Posttransplantacijski limfoproliferativni sindrom uzrokovan Epstein-Barr-virusom

master's thesis

• Title (english): Post-transplant lymphoproliferative disease caused by Epstein-Barr virus
• Author: Juraj Matej Kovač
• Mentor: Adriana Vince (mentor)
• Committee member: Dalibor Vukelić (predsjednik povjerenstva)
• Committee member: Goran Tešović (član povjerenstva)
• Committee member: Adriana Vince (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Infectious Diseases); Zagreb
• Defense date and country: 2020-07-17, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Infectology

Abstract

Posttransplantacijski limfoproliferativni sindrom najčešća je komplikacija presadbe solidnih organa i krvotvornih matičnih stanica. U više od polovice svih slučajeva bolest je povezana s infekcijom Epstein-Barrinim virusom, ubikvitarnim patogenom kojim budu inficirane gotovo sve odrasle osobe. Virus je poznatiji kao uzročnik infektivne mononukleoze, a povezuje se i s nastankom raznih drugih bolesti, poput Burkittova limfoma, nazofaringealnoga karcinoma i Hodgkinova limfoma. Bolest se češće javlja u pedijatrijskoj populaciji, a s obzirom na vrstu presatka pogođeniji su primatelji solidnih organa nego primatelji krvotvornih matičnih stanica. Rizični čimbenici uključuju mladu ili stariju životnu dob te predtransplantacijsku seronegativnost na virusne antigene. Sindrom se morfološki klasificira u četiri tipa – nedestruktivni, polimorfni, monomorfni i tip klasičnoga Hodgkinova limfoma – a prema vremenu nastanka dijeli se na rani i kasni oblik. U kliničkoj slici dominiraju sustavni simptomi i znakovi poput vrućice, umora, grlobolje, gubitka tjelesne mase, anoreksije, mučnine i povraćanja, te simptomi i znakovi vezani uz presadak. Bolest se prvenstveno dijagnosticira patohistološkim nalazom, a od koristi su i radiološka te endoskopska dijagnostika. Praćenje titra virusne DNA pokazalo se nezaobilaznim ne samo u dijagnostici nego i u prevenciji i liječenju bolesti. Terapija se razlikuje između bolesnika s organskim i matičnostaničnim presatkom. Nastanak bolesti nastoji se spriječiti predtransplantacijskom kemoprofilaksom i imunoprofilaksom te posttransplantacijskim praćenjem EBV-viremije uz smanjenje imunosupresije i primjenu rituksimaba. Rituksimab čini i prvu liniju liječenja nastale bolesti. U ostale terapijske opcije ubrajaju se radioterapija, kirurška ekscizija, kemoterapija te primjena citotoksičnih limfocita T.

Abstract (english)

Post-transplant lymphoproliferative disease is the most frequent complication of both solid organ and hematopoetic stem cell transplantation. More than half of the cases of the disease are associated with Epstein-Barr virus infection. The ubiquitous virus infects virtually all adults. It is better known as the cause of infectious mononucleosis and is associated with
many other illnesses, such as Burkitt lymphoma, nasopharyngeal carcinoma and Hodgkin lymphoma. The disease occurs more frequently in pediatric population and solid organ transplant recipients are affected more often than stem cell transplant recipients. Risk factors include young or older age and pre-transplant viral seronegativity. Based on its morphology, the disease is classified into four categories – non-destructive, polymorphic, monomorphic and classic Hodgkin lymphoma. With regards to the onset, the disease can be either early-onset or late-onset. The clinical presentation is characterised by systemic signs and symptoms, such as fever, malaise, sore throat, weight loss, anorexia, nausea and vomiting, and allograft-specific symptoms. The diagnosis is primarily made with pathohistology, although radiologic and endoscopic techniques may also be of use. Viral load monitoring has proved itself valuable not only in diagnosis but also in disease prevention and therapy. The treatment is different in solid organ transplant recipients compared to stem cell transplant recipients. Prevention of the disease consists of pre-transplant chemoprophylaxis and immunoprophylaxis, and post-transplant viral load monitoring combined with reduction in immunosuppresion and rituximab. Rituximab is the first line therapy. Other treatment options include radiotherapy, surgical excision, chemotherapy, and cytotoxic T lymphocytes.

• Keywords: posttransplantacijski limfoproliferativni sindrom; Epstein-Barr-virus; prevencija; profilaksa; liječenje
• Keywords (english): post-transplant lymphoproliferative disease; Epstein-Barr virus; prevention; prophylaxis; treatment
• Language: croatian
• URN:NBN: urn:nbn:hr:105:491882
• Study programme: Title: Medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2021-04-14 07:30:16