{"id":6448,"date":"2026-07-23T08:46:48","date_gmt":"2026-07-23T06:46:48","guid":{"rendered":"https:\/\/rijetke-bolesti.com\/?p=6448"},"modified":"2026-07-23T08:55:06","modified_gmt":"2026-07-23T06:55:06","slug":"%f0%9f%a6%93-kad-epstein-barr-virus-otkrije-skriveni-poremecaj-imunoloskog-sustava","status":"publish","type":"post","link":"https:\/\/rijetke-bolesti.com\/en\/%f0%9f%a6%93-kad-epstein-barr-virus-otkrije-skriveni-poremecaj-imunoloskog-sustava\/","title":{"rendered":"\ud83e\udd93 Kad Epstein-Barr virus otkrije skriveni poreme\u0107aj imunolo\u0161kog sustava"},"content":{"rendered":"

Ve\u0107ina djece i odraslih tijekom \u017eivota preboli infekciju Epstein-Barr virusom (EBV) bez ozbiljnih posljedica. Me\u0111utim, kod manjeg broja bolesnika te\u0161ka, neobi\u010dno dugotrajna ili komplikacijama pra\u0107ena EBV infekcija mo\u017ee biti prvi znak do tada neprepoznate PRIMARNE IMUNODEFICIJENCIJE (PID).

U ovoj objavi predstavljamo rijetke, ali klini\u010dki iznimno va\u017ene poreme\u0107aje povezane s poreme\u0107enom kontrolom EBV infekcije:

\ud83d\udd39 XLP tip 1 (SH2D1A deficijencija)
\ud83d\udd39 XLP tip 2 (XIAP deficijencija)
\ud83d\udd39 ITK deficijencija
\ud83d\udd39 MAGT1 deficijencija (XMEN sindrom)

Iako su genetski razli\u010diti, ove bolesti dijele zajedni\u010dku karakteristiku \u2013 nemogu\u0107nost adekvatnog imunolo\u0161kog odgovora na EBV, \u0161to mo\u017ee rezultirati te\u0161kom infektivnom mononukleozom, hemofagocitnom limfohistiocitozom (HLH), kroni\u010dnom viremijom, limfoproliferativnim bolestima i limfomima.

Poseban izazov predstavlja \u010dinjenica da su mnogi bolesnici prije prve ozbiljne infekcije potpuno zdravi i bez znakova imunodeficijencije.

Upravo zato je va\u017eno prepoznati situacije koje zahtijevaju dodatnu obradu i genetsko testiranje.

\ud83e\uddec Na primarnu imunodeficijenciju treba pomisliti kod:
\u2714\ufe0f neuobi\u010dajeno te\u0161kih ili rekurentnih infekcija
\u2714\ufe0f te\u0161ke ili komplicirane EBV infekcije
\u2714\ufe0f razvoja HLH-a
\u2714\ufe0f limfoproliferacije ili limfoma u dje\u010djoj i mla\u0111oj \u017eivotnoj dobi
\u2714\ufe0f pozitivne obiteljske anamneze sli\u010dnih poreme\u0107aja

Rano postavljena dijagnoza omogu\u0107uje ciljano lije\u010denje, pravovremeno pra\u0107enje komplikacija i, kod odre\u0111enih bolesnika, transplantaciju krvotvornih mati\u010dnih stanica kao potencijalno jedinu kurativnu terapiju.

Jer iza naizgled \u201eobi\u010dne\u201c virusne infekcije ponekad se krije rijetka bolest koju ne smijemo propustiti prepoznati.

\ud83e\udd93 Think Zebra \u2013 jer rijetke bolesti postoje, a prepoznavanje mijenja \u017eivote.<\/p>\n\n\n\n

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Tekst pripremila: Katja \u0160piranec, dr. med.<\/strong><\/p>\n\n\n\n

Literatura:<\/p>\n\n\n\n

    \n
  1. Poli MC, Aksentijevich I, Bousfiha AA, et al.\u00a0Human inborn errors of immunity: 2024 update on the classification from the International Union of Immunological Societies Expert Committee.<\/em>\u00a0Journal of Human Immunity. 2025.<\/li>\n\n\n\n
  2. Bousfiha AA, Jeddane L, Moundir A, et al.\u00a0The 2024 update of IUIS phenotypic classification of human inborn errors of immunity.<\/em>\u00a0Journal of Human Immunity. 2025.<\/li>\n\n\n\n
  3. International Union of Immunological Societies (IUIS).\u00a0Inborn Errors of Immunity (IEI) Classification \u2013 2024 Update.<\/em><\/li>\n\n\n\n
  4. Tangye SG, Latour S.\u00a0Primary immunodeficiencies reveal the molecular requirements for effective host control of Epstein\u2013Barr virus infection.<\/em>\u00a0Blood. 2020.<\/li>\n\n\n\n
  5. Latour S, Winter S.\u00a0Inherited immunodeficiencies with high predisposition to Epstein\u2013Barr virus\u2013driven diseases.<\/em>\u00a0Frontiers in Immunology. 2018.<\/li>\n\n\n\n
  6. Booth C, Gilmour KC, Veys P, et al.\u00a0X-linked lymphoproliferative disease due to SH2D1A deficiency.<\/em>\u00a0Blood. 2011.<\/li>\n\n\n\n
  7. Chaigne-Delalande B, Li FY, O’Connor GM, et al.\u00a0Mg2+ regulates cytotoxic functions of NK and CD8 T cells in chronic EBV infection through NKG2D.<\/em>\u00a0Nature. 2013.<\/li>\n<\/ol>\n\n\n\n

    Stavovi i sadr\u017eaj izneseni u ovom tekstu odgovornost su autorice i ne predstavljaju nu\u017eno slu\u017ebene stavove Hrvatskog saveza za rijetke bolesti.<\/p>\n\n\n\n

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    Ve\u0107ina djece i odraslih tijekom \u017eivota preboli infekciju Epstein-Barr virusom (EBV) bez ozbiljnih posljedica. Me\u0111utim, kod manjeg broja bolesnika te\u0161ka, neobi\u010dno dugotrajna ili komplikacijama pra\u0107ena EBV infekcija mo\u017ee biti prvi znak do tada neprepoznate PRIMARNE IMUNODEFICIJENCIJE (PID). U ovoj objavi predstavljamo rijetke, ali klini\u010dki iznimno…<\/p>","protected":false},"author":3,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"content-type":"","footnotes":""},"categories":[1,34],"tags":[],"class_list":["post-6448","post","type-post","status-publish","format-standard","hentry","category-uncategorized","category-rijetka-infoteka"],"yoast_head":"\n\ud83e\udd93 Kad Epstein-Barr virus otkrije skriveni poreme\u0107aj imunolo\u0161kog sustava - Hrvatski savez za rijetke bolesti<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/rijetke-bolesti.com\/en\/\ud83e\udd93-kad-epstein-barr-virus-otkrije-skriveni-poremecaj-imunoloskog-sustava\/\" \/>\n<meta property=\"og:locale\" content=\"en_GB\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"\ud83e\udd93 Kad Epstein-Barr virus otkrije skriveni poreme\u0107aj imunolo\u0161kog sustava - Hrvatski savez za rijetke bolesti\" \/>\n<meta property=\"og:description\" content=\"Ve\u0107ina djece i odraslih tijekom \u017eivota preboli infekciju Epstein-Barr virusom (EBV) bez ozbiljnih posljedica. 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