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Erdheim–Chester disease

April 11, 2024

Erdheim–Chester disease (ECD) is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis). It was declared a histiocytic neoplasm by the World Health Organization in 2016.[1] Onset typically is in middle age, although younger patients have been documented. The disease involves an infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones.[2]

Erdheim–Chester disease
Other namesErdheim–Chester syndrome or Polyostotic sclerosing histiocytosis
Chester-Erdheim disease
SpecialtyOncology 

Signs and symptoms edit

Long bone involvement is almost universal in ECD patients and is bilateral and symmetrical in nature. More than 50% of cases have some sort of extraskeletal involvement. This can include kidney, skin, brain and lung involvement, and less frequently retroorbital tissue, pituitary gland and heart involvement is observed.[3]

Bone pain is the most frequent of all symptoms associated with ECD and mainly affects the lower limbs, knees and ankles. The pain is often described as mild but permanent, and juxtaarticular in nature. Exophthalmos occurs in some patients and is usually bilateral, symmetric and painless, and in most cases it occurs several years before the final diagnosis. Recurrent pericardial effusion can be a manifestation,[4] as can morphological changes in adrenal size and infiltration.[5]

A review of 59 case studies by Veyssier-Belot et al. in 1996 reported the following symptoms in order of frequency of occurrence:[6]

Diagnosis edit

Radiologic osteosclerosis and histology are the main diagnostic features. Diagnosis can often be difficult because of the rareness of ECD as well as the need to differentiate it from LCH. A diagnosis from neurological imaging may not be definitive. The presence of symmetrical cerebellar and pontine signal changes on T2-weighted images seem to be typical of ECD, however, multiple sclerosis and metabolic diseases must also be considered in the differential diagnosis.[7] ECD is not a common cause of exophthalmos but can be diagnosed by biopsy. However, like all biopsies, this may be inconclusive.[8] Video-assisted thoracoscopic surgery may be used for diagnostic confirmation and also for therapeutic relief of recurrent pericardial fluid drainage.[9]

Histology edit

Histologically, ECD differs from Langerhans cell histiocytosis (LCH) in a number of ways. Unlike LCH, ECD does not stain positive for S-100 proteins or Group 1 CD1a glycoproteins, and electron microscopy of cell cytoplasm does not disclose Birbeck granules.[6] Tissue samples show xanthomatous or xanthogranulomatous infiltration by lipid-laden or foamy histiocytes, and are usually surrounded by fibrosis. Bone biopsy is said to offer the greatest likelihood of reaching a diagnosis. It would appear that approximately half these patients harbor point mutations of the BRAF gene at codon 600 substituting the amino acid glutamine for valine. In some, there is histiocyte proliferation, and on staining, the section is CD68+ and CD1a-.[citation needed]

Treatment edit

There are two FDA-approved targeted drugs to treat ECD.

  • Vemurafenib, an oral agent approved in 2019, targets the BRAF protein. It was approved after showing dramatic efficacy in ECD patients harboring the BRAF V600E mutation.[10][11]
  • Cobimetinib, an oral inhibitor of MEK1 and MEK2, was approved in November 2022.[12]

Other treatment options include:

Prognosis edit

Erdheim–Chester disease was previously associated with high mortality rates.[14] However, long-term survival is now more promising. Recent studies have reported that some patients receiving targeted therapies showed no disease progression. Targeted therapies using BRAF, MEK and/or other inhibitors have been dramatically efficacious.[10][13][15][16] In 2019, the Mayo Clinic published guidelines for the diagnosis and treatment of the disease, stressing the importance of genetic testing: "Recent insights into their genomic architecture demonstrating mitogen-activated protein kinase/extracellular signal-regulated kinase pathway mutations have now enabled potential treatment with targeted therapies in most patients."[17]

Epidemiology edit

Approximately 500 cases had been reported in the literature as of 2014.[18] ECD affects predominantly adults, with a mean age of 53 years.[6]

History edit

The first case of ECD was reported by the American pathologist William Chester in 1930, during his visit to the Austrian pathologist Jakob Erdheim in Vienna.[19]

Society and culture edit

The Erdheim–Chester Disease Global Alliance is a support and advocacy group with the goal of raising awareness of and promoting research into ECD.[20][21] ECD families and patients are also supported by the Histiocytosis Association, Inc.[21][22]

Media edit

In the TV show House, season 2 episode 17, "All In", the final diagnosis of a 6-year-old boy who presents with bloody diarrhea and ataxia is Erdheim–Chester disease.[23]

References edit

  1. ^ "Erdheim-Chester Disease Declared a Histiocytic Neoplasm" (PDF). 18 May 2016. Retrieved 2018-07-18 – via erdheim-chester.org.
  2. ^ "Erdheim–Chester disease". Medical Subject Headings. United States National Library of Medicine. 8 July 2008. Retrieved 19 June 2008.
  3. ^ "Erdheim-Chester Disease". Histiocytosis Association. Retrieved 2017-12-21.
  4. ^ Lutz SZ, Schmalzing M, Vogel-Claussen J, Adam P, May AE (September 2011). "[Recurrent pericardial effusion as first manifestation of Erdheim-Chester disease]" [Recurrent pericardial effusion as first manifestation of Erdheim-Chester disease]. Deutsche Medizinische Wochenschrift (in German). 136 (39): 1952–1956. doi:10.1055/s-0031-1286368. PMID 21935854.
  5. ^ Haroche J, Amoura Z, Touraine P, Seilhean D, Graef C, Birmelé B, et al. (June 2007). "Bilateral adrenal infiltration in Erdheim-Chester disease. Report of seven cases and literature review". The Journal of Clinical Endocrinology and Metabolism. 92 (6): 2007–2012. doi:10.1210/jc.2006-2018. PMID 17405844.
  6. ^ a b c Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, Wechsler J, Brun B, Remy M, et al. (May 1996). "Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases". Medicine. 75 (3): 157–169. doi:10.1097/00005792-199605000-00005. PMID 8965684. S2CID 32150913.
  7. ^ Weidauer S, von Stuckrad-Barre S, Dettmann E, Zanella FE, Lanfermann H (April 2003). "Cerebral Erdheim-Chester disease: case report and review of the literature". Neuroradiology. 45 (4): 241–245. doi:10.1007/s00234-003-0950-z. PMID 12687308. S2CID 9513277.
  8. ^ a b "Erdheim Chester Disease". M. D. Anderson Cancer Center. Retrieved 2007-08-26.
  9. ^ Egan A, Sorajja D, Jaroszewski D, Mookadam F (2012). "Erdheim-Chester disease: The role of video-assisted thoracoscopic surgery in diagnosing and treating cardiac involvement". International Journal of Surgery Case Reports. 3 (3): 107–110. doi:10.1016/j.ijscr.2011.12.001. PMC 3267285. PMID 22288060.
  10. ^ a b Haroche J, Cohen-Aubart F, Emile JF, Arnaud L, Maksud P, Charlotte F, et al. (February 2013). "Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation". Blood. 121 (9): 1495–1500. doi:10.1182/blood-2012-07-446286. PMID 23258922.
  11. ^ "FDA Approves First Treatment for Erdheim-Chester Disease". Pharmacy Practice News. 6 November 2017.
  12. ^ Rosa K (2 November 2022). "FDA Approves Cobimetinib for Histiocytic Neoplasms". OncLive. Retrieved 2022-11-19.
  13. ^ a b Abeykoon JP, Lasho TL, Dasari S, Rech KL, Ranatunga WK, Manske MK, et al. (March 2022). "Sustained, complete response to pexidartinib in a patient with CSF1R-mutated Erdheim-Chester disease". American Journal of Hematology. 97 (3): 293–302. doi:10.1002/ajh.26441. PMC 9536810. PMID 34978715.
  14. ^ Myra C, Sloper L, Tighe PJ, McIntosh RS, Stevens SE, Gregson RH, et al. (June 2004). "Treatment of Erdheim-Chester disease with cladribine: a rational approach". The British Journal of Ophthalmology. 88 (6): 844–847. doi:10.1136/bjo.2003.035584. PMC 1772168. PMID 15148234.
  15. ^ Aziz SN, Proano L, Cruz C, Tenemaza MG, Monteros G, Hassen G, et al. (June 2022). "Vemurafenib in the Treatment of Erdheim Chester Disease: A Systematic Review". Cureus. 14 (6): e25935. doi:10.7759/cureus.25935. PMC 9282605. PMID 35844342.
  16. ^ The ASCO Post Staff (2 November 2022). "FDA Approves Oral MEK Inhibitor Cobimetinib for Histiocytic Neoplasms". The ASCO Post. Retrieved 2022-11-19.
  17. ^ Goyal G, Young JR, Koster MJ, Tobin WO, Vassallo R, Ryu JH, et al. (October 2019). "The Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease". Mayo Clinic Proceedings. 94 (10): 2054–2071. doi:10.1016/j.mayocp.2019.02.023. PMID 31472931. S2CID 201713697.
  18. ^ Haroche J, Arnaud L, Cohen-Aubart F, Hervier B, Charlotte F, Emile JF, Amoura Z (April 2014). "Erdheim-Chester disease". Current Rheumatology Reports. 16 (4): 412. doi:10.1007/s11926-014-0412-0. PMID 24532298.
  19. ^ Chester W (1930). "Über Lipoidgranulomatose". Virchows Archiv für Pathologische Anatomie und Physiologie und für Klinische Medizin. 279 (2): 561–602. doi:10.1007/BF01942684. S2CID 27359311.
  20. ^ "Erdheim–Chester Disease". ECD Global Alliance. Retrieved 2009-05-08.
  21. ^ a b "Erdheim Chester disease". NORD (National Organization for Rare Disorders). Retrieved 2016-03-01.
  22. ^ "What Do I Do Now? - Erdheim-Chester Disease". Histiocytosis Association. Retrieved 2016-03-01.
  23. ^ "Internet Movie Database". IMDB. Retrieved 27 October 2021.

Further reading edit

  • Aouba A, Georgin-Lavialle S, Pagnoux C, Martin Silva N, Renand A, Galateau-Salle F, et al. (November 2010). "Rationale and efficacy of interleukin-1 targeting in Erdheim-Chester disease". Blood. 116 (20): 4070–4076. doi:10.1182/blood-2010-04-279240. PMID 20724540.
  • Arnaud L, Malek Z, Archambaud F, Kas A, Toledano D, Drier A, et al. (October 2009). "18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim-Chester disease". Arthritis and Rheumatism. 60 (10): 3128–3138. doi:10.1002/art.24848. PMID 19790052.
  • Arnaud L, Pierre I, Beigelman-Aubry C, Capron F, Brun AL, Rigolet A, et al. (November 2010). "Pulmonary involvement in Erdheim-Chester disease: a single-center study of thirty-four patients and a review of the literature". Arthritis and Rheumatism. 62 (11): 3504–3512. doi:10.1002/art.27672. PMID 20662053.
  • Boissel N, Wechsler B, Leblond V (November 2001). "Treatment of refractory Erdheim-Chester disease with double autologous hematopoietic stem-cell transplantation". Annals of Internal Medicine. 135 (9): 844–845. doi:10.7326/0003-4819-135-9-200111060-00027. PMID 11694122.
  • Braiteh F, Boxrud C, Esmaeli B, Kurzrock R (November 2005). "Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha". Blood. 106 (9): 2992–2994. doi:10.1182/blood-2005-06-2238. PMID 16020507.
  • Brun AL, Touitou-Gottenberg D, Haroche J, Toledano D, Cluzel P, Beigelman-Aubry C, et al. (November 2010). "Erdheim-Chester disease: CT findings of thoracic involvement". European Radiology. 20 (11): 2579–2587. doi:10.1007/s00330-010-1830-7. PMID 20563815. S2CID 5775587.
  • de Abreu MR, Castro MO, Chung C, Trudell D, Biswal S, Wesselly M, Resnick D (2009). "Erdheim-Chester disease: case report with unique postmortem magnetic resonance imaging, high-resolution radiography, and pathologic correlation". Clinical Imaging. 33 (2): 150–153. doi:10.1016/j.clinimag.2008.09.009. PMID 19237062.
  • Drier A, Haroche J, Savatovsky J, Godenèche G, Dormont D, Chiras J, et al. (May 2010). "Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings". Radiology. 255 (2): 586–594. doi:10.1148/radiol.10090320. PMID 20413768.
  • Haroche J, Amoura Z, Dion E, Wechsler B, Costedoat-Chalumeau N, Cacoub P, et al. (November 2004). "Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of 6 new cases and a literature review". Medicine. 83 (6): 371–392. doi:10.1097/01.md.0000145368.17934.91. PMID 15525849. S2CID 1426013.
  • Haroche J, Cluzel P, Toledano D, Montalescot G, Touitou D, Grenier PA, et al. (June 2009). "Images in cardiovascular medicine. Cardiac involvement in Erdheim-Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients". Circulation. 119 (25): e597–e598. doi:10.1161/CIRCULATIONAHA.108.825075. PMID 19564564.
  • Haroche J, Amoura Z, Trad SG, Wechsler B, Cluzel P, Grenier PA, Piette JC (October 2006). "Variability in the efficacy of interferon-alpha in Erdheim-Chester disease by patient and site of involvement: results in eight patients". Arthritis and Rheumatism. 54 (10): 3330–3336. doi:10.1002/art.22165. PMID 17009306.
  • Haroche J, Amoura Z, Charlotte F, Salvatierra J, Wechsler B, Graux C, et al. (June 2008). "Imatinib mesylate for platelet-derived growth factor receptor-beta-positive Erdheim-Chester histiocytosis". Blood. 111 (11): 5413–5415. doi:10.1182/blood-2008-03-148304. PMID 18502845.
  • Janku F, Amin HM, Yang D, Garrido-Laguna I, Trent JC, Kurzrock R (November 2010). "Response of histiocytoses to imatinib mesylate: fire to ashes". Journal of Clinical Oncology. 28 (31): e633–e636. doi:10.1200/JCO.2010.29.9073. PMID 20733125.
  • Lachenal F, Cotton F, Desmurs-Clavel H, Haroche J, Taillia H, Magy N, et al. (October 2006). "Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature". Journal of Neurology. 253 (10): 1267–1277. doi:10.1007/s00415-006-0160-9. PMID 17063320. S2CID 27976718.
  • Mossetti G, Rendina D, Numis FG, Somma P, Postiglione L, Nunziata V (2003). "Biochemical markers of bone turnover, serum levels of interleukin-6/interleukin-6 soluble receptor and bisphosphonate treatment in Erdheim-Chester disease". Clinical and Experimental Rheumatology. 21 (2): 232–236. PMID 12747282.
  • Perlat A, Decaux O, Sébillot M, Grosbois B, Desfourneaux V, Meadeb J (May 2009). "Erdheim-Chester disease with predominant mesenteric localization: lack of efficacy of interferon alpha". Joint Bone Spine. 76 (3): 315–317. doi:10.1016/j.jbspin.2008.09.013. PMID 19119043.

External links edit

April 11, 2024
erdheim, chester, disease, extremely, rare, disease, characterized, abnormal, multiplication, specific, type, white, blood, cells, called, histiocytes, tissue, macrophages, technically, this, disease, termed, langerhans, cell, histiocytosis, declared, histiocy. Erdheim Chester disease ECD is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes or tissue macrophages technically this disease is termed a non Langerhans cell histiocytosis It was declared a histiocytic neoplasm by the World Health Organization in 2016 1 Onset typically is in middle age although younger patients have been documented The disease involves an infiltration of lipid laden macrophages multinucleated giant cells an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow and a generalized sclerosis of the long bones 2 Erdheim Chester diseaseOther namesErdheim Chester syndrome or Polyostotic sclerosing histiocytosisChester Erdheim diseaseSpecialtyOncology Contents 1 Signs and symptoms 2 Diagnosis 2 1 Histology 3 Treatment 4 Prognosis 5 Epidemiology 6 History 7 Society and culture 7 1 Media 8 References 9 Further reading 10 External linksSigns and symptoms editLong bone involvement is almost universal in ECD patients and is bilateral and symmetrical in nature More than 50 of cases have some sort of extraskeletal involvement This can include kidney skin brain and lung involvement and less frequently retroorbital tissue pituitary gland and heart involvement is observed 3 Bone pain is the most frequent of all symptoms associated with ECD and mainly affects the lower limbs knees and ankles The pain is often described as mild but permanent and juxtaarticular in nature Exophthalmos occurs in some patients and is usually bilateral symmetric and painless and in most cases it occurs several years before the final diagnosis Recurrent pericardial effusion can be a manifestation 4 as can morphological changes in adrenal size and infiltration 5 A review of 59 case studies by Veyssier Belot et al in 1996 reported the following symptoms in order of frequency of occurrence 6 Bone pain Retroperitoneal fibrosis Diabetes insipidus Exophthalmos Xanthomas Neurological and central nervous system involvement Dyspnea caused by interlobular septal and pleural thickening Kidney failure Hypopituitarism Liver failureDiagnosis editRadiologic osteosclerosis and histology are the main diagnostic features Diagnosis can often be difficult because of the rareness of ECD as well as the need to differentiate it from LCH A diagnosis from neurological imaging may not be definitive The presence of symmetrical cerebellar and pontine signal changes on T2 weighted images seem to be typical of ECD however multiple sclerosis and metabolic diseases must also be considered in the differential diagnosis 7 ECD is not a common cause of exophthalmos but can be diagnosed by biopsy However like all biopsies this may be inconclusive 8 Video assisted thoracoscopic surgery may be used for diagnostic confirmation and also for therapeutic relief of recurrent pericardial fluid drainage 9 Histology edit Histologically ECD differs from Langerhans cell histiocytosis LCH in a number of ways Unlike LCH ECD does not stain positive for S 100 proteins or Group 1 CD1a glycoproteins and electron microscopy of cell cytoplasm does not disclose Birbeck granules 6 Tissue samples show xanthomatous or xanthogranulomatous infiltration by lipid laden or foamy histiocytes and are usually surrounded by fibrosis Bone biopsy is said to offer the greatest likelihood of reaching a diagnosis It would appear that approximately half these patients harbor point mutations of the BRAF gene at codon 600 substituting the amino acid glutamine for valine In some there is histiocyte proliferation and on staining the section is CD68 and CD1a citation needed Treatment editThere are two FDA approved targeted drugs to treat ECD Vemurafenib an oral agent approved in 2019 targets the BRAF protein It was approved after showing dramatic efficacy in ECD patients harboring the BRAF V600E mutation 10 11 Cobimetinib an oral inhibitor of MEK1 and MEK2 was approved in November 2022 12 Other treatment options include Interferon a 8 High dose corticosteroid therapy Chemotherapy Pexidartinib a drug that targets a mutation in the CSF1R pathway and has shown sustained complete response in limited use 13 Radiation therapy Surgical debulking CiclosporinPrognosis editErdheim Chester disease was previously associated with high mortality rates 14 However long term survival is now more promising Recent studies have reported that some patients receiving targeted therapies showed no disease progression Targeted therapies using BRAF MEK and or other inhibitors have been dramatically efficacious 10 13 15 16 In 2019 the Mayo Clinic published guidelines for the diagnosis and treatment of the disease stressing the importance of genetic testing Recent insights into their genomic architecture demonstrating mitogen activated protein kinase extracellular signal regulated kinase pathway mutations have now enabled potential treatment with targeted therapies in most patients 17 Epidemiology editApproximately 500 cases had been reported in the literature as of 2014 18 ECD affects predominantly adults with a mean age of 53 years 6 History editThe first case of ECD was reported by the American pathologist William Chester in 1930 during his visit to the Austrian pathologist Jakob Erdheim in Vienna 19 Society and culture editThe Erdheim Chester Disease Global Alliance is a support and advocacy group with the goal of raising awareness of and promoting research into ECD 20 21 ECD families and patients are also supported by the Histiocytosis Association Inc 21 22 Media edit In the TV show House season 2 episode 17 All In the final diagnosis of a 6 year old boy who presents with bloody diarrhea and ataxia is Erdheim Chester disease 23 References edit Erdheim Chester Disease Declared a Histiocytic Neoplasm PDF 18 May 2016 Retrieved 2018 07 18 via erdheim chester org Erdheim Chester disease Medical Subject Headings United States National Library of Medicine 8 July 2008 Retrieved 19 June 2008 Erdheim Chester Disease Histiocytosis Association Retrieved 2017 12 21 Lutz SZ Schmalzing M Vogel Claussen J Adam P May AE September 2011 Recurrent pericardial effusion as first manifestation of Erdheim Chester disease Recurrent pericardial effusion as first manifestation of Erdheim Chester disease Deutsche Medizinische Wochenschrift in German 136 39 1952 1956 doi 10 1055 s 0031 1286368 PMID 21935854 Haroche J Amoura Z Touraine P Seilhean D Graef C Birmele B et al June 2007 Bilateral adrenal infiltration in Erdheim Chester disease Report of seven cases and literature review The Journal of Clinical Endocrinology and Metabolism 92 6 2007 2012 doi 10 1210 jc 2006 2018 PMID 17405844 a b c Veyssier Belot C Cacoub P Caparros Lefebvre D Wechsler J Brun B Remy M et al May 1996 Erdheim Chester disease Clinical and radiologic characteristics of 59 cases Medicine 75 3 157 169 doi 10 1097 00005792 199605000 00005 PMID 8965684 S2CID 32150913 Weidauer S von Stuckrad Barre S Dettmann E Zanella FE Lanfermann H April 2003 Cerebral Erdheim Chester disease case report and review of the literature Neuroradiology 45 4 241 245 doi 10 1007 s00234 003 0950 z PMID 12687308 S2CID 9513277 a b Erdheim Chester Disease M D Anderson Cancer Center Retrieved 2007 08 26 Egan A Sorajja D Jaroszewski D Mookadam F 2012 Erdheim Chester disease The role of video assisted thoracoscopic surgery in diagnosing and treating cardiac involvement International Journal of Surgery Case Reports 3 3 107 110 doi 10 1016 j ijscr 2011 12 001 PMC 3267285 PMID 22288060 a b Haroche J Cohen Aubart F Emile JF Arnaud L Maksud P Charlotte F et al February 2013 Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation Blood 121 9 1495 1500 doi 10 1182 blood 2012 07 446286 PMID 23258922 FDA Approves First Treatment for Erdheim Chester Disease Pharmacy Practice News 6 November 2017 Rosa K 2 November 2022 FDA Approves Cobimetinib for Histiocytic Neoplasms OncLive Retrieved 2022 11 19 a b Abeykoon JP Lasho TL Dasari S Rech KL Ranatunga WK Manske MK et al March 2022 Sustained complete response to pexidartinib in a patient with CSF1R mutated Erdheim Chester disease American Journal of Hematology 97 3 293 302 doi 10 1002 ajh 26441 PMC 9536810 PMID 34978715 Myra C Sloper L Tighe PJ McIntosh RS Stevens SE Gregson RH et al June 2004 Treatment of Erdheim Chester disease with cladribine a rational approach The British Journal of Ophthalmology 88 6 844 847 doi 10 1136 bjo 2003 035584 PMC 1772168 PMID 15148234 Aziz SN Proano L Cruz C Tenemaza MG Monteros G Hassen G et al June 2022 Vemurafenib in the Treatment of Erdheim Chester Disease A Systematic Review Cureus 14 6 e25935 doi 10 7759 cureus 25935 PMC 9282605 PMID 35844342 The ASCO Post Staff 2 November 2022 FDA Approves Oral MEK Inhibitor Cobimetinib for Histiocytic Neoplasms The ASCO Post Retrieved 2022 11 19 Goyal G Young JR Koster MJ Tobin WO Vassallo R Ryu JH et al October 2019 The Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms Erdheim Chester Disease Langerhans Cell Histiocytosis and Rosai Dorfman Disease Mayo Clinic Proceedings 94 10 2054 2071 doi 10 1016 j mayocp 2019 02 023 PMID 31472931 S2CID 201713697 Haroche J Arnaud L Cohen Aubart F Hervier B Charlotte F Emile JF Amoura Z April 2014 Erdheim Chester disease Current Rheumatology Reports 16 4 412 doi 10 1007 s11926 014 0412 0 PMID 24532298 Chester W 1930 Uber Lipoidgranulomatose Virchows Archiv fur Pathologische Anatomie und Physiologie und fur Klinische Medizin 279 2 561 602 doi 10 1007 BF01942684 S2CID 27359311 Erdheim Chester Disease ECD Global Alliance Retrieved 2009 05 08 a b Erdheim Chester disease NORD National Organization for Rare Disorders Retrieved 2016 03 01 What Do I Do Now Erdheim Chester Disease Histiocytosis Association Retrieved 2016 03 01 Internet Movie Database IMDB Retrieved 27 October 2021 Further reading editAouba A Georgin Lavialle S Pagnoux C Martin Silva N Renand A Galateau Salle F et al November 2010 Rationale and efficacy of interleukin 1 targeting in Erdheim Chester disease Blood 116 20 4070 4076 doi 10 1182 blood 2010 04 279240 PMID 20724540 Arnaud L Malek Z Archambaud F Kas A Toledano D Drier A et al October 2009 18F fluorodeoxyglucose positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim Chester disease Arthritis and Rheumatism 60 10 3128 3138 doi 10 1002 art 24848 PMID 19790052 Arnaud L Pierre I Beigelman Aubry C Capron F Brun AL Rigolet A et al November 2010 Pulmonary involvement in Erdheim Chester disease a single center study of thirty four patients and a review of the literature Arthritis and Rheumatism 62 11 3504 3512 doi 10 1002 art 27672 PMID 20662053 Boissel N Wechsler B Leblond V November 2001 Treatment of refractory Erdheim Chester disease with double autologous hematopoietic stem cell transplantation Annals of Internal Medicine 135 9 844 845 doi 10 7326 0003 4819 135 9 200111060 00027 PMID 11694122 Braiteh F Boxrud C Esmaeli B Kurzrock R November 2005 Successful treatment of Erdheim Chester disease a non Langerhans cell histiocytosis with interferon alpha Blood 106 9 2992 2994 doi 10 1182 blood 2005 06 2238 PMID 16020507 Brun AL Touitou Gottenberg D Haroche J Toledano D Cluzel P Beigelman Aubry C et al November 2010 Erdheim Chester disease CT findings of thoracic involvement European Radiology 20 11 2579 2587 doi 10 1007 s00330 010 1830 7 PMID 20563815 S2CID 5775587 de Abreu MR Castro MO Chung C Trudell D Biswal S Wesselly M Resnick D 2009 Erdheim Chester disease case report with unique postmortem magnetic resonance imaging high resolution radiography and pathologic correlation Clinical Imaging 33 2 150 153 doi 10 1016 j clinimag 2008 09 009 PMID 19237062 Drier A Haroche J Savatovsky J Godeneche G Dormont D Chiras J et al May 2010 Cerebral facial and orbital involvement in Erdheim Chester disease CT and MR imaging findings Radiology 255 2 586 594 doi 10 1148 radiol 10090320 PMID 20413768 Haroche J Amoura Z Dion E Wechsler B Costedoat Chalumeau N Cacoub P et al November 2004 Cardiovascular involvement an overlooked feature of Erdheim Chester disease report of 6 new cases and a literature review Medicine 83 6 371 392 doi 10 1097 01 md 0000145368 17934 91 PMID 15525849 S2CID 1426013 Haroche J Cluzel P Toledano D Montalescot G Touitou D Grenier PA et al June 2009 Images in cardiovascular medicine Cardiac involvement in Erdheim Chester disease magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients Circulation 119 25 e597 e598 doi 10 1161 CIRCULATIONAHA 108 825075 PMID 19564564 Haroche J Amoura Z Trad SG Wechsler B Cluzel P Grenier PA Piette JC October 2006 Variability in the efficacy of interferon alpha in Erdheim Chester disease by patient and site of involvement results in eight patients Arthritis and Rheumatism 54 10 3330 3336 doi 10 1002 art 22165 PMID 17009306 Haroche J Amoura Z Charlotte F Salvatierra J Wechsler B Graux C et al June 2008 Imatinib mesylate for platelet derived growth factor receptor beta positive Erdheim Chester histiocytosis Blood 111 11 5413 5415 doi 10 1182 blood 2008 03 148304 PMID 18502845 Janku F Amin HM Yang D Garrido Laguna I Trent JC Kurzrock R November 2010 Response of histiocytoses to imatinib mesylate fire to ashes Journal of Clinical Oncology 28 31 e633 e636 doi 10 1200 JCO 2010 29 9073 PMID 20733125 Lachenal F Cotton F Desmurs Clavel H Haroche J Taillia H Magy N et al October 2006 Neurological manifestations and neuroradiological presentation of Erdheim Chester disease report of 6 cases and systematic review of the literature Journal of Neurology 253 10 1267 1277 doi 10 1007 s00415 006 0160 9 PMID 17063320 S2CID 27976718 Mossetti G Rendina D Numis FG Somma P Postiglione L Nunziata V 2003 Biochemical markers of bone turnover serum levels of interleukin 6 interleukin 6 soluble receptor and bisphosphonate treatment in Erdheim Chester disease Clinical and Experimental Rheumatology 21 2 232 236 PMID 12747282 Perlat A Decaux O Sebillot M Grosbois B Desfourneaux V Meadeb J May 2009 Erdheim Chester disease with predominant mesenteric localization lack of efficacy of interferon alpha Joint Bone Spine 76 3 315 317 doi 10 1016 j jbspin 2008 09 013 PMID 19119043 External links edit01067 at CHORUS Retrieved from https en wikipedia org w index php title Erdheim Chester disease amp oldid 1194024179, wikipedia, wiki, book, books, library,

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