{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T00:14:43Z","timestamp":1785370483350,"version":"3.55.0"},"reference-count":49,"publisher":"Akademiai Kiado Zrt.","issue":"4","license":[{"start":{"date-parts":[[2022,1,23]],"date-time":"2022-01-23T00:00:00Z","timestamp":1642896000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["akjournals.com"],"crossmark-restriction":true},"short-container-title":["OH"],"published-print":{"date-parts":[[2022,1,23]]},"abstract":"<jats:p><jats:bold>\u00d6sszefoglal\u00f3.<\/jats:bold> Az autoimmun lymphoproliferativ szindr\u00f3ma egy ritka,\n                    immundeficienti\u00e1val j\u00e1r\u00f3 genetikai betegs\u00e9g. H\u00e1tter\u00e9ben az extrinszik\n                    apoptotikus \u00fatvonal g\u00e9njeinek \u00f6r\u00f6kletes vagy szerzett mut\u00e1ci\u00f3i \u00e9s a\n                    k\u00f6vetkezm\u00e9nyesen kialakul\u00f3, aktiv\u00e1lt lymphocyt\u00e1k negat\u00edv szelekci\u00f3j\u00e1nak a\n                    defektusa \u00e1ll. Az autoimmun lymphoproliferativ szindr\u00f3ma klinikai megjelen\u00e9s\u00e9re\n                    jellemz\u0151 a j\u00f3indulat\u00fa lymphocytaburj\u00e1nz\u00e1s k\u00f6vetkezt\u00e9ben kialakul\u00f3\n                    lymphadenopathia \u00e9s l\u00e9pmegnagyobbod\u00e1s. Gyakran t\u00e1rsul olyan autoimmun\n                    k\u00f3rk\u00e9pekkel, mint az autoimmun haemolyticus anaemia vagy az autoimmun\n                    thrombocytopenia. A betegs\u00e9gben jellemz\u0151 laborat\u00f3riumi elt\u00e9r\u00e9sek a k\u00f6vetkez\u0151k:\n                    az \u03b1\u03b2<jats:sup>+<\/jats:sup> CD4<jats:sup>\u2013<\/jats:sup>\/CD8<jats:sup>\u2013<\/jats:sup> kett\u0151s negat\u00edv T-sejtek\n                    szaporulata, a szol\u00fabilis Fas-ligand, az interleukin-10 \u00e9s interleukin-18,\n                    valamint a B<jats:sub>12<\/jats:sub>-vitamin sz\u00e9rumszintj\u00e9nek emelked\u00e9se. A k\u00f3rk\u00e9p\n                    diagn\u00f3zis\u00e1hoz hozz\u00e1tartozik az <jats:italic>in vitro<\/jats:italic> Fas-medi\u00e1lt apopt\u00f3zis\n                    funkci\u00f3j\u00e1nak vizsg\u00e1lata, valamint a genetikai vizsg\u00e1lat.\n                    Differenci\u00e1ldiagnosztikai szempontb\u00f3l fontos elk\u00fcl\u00f6n\u00edteni a lymphom\u00e1kt\u00f3l,\n                    valamint az autoimmun lymphoproliferativ szindr\u00f3maszer\u0171 betegs\u00e9gekt\u0151l. A kezel\u00e9s\n                    alapja a t\u00e1rsul\u00f3 autoimmun k\u00f3rk\u00e9pek t\u00fcneteinek cs\u00f6kkent\u00e9se immunszuppressz\u00edv\n                    ter\u00e1pi\u00e1val. Orv Hetil. 2022; 163(4): 123\u2013131.<\/jats:p>\n                <jats:p><jats:bold>Summary.<\/jats:bold> The autoimmune lymphoproliferative syndrome is a rare\n                    genetic disorder causing immunodeficiency. In the background of the disease,\n                    germline or somatic mutations of genes participating in the extrinsic apoptotic\n                    pathway and the consequential defect in the negative selection of activated\n                    lymphocytes were discovered. The clinical appearance of autoimmune\n                    lymphoproliferative syndrome consists of non-malignant lymphoproliferation,\n                    lymphadenopathy and splenomegaly, it is frequently accompanied by autoimmune\n                    disorders such as autoimmune haemolytic anaemia or autoimmune thrombocytopenia.\n                    The main diagnostic laboratory findings of this disease are the following: an\n                    elevation in \u03b1\u03b2<jats:sup>+<\/jats:sup>, CD4<jats:sup>\u2013<\/jats:sup>\/CD8<jats:sup>\u2013<\/jats:sup> double-negative T\n                    cell count, elevated serum levels of soluble Fas-ligand, interleukin-10,\n                    interleukin-18 and vitamin B<jats:sub>12<\/jats:sub>. Other useful laboratory tests are the\n                        <jats:italic>in vitro<\/jats:italic> Fas-mediated apoptotic functional assay and the\n                    genetic screening for gene mutations. Differential diagnosis should exclude\n                    malignant lymphoproliferation in lymphomas and non-malignant autoimmune\n                    lymphoprolipherative syndrome-like diseases. The main aim of the treatment is\n                    the amelioration of the accompanying autoimmune disease with immunosuppressive\n                    therapy. Orv Hetil. 2022; 163(4): 123\u2013131.<\/jats:p>","DOI":"10.1556\/650.2022.32353","type":"journal-article","created":{"date-parts":[[2022,11,18]],"date-time":"2022-11-18T20:18:36Z","timestamp":1668802716000},"page":"123-131","update-policy":"https:\/\/doi.org\/10.1556\/policypage.crossmark.crossref.1","source":"Crossref","is-referenced-by-count":1,"title":["Nem malignus, nem infectiosus lymphoproliferatio: kih\u00edv\u00e1sok az autoimmun lymphoproliferativ szindr\u00f3ma diagnosztik\u00e1j\u00e1ban \u00e9s kezel\u00e9s\u00e9ben"],"prefix":"10.1556","volume":"163","author":[{"given":"Emese","family":"Moln\u00e1r","sequence":"first","affiliation":[{"name":"Orsz\u00e1gos V\u00e9rell\u00e1t\u00f3 Szolg\u00e1lat, K\u00f6z\u00e9p-magyarorsz\u00e1gi Region\u00e1lis V\u00e9rell\u00e1t\u00f3 K\u00f6zpont Budapest, Karolina \u00fat 19\u201321., 1113"},{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Transzfuziol\u00f3giai Tansz\u00e9k Budapest"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e1bor","family":"Kov\u00e1cs","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Transzfuziol\u00f3giai Tansz\u00e9k Budapest"},{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, \u00c9lettani Int\u00e9zet Budapest"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L\u00edvia","family":"Varga","sequence":"additional","affiliation":[{"name":"Orsz\u00e1gos V\u00e9rell\u00e1t\u00f3 Szolg\u00e1lat, K\u00f6z\u00e9p-magyarorsz\u00e1gi Region\u00e1lis V\u00e9rell\u00e1t\u00f3 K\u00f6zpont Budapest, Karolina \u00fat 19\u201321., 1113"},{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Transzfuziol\u00f3giai Tansz\u00e9k Budapest"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Szabolcs","family":"Tasn\u00e1dy","sequence":"additional","affiliation":[{"name":"D\u00e9l-pesti Centrumk\u00f3rh\u00e1z, Orsz\u00e1gos Hematol\u00f3giai \u00e9s Infektol\u00f3giai Int\u00e9zet, K\u00f6zponti Laborat\u00f3rium Budapest"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andr\u00e1s","family":"Bors","sequence":"additional","affiliation":[{"name":"D\u00e9l-pesti Centrumk\u00f3rh\u00e1z, Orsz\u00e1gos Hematol\u00f3giai \u00e9s Infektol\u00f3giai Int\u00e9zet, Molekul\u00e1ris Genetikai Laborat\u00f3rium Budapest"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lenke","family":"Tank\u00f3","sequence":"additional","affiliation":[{"name":"D\u00e9l-pesti Centrumk\u00f3rh\u00e1z, Orsz\u00e1gos Hematol\u00f3giai \u00e9s Infektol\u00f3giai Int\u00e9zet, Molekul\u00e1ris Genetikai Laborat\u00f3rium Budapest"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D\u00f3ra","family":"Csab\u00e1n","sequence":"additional","affiliation":[{"name":"D\u00e9l-pesti Centrumk\u00f3rh\u00e1z, Orsz\u00e1gos Hematol\u00f3giai \u00e9s Infektol\u00f3giai Int\u00e9zet, Molekul\u00e1ris Genetikai Laborat\u00f3rium Budapest"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katalin","family":"Kap\u00f3cs","sequence":"additional","affiliation":[{"name":"D\u00e9l-pesti Centrumk\u00f3rh\u00e1z, Orsz\u00e1gos Hematol\u00f3giai \u00e9s Infektol\u00f3giai Int\u00e9zet, Molekul\u00e1ris Genetikai Laborat\u00f3rium Budapest"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zsuzsanna","family":"Nemes-Nagy","sequence":"additional","affiliation":[{"name":"Orsz\u00e1gos V\u00e9rell\u00e1t\u00f3 Szolg\u00e1lat, K\u00f6z\u00e9p-magyarorsz\u00e1gi Region\u00e1lis V\u00e9rell\u00e1t\u00f3 K\u00f6zpont Budapest, Karolina \u00fat 19\u201321., 1113"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hajnalka","family":"Andrikovics","sequence":"additional","affiliation":[{"name":"D\u00e9l-pesti Centrumk\u00f3rh\u00e1z, Orsz\u00e1gos Hematol\u00f3giai \u00e9s Infektol\u00f3giai Int\u00e9zet, Molekul\u00e1ris Genetikai Laborat\u00f3rium Budapest"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"425","reference":[{"key":"ref0","doi-asserted-by":"publisher","first-page":"1449","DOI":"10.1126\/science.7533326","article-title":"The Fas death factor","volume":"267","author":"Nagata S","year":"1995","unstructured":"1 Nagata S, Golstein P. 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