{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,3,27]],"date-time":"2024-03-27T17:16:30Z","timestamp":1711559790664},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2013,12,1]],"date-time":"2013-12-01T00:00:00Z","timestamp":1385856000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Med Case Reports"],"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Introduction<\/jats:title>\n            <jats:p>Evans syndrome is a rare hematological disease commonly defined as Coombs-positive hemolytic anemia and immune thrombocytopenia. Pathophysiology of this disease involves decreased cluster of differentiation (CD)4+ T-helper cell counts, increased CD8+ T-suppressor cell counts, a decreased CD4\/CD8 ratio, and reduced serum immunoglobulin G, M and A levels - indicating a complex immune dysregulation. Association with other autoimmune diseases has been described although involvement of the central nervous system has not been reported so far.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Case presentation<\/jats:title>\n            <jats:p>We here present a case of a 28-year-old woman of Turkish origin with progressive, disseminated, partly mass-forming lymphoplasmacellular infiltration (CD3+ and CD138+ cells) of the brain in association with Evans syndrome. No other central nervous system disorder could be identified on neuropathological evaluation. Although treatment with rituximab was effective to normalize erythrocyte and thrombocyte levels in her peripheral blood, it failed to dampen the inflammation in her central nervous system or prevent clinical progression. Initiation of treatment with cyclophosphamide resulted in stabilization of her central nervous system inflammation and the disease course.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p>The complex immune dysregulation resulting in the antibody-mediated pathologies that can be regarded as the cause of both lymphoplasmacellular encephalitis and\u00a0Evans syndrome\u00a0renders this association to be of clinical relevance for both neurologists and hematologists. Our experience also sheds light on the effectiveness of different treatments for both disorders and we advise clinicians to take a closer look when encountering a combination of peripheral blood diseases with affection of the central nervous system.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1186\/1752-1947-7-262","type":"journal-article","created":{"date-parts":[[2013,12,3]],"date-time":"2013-12-03T16:14:07Z","timestamp":1386087247000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Evans syndrome associated with sterile inflammation of the central nervous system: a case report"],"prefix":"10.1186","volume":"7","author":[{"given":"Ole Jan","family":"Simon","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tanja","family":"Kuhlmann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan","family":"Bittner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carsten","family":"M\u00fcller-Tidow","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jochen","family":"Weigt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heinz","family":"Wiendl","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sven G","family":"Meuth","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2013,12,3]]},"reference":[{"key":"2640_CR1","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1001\/archinte.1951.03810010058005","volume":"87","author":"RS Evans","year":"1951","unstructured":"Evans RS, Takahashi K, Duane RT, Payne R, Liu C: Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951, 87: 48-65. 10.1001\/archinte.1951.03810010058005.","journal-title":"AMA Arch Intern Med"},{"key":"2640_CR2","doi-asserted-by":"publisher","first-page":"2443","DOI":"10.1182\/blood-2004-09-3542","volume":"105","author":"DT Teachey","year":"2005","unstructured":"Teachey DT, Manno CS, Axsom KM, Andrews T, Choi JK, Greenbaum BH, McMann JM, Sullivan KE, Travis SF, Grupp SA: Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). Blood. 2005, 105: 2443-2448. 10.1182\/blood-2004-09-3542.","journal-title":"Blood"},{"key":"2640_CR3","doi-asserted-by":"publisher","first-page":"362","DOI":"10.1016\/j.jbspin.2011.07.004","volume":"79","author":"GL Costallat","year":"2012","unstructured":"Costallat GL, Appenzeller S, Costallat LT: Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome. Joint Bone Spine. 2012, 79: 362-364. 10.1016\/j.jbspin.2011.07.004.","journal-title":"Joint Bone Spine"},{"key":"2640_CR4","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1002\/ajh.2830150409","volume":"15","author":"W Wang","year":"1983","unstructured":"Wang W, Herrod H, Pui CH, Presbury G, Wilimas J: Immunoregulatory abnormalities in Evans syndrome. Am J Hematol. 1983, 15: 381-390. 10.1002\/ajh.2830150409.","journal-title":"Am J Hematol"},{"key":"2640_CR5","doi-asserted-by":"publisher","first-page":"968","DOI":"10.1046\/j.1365-2141.2000.02296.x","volume":"110","author":"M Karakantza","year":"2000","unstructured":"Karakantza M, Mouzaki A, Theodoropoulou M, Bussel JB, Maniatis A: Th1 and Th2 cytokines in a patient with Evans\u2019 syndrome and profound lymphopenia. Brit J Haematol. 2000, 110: 968-970. 10.1046\/j.1365-2141.2000.02296.x.","journal-title":"Brit J Haematol"},{"key":"2640_CR6","doi-asserted-by":"publisher","first-page":"1484","DOI":"10.4067\/S0034-98872011001100015","volume":"139","author":"EJ Carey","year":"2011","unstructured":"Carey EJ, Somaratne K, Rakela J: Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome. Rev Med Chil. 2011, 139: 1484-1487. 10.4067\/S0034-98872011001100015.","journal-title":"Rev Med Chil"},{"key":"2640_CR7","doi-asserted-by":"publisher","first-page":"279","DOI":"10.5045\/kjh.2011.46.4.279","volume":"46","author":"HJ Oh","year":"2011","unstructured":"Oh HJ, Yun MJ, Lee ST, Lee SJ, Oh SY, Sohn I: Evans syndrome following long-standing Hashimoto\u2019s thyroiditis and successful treatment with rituximab. Korean J Hematol. 2011, 46: 279-282. 10.5045\/kjh.2011.46.4.279.","journal-title":"Korean J Hematol"},{"key":"2640_CR8","doi-asserted-by":"publisher","first-page":"144","DOI":"10.1016\/j.jaad.2009.10.035","volume":"66","author":"KD Jung","year":"2012","unstructured":"Jung KD, Kim PS, Park HY, Kim CR, Byun JY, Lee DY, Lee JH, Yang JM, Lee ES: Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome. J Am Acad Dermatol. 2012, 66: 144-147. 10.1016\/j.jaad.2009.10.035.","journal-title":"J Am Acad Dermatol"},{"key":"2640_CR9","first-page":"374","volume":"1","author":"T Guez","year":"1992","unstructured":"Guez T, Smadja D, Mas JL, Sicard D: Chronic inflammatory demyelinating polyradiculoneuropathy associated with Evans\u2019 syndrome. Eur J Med. 1992, 1: 374-376.","journal-title":"Eur J Med"},{"key":"2640_CR10","doi-asserted-by":"publisher","first-page":"254","DOI":"10.1097\/01.md.0000133624.65946.40","volume":"83","author":"M Michel","year":"2004","unstructured":"Michel M, Chanet V, Galicier L, Ruivard M, Levy Y, Hermine O, Oksenhendler E, Schaeffer A, Bierling P, Godeau B: Autoimmune thrombocytopenic purpura and common variable immunodeficiency: analysis of 21 cases and review of the literature. Medicine. 2004, 83: 254-263. 10.1097\/01.md.0000133624.65946.40.","journal-title":"Medicine"},{"key":"2640_CR11","doi-asserted-by":"publisher","first-page":"303","DOI":"10.1002\/ajh.20180","volume":"77","author":"E Mantadakis","year":"2004","unstructured":"Mantadakis E, Danilatou V, Stiakaki E, Kalmanti M: Rituximab for refractory Evans syndrome and other immune-mediated hematologic diseases. Am J Hematol. 2004, 77: 303-310. 10.1002\/ajh.20180.","journal-title":"Am J Hematol"},{"key":"2640_CR12","doi-asserted-by":"publisher","first-page":"1691","DOI":"10.3324\/haematol.11540","volume":"92","author":"B Bader-Meunier","year":"2007","unstructured":"Bader-Meunier B, Aladjidi N, Bellmann F, Monpoux F, Nelken B, Robert A, Armari-Alla C, Picard C, Ledeist F, Munzer M, Yacouben K, Bertrand Y, Pariente A, Chauss\u00e9 A, Perel Y, Leverger G: Rituximab therapy for childhood Evans syndrome. Haematologica. 2007, 92: 1691-1694. 10.3324\/haematol.11540.","journal-title":"Haematologica"},{"key":"2640_CR13","doi-asserted-by":"publisher","first-page":"347","DOI":"10.1002\/pbc.20312","volume":"45","author":"PT Jubinsky","year":"2005","unstructured":"Jubinsky PT, Rashid N: Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance. Pediatr Blood Cancer. 2005, 45: 347-350. 10.1002\/pbc.20312.","journal-title":"Pediatr Blood Cancer"},{"key":"2640_CR14","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1016\/j.jneuroim.2006.06.029","volume":"180","author":"AH Cross","year":"2006","unstructured":"Cross AH, Stark JL, Lauber J, Ramsbottom MJ, Lyons JA: Rituximab reduces B cells and T cells in cerebrospinal fluid of multiple sclerosis patients. J Neuroimmunol. 2006, 180: 63-70. 10.1016\/j.jneuroim.2006.06.029.","journal-title":"J Neuroimmunol"},{"key":"2640_CR15","doi-asserted-by":"publisher","first-page":"460","DOI":"10.1097\/00043426-200009000-00016","volume":"22","author":"R Jasty","year":"2000","unstructured":"Jasty R, Strouse PJ, Castle VP: Fatal lymphoproliferative disease as a complication of Evans syndrome. J Pediat Hematol Onc. 2000, 22: 460-463. 10.1097\/00043426-200009000-00016.","journal-title":"J Pediat Hematol Onc"}],"container-title":["Journal of Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/1752-1947-7-262.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/1752-1947-7-262\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/1752-1947-7-262","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/1752-1947-7-262.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,2]],"date-time":"2021-09-02T02:27:37Z","timestamp":1630549657000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmedicalcasereports.biomedcentral.com\/articles\/10.1186\/1752-1947-7-262"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,12]]},"references-count":15,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2013,12]]}},"alternative-id":["2640"],"URL":"https:\/\/doi.org\/10.1186\/1752-1947-7-262","relation":{},"ISSN":["1752-1947"],"issn-type":[{"value":"1752-1947","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,12]]},"assertion":[{"value":"11 May 2013","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 October 2013","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 December 2013","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"262"}}