{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T11:00:32Z","timestamp":1785322832826,"version":"3.55.0"},"reference-count":44,"publisher":"Akademiai Kiado Zrt.","issue":"47","license":[{"start":{"date-parts":[[2024,11,24]],"date-time":"2024-11-24T00:00:00Z","timestamp":1732406400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.akjournals.com"],"crossmark-restriction":true},"short-container-title":["OH"],"published-print":{"date-parts":[[2024,11,24]]},"abstract":"<jats:p>\n                    <jats:italic>Bevezet\u00e9s:<\/jats:italic>\n                    Primer vagy k\u00f6nny\u0171l\u00e1nc-amyloidosis eset\u00e9ben k\u00f3rosan\n                    termel\u0151d\u0151 monoklon\u00e1lis immunglobulink\u00f6nny\u0171l\u00e1nc-fragmentumok rak\u00f3dnak le a\n                    szervekben, a kialakul\u00f3 amyloiddepozitumok pedig diszfunkci\u00f3t okoznak.\n                    <jats:italic>C\u00e9lkit\u0171z\u00e9s \u00e9s m\u00f3dszer:<\/jats:italic>\n                    Kutat\u00e1sunk sor\u00e1n a Semmelweis\n                    Egyetem Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinik\u00e1j\u00e1n 2010 \u00e9s 2024 k\u00f6z\u00f6tt\n                    k\u00f6nny\u0171l\u00e1nc-amyloidosissal diagnosztiz\u00e1lt betegek klinikai, illetve t\u00fal\u00e9l\u00e9si\n                    adatait vizsg\u00e1ltuk, hat id\u0151szakra bontva. K\u00fcl\u00f6n\u00f6s figyelmet ford\u00edtottunk a\n                    progn\u00f3zist lehets\u00e9gesen befoly\u00e1sol\u00f3 t\u00e9nyez\u0151k vizsg\u00e1lat\u00e1ra, \u00edgy tanulm\u00e1nyoztuk a\n                    t\u00fal\u00e9l\u00e9s kapcsolat\u00e1t a diagn\u00f3zis idej\u00e9vel, a betegek \u00e9letkor\u00e1val, a citogenetikai\n                    h\u00e1tt\u00e9rrel, tov\u00e1bb\u00e1 a k\u00fcl\u00f6nb\u00f6z\u0151 kezel\u00e9si s\u00e9m\u00e1k szerint.\n                    <jats:italic>Eredm\u00e9nyek:<\/jats:italic>\n                    A vizsg\u00e1lt id\u0151szakban \u00f6sszesen 56,\n                    k\u00f6nny\u0171l\u00e1nc-amyloidosisos beteget diagnosztiz\u00e1ltunk. A n\u00f6vekv\u0151 esetsz\u00e1mokb\u00f3l\n                    megfigyelhet\u0151 az a tendencia, hogy egyre gyakrabban ismerik fel a betegs\u00e9get, a\n                    legt\u00f6bbsz\u00f6r viszont tov\u00e1bbra is csak el\u0151rehaladott st\u00e1diumban sz\u00fcletik meg a\n                    diagn\u00f3zis. A teljes csoport medi\u00e1nt\u00fal\u00e9l\u00e9se 44 h\u00f3nap volt, \u00e9s nem mutatott\n                    szignifik\u00e1ns javul\u00e1st a vizsg\u00e1lt id\u0151szakok sor\u00e1n. A progn\u00f3zist a legink\u00e1bb a\n                    betegek \u00e9letkora, illetve a diagn\u00f3ziskor meg\u00e1llap\u00edtott st\u00e1diumbeoszt\u00e1s\n                    befoly\u00e1solta. A k\u00f6nny\u0171l\u00e1nc-amyloidosis ter\u00e1pi\u00e1ja sok v\u00e1ltoz\u00e1son ment \u00e1t az\n                    elm\u00falt 15 \u00e9vben. Az \u00fajabb t\u00edpus\u00fa szerek \u2013 mint a daratumumab \u00e9s a venetoclax \u2013\n                    haszn\u00e1lata mellett gyakrabban alakult ki komplett hematol\u00f3giai remisszi\u00f3,\n                    illetve szervv\u00e1laszt is nagyobb ar\u00e1nyban figyelt\u00fcnk meg a kor\u00e1bban alkalmazott\n                    kezel\u00e9sekkel \u00f6sszehasonl\u00edtva.\n                    <jats:italic>K\u00f6vetkeztet\u00e9s:<\/jats:italic>\n                    Az \u00fajabb t\u00edpus\u00fa\n                    gy\u00f3gyszerekre adott kedvez\u0151bb ter\u00e1pi\u00e1s v\u00e1lasz hat\u00e1s\u00e1ra sz\u00e1m\u00edthatunk a j\u00f6v\u0151ben a\n                    t\u00fal\u00e9l\u00e9si statisztik\u00e1k javul\u00e1s\u00e1ra is, az ut\u00f3bbit azonban a vizsg\u00e1latunkban eddig\n                    nem tudtuk igazolni. A kor\u00e1bbi st\u00e1diumban felfedezett betegs\u00e9g jelent\u0151sen\n                    kedvez\u0151bb progn\u00f3zist eredm\u00e9nyez, ez\u00e9rt a korszer\u0171 ter\u00e1pia mellett a korai\n                    felismer\u00e9s szerep\u00e9t sem lehet el\u00e9gg\u00e9 hangs\u00falyozni. Orv Hetil. 2024; 165(47):\n                    1860\u20131870.\n                  <\/jats:p>","DOI":"10.1556\/650.2024.33172","type":"journal-article","created":{"date-parts":[[2024,11,25]],"date-time":"2024-11-25T12:25:10Z","timestamp":1732537510000},"page":"1860-1870","update-policy":"https:\/\/doi.org\/10.1556\/policypage.crossmark.crossref.2","source":"Crossref","is-referenced-by-count":1,"title":["V\u00e1ltoz\u00e1sok a k\u00f6nny\u0171l\u00e1nc-amyloidosis kezel\u00e9s\u00e9ben \u2013 egy nagy budapesti centrum adatai"],"prefix":"10.1556","volume":"165","author":[{"given":"Fruzsina","family":"Sz\u00e9l","sequence":"first","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar Budapest Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1d\u00e1m","family":"Wiedemann","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1d\u00e1m","family":"Vida","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniella","family":"Nagy","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1gnes","family":"Feh\u00e9r","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vir\u00e1g R\u00e9ka","family":"Szita","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1gnes","family":"K\u00e1rp\u00e1ti","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zsolt","family":"Nagy","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P\u00e9ter","family":"Farkas","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zolt\u00e1n","family":"Pozsonyi","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tam\u00e1s","family":"Masszi","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gergely","family":"Varga","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"425","reference":[{"key":"R1","doi-asserted-by":"crossref","unstructured":"Makovitzky J. 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