Pathol Erdheim-Chester Volpicelli ER, Arturo P. Saavedra, MD, Erdheim–Chester disease (ECD) is a rare, systemic, non-familial histiocytic 21143617 Dec disease presenting Doyle L, Annes PhD, Department of disorder, first described by Jakob Erdheim and William Chester in 1930. Most 12 with cutaneous JP, Murray MF, Dermatology, Brigham patients have multiple sites of involvement at presentation. The most common site involvement: a Jacobsen E, and Women's Hospital, of involvement is the long bones of the axial skeleton, which is seen almost case report and Murphy GF, Harvard Medical School, universally, followed by the nervous system, heart, lungs, orbit and literature review. Saavedra AP 221 Longwood Avenue, retroperitoneum, which are seen in up to 50% of cases.1 Cutaneous involvement is Boston, MA 02115, USA rarely a presenting symptom of ECD, with two reported cases in the English Tel: +1 617 732 4918 literature.2 The diagnosis of ECD is rarely made by skin biopsy because of the begin_of_the_skype_hig relative rarity of cutaneous involvement as a presenting feature, and also perhaps hlighting +1 617 because of the difficulty in distinguishing the histopathological appearance from 732 4918 potential mimics. The importance of distinguishing ECD from other cutaneous end_of_the_skype_highl disorders with similar pathology lies in the implications for both treatment and ighting Fax: +1 617 582 prognosis. ECD is an aggressive, often fatal disorder, with death from disease 6060 e-mail: occurring in greater than 50% of patients. [email protected] g 2010 Rheumatol Int FDG-PET in the Steňová E, 1st Department of A 62-year-old man presented with diabetes insipidus, pulmonary fibrosis, right atrial 21136259 Dec 7 Erdheim-Chester Steňo B, Internal Medicine, tumor and bilateral knee osteoarthritis with cystic lesions of distal femur and disease: its Povinec P, Comenius University in proximal tibia. Scintigraphy and histological examination of right femur bone biopsy diagnostic and Ondriaš F, Bratislava, Bratislava, revealed changes characterized for Paget's disease. Re-evaluation of the computer follow-up role. Rampalová J. Slovakia, tomography (CT) scans and histological samples revealed diffuse infiltrates of [email protected]. foamy histiocytes in the bone marrow what was consistent with Erdheim-Chester disease. Positron emission tomography/computed tomography (PET/CT) was performed to access the activity and extent of disease. 2010 Journal of Response of Filip Janku, The University of Texas No abstract included. Final statement of paper states, “LCH and ECD are rare and Nov Clinical Histiocytoses to Hesham M. M. D. Anderson Cancer often difficult to treat disorders. Our observation suggests that imatinib may be an Oncology Imatinib Amin, Dan Yang, Center, Houston, TX effective treatment option for some patients with these diseases.” Mesylate: Fire to Ignacio Garrido- Ashes Laguna, Jonathan C. Trent, and Razelle Kurzrock Pg 2 of 79 1/21/2011 Publ Publication Title Author(s) Author Contact Editted Abstract PMID Date 2010 J Neurol CP3 Progressive Marshall C, drcharlesmarshall@gma A 72-year-old woman presented with a 2-year history of slowly progressive ataxia, 20972058 Nov Neurosurg ataxia with unusual Giovannoni G il.com accompanied by 13 kg weight loss. MRI imaging of the brain revealed T2- Psychiatry radiographic weighted hyperintensities in the pons,
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