{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T17:17:40Z","timestamp":1781630260795,"version":"3.54.5"},"reference-count":25,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2008,1,8]],"date-time":"2008-01-08T00:00:00Z","timestamp":1199750400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2008,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Hemophagocytic lymphohistiocytosis (HLH) is a complex inflammatory disease with multiple diagnostic and therapeutic pitfalls. The congenital form, referred to as familial hemophagocytic lymphohistiocytosis (FHL), is often associated with cerebromeningeal involvement, whereas neurological complications are not characteristic of the adult form of secondary HLH (sHLH). Here we report the case of a 20\u2010year\u2010old woman with adult\u2010onset Still's disease (AOSD), retinal microangiopathy and concurrent macrophage activation syndrome (MAS), in the context of sHLH. Following treatment with etanercept, ibuprofen, methylprednisolon, and phenylbutazone for 3 weeks, MAS deteriorated and fatal cerebral edema occurred within only 24 h. The clinical signs and neuropathological findings are discussed with special emphasis on possible relationships between the aggravation of MAS and therapeutic interventions for AOSD. In conclusion, even the slightest sign of mental decline in a patient with AOSD must be considered central nervous system MAS which can be rapidly fatal. Am. J. Hematol., 2008. \u00a9 2008 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.21084","type":"journal-article","created":{"date-parts":[[2008,1,8]],"date-time":"2008-01-08T22:46:47Z","timestamp":1199832407000},"page":"424-427","source":"Crossref","is-referenced-by-count":27,"title":["Retinal microangiopathy and rapidly fatal cerebral edema in a patient with adult\u2010onset Still's disease and concurrent macrophage activation syndrome"],"prefix":"10.1002","volume":"83","author":[{"given":"Sara","family":"Gianella","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominik J.","family":"Schaer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Urs","family":"Schwarz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Kurrer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank L.","family":"Heppner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg","family":"Fehr","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg D.","family":"Seebach","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,1,8]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0889-8588(05)70520-7"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00443367"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21039"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-08-2997"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-911X(199709)29:3<157::AID-MPO1>3.0.CO;2-C"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1097\/00002281-200309000-00011"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(92)91008-V"},{"key":"e_1_2_1_9_2","first-page":"424","article-title":"Preliminary criteria for classification of adult Still's disease","volume":"19","author":"Yamaguchi M","year":"1992","journal-title":"J Rheumatol"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.survophthal.2005.12.003"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.berh.2004.05.004"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.22477"},{"key":"e_1_2_1_13_2","first-page":"1645","article-title":"Adult onset otill's disease associated hemophagocytosis","volume":"24","author":"Kumakura S","year":"1997","journal-title":"J Rheumatol"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.85.5.421"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1097\/00124743-200108000-00013"},{"key":"e_1_2_1_16_2","first-page":"299","article-title":"Haemophagocytic syndrome in adults: Current concepts and challenges ahead","volume":"135","author":"Emmenegger U","year":"2005","journal-title":"Swiss Med Wkly"},{"key":"e_1_2_1_17_2","first-page":"401","article-title":"Macrophage activation syndrome following initiation of etanercept in a child with systemic onset juvenil rheumatoid arthritis","volume":"30","author":"Ramanan AV","year":"2003","journal-title":"JRheumatol"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1097\/00002281-200209000-00012"},{"key":"e_1_2_1_19_2","first-page":"132","article-title":"Is Reye's syndrome caused by augmented release of tumor necrosis factor?","author":"Larrick JW","year":"1987","journal-title":"Lancet"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(97)70196-3"},{"key":"e_1_2_1_21_2","first-page":"263","article-title":"Treatment of four patient with erythrophagocytic lymphohistiocytosis by a combination of epipodophyllotoxin, steroides, intratechal methotrexat and cranial irradiation","volume":"76","author":"Fischer A","year":"1985","journal-title":"Pediatrics"},{"key":"e_1_2_1_22_2","first-page":"533","article-title":"Intravenously administered gamma\u2010globulins in reactive hemophagocytic syndrome. Multicenter study to assess their importance, by the immunoglobulins group of experts of CEDIT of the AP\u2010HP","volume":"151","author":"Larroche C","year":"2000","journal-title":"Ann Med Interne"},{"key":"e_1_2_1_23_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1744-9987.1998.tb00127.x"},{"key":"e_1_2_1_24_2","first-page":"2120","article-title":"Etanercept in the treatment of macrophage activation syndrome","volume":"28","author":"Prahalad S","year":"2001","journal-title":"J Rheumatol"},{"key":"e_1_2_1_25_2","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19800515)45:10<2516::AID-CNCR2820451008>3.0.CO;2-V"},{"key":"e_1_2_1_26_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.21061"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.21084","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.21084","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,7]],"date-time":"2023-10-07T12:37:51Z","timestamp":1696682271000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.21084"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,1,8]]},"references-count":25,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2008,5]]}},"alternative-id":["10.1002\/ajh.21084"],"URL":"https:\/\/doi.org\/10.1002\/ajh.21084","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,1,8]]}}}