Anaplastični limfom velikih stanica

master's thesis

• Title (english): Anaplastic large cell lymphoma
• Author: Marko Lagančić
• Mentor: Srećko Budi (mentor)
• Committee member: Rado Žic (predsjednik povjerenstva)
• Committee member: Tomislav Meštrović (član povjerenstva)
• Committee member: Srećko Budi (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Surgery); Zagreb
• Defense date and country: 2020-07-17, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Surgery
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Oncology

Abstract

Anaplastični limfom velikih stanica (ALCL) rijedak je podtip ne-Hodgkinova limfoma (NHL) i potječe od zrelih T-stanica. Suvremena WHO Klasifikacija hematoloških malignoma prepoznaje primarni kožni ALCL (PC-ALCL), dva podtipa sistemskog
ALCL (ALK-pozitivni i ALK-negativni) i anaplastični limfom velikih stanica povezan s implantatima dojki (BIA-ALCL) kao novouvršteni patološki entitet. PC-ALCL se CD30+ klasificira kao jedan od primarno kožnih T-staničnih limfoproliferativnih
poremećaja. ALK-pozitivni ALCL obično nastaje kod djece i mlađih odraslih osoba, dok je ALK-negativni ALCL češći kod pacijenata starijih od 55 godina. Oba sistemska podtipa ovog limfoma liječe se kao agresivni limfomi, ali tijek bolesti može se
razlikovati. ALK-pozitivni ALCL dobro odgovara na standardnu kemoterapiju i većina pacijenata time postiže dugoročnu remisiju. ALK-negativni ALCL također u početku pokazuje odgovor na terapiju, ali veća je vjerojatnost povrata bolesti (relapsa). BIA-ALCL je poseban oblik CD30-pozitivnog i ALK-negativnog ALCL koji nastaje u vezi s implantatom dojki (obično 8-10 godina nakon kirurškog zahvata). Unatoč niskoj incidenciji ove nove bolesti, uz sve veću primjenu implantata u kozmetske i rekonstrukcijske svrhe, BIA-ALCL postaje sve veći medicinski izazov. Etiopatogeneza BIA-ALCL je najvjerojatnije multifaktorijalna, a trenutne teorije temeljene na dokazima predlažu kombinaciju kronične upale, stvaranja biofilma, teksture implantata, genetske predispozicije i vremena. Čini se da su implantati teksturiranih površina povezani s praktički svim slučajevima BIA-ALCL. Klasična klinička prezentacija bolesti je u obliku kasno nastupajućeg, perzistentnog seroma oko implantata. Većina pacijenta javlja se s lokaliziranom bolešću, što uglavnom znači i izvrsnu prognozu. Potpuna kirurška ekscizija ima ključnu ulogu u liječenju.

Abstract (english)

Anaplastic large cell lymphoma (ALCL) is a rare type of non-Hodgkin lymphoma (NHL) and it originates from mature T-cells. The contemporary WHO Classification of Haematologic Malignancies recognizes primary cutaneous ALCL (PC-ALCL), two
subtypes of systemic ALCL (ALK-positive and ALK-negative) and breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) as a newly included CD30+ pathological entity. PC-ALCL is classified as one of the primary cutaneous T-cell lymphoproliferative disorders. ALK-positive ALCL usually affects children and young adults, whereas ALK-negative ALCL is more common in patients over the age of 55 years. Both systemic lymphomas are treated as aggressive lymphomas, but the disease course may be different. ALK-positive ALCL responds well to standard chemotherapy treatments, putting most patients into long-term remission. ALK-negative ALCL initially responds to treatment as well, but the disease is more likely to relapse. BIA-ALCL is a distinct form of CD30-positive and ALK-negative ALCL that arises in association with a breast implant (usually 8-10 years after surgey). Despite the low incidence of this new disease, the increasing use of breast
implants for cosmetic or post-mastectomy reconstruction purposes places BIA-ALC as an emerging and compelling medical challenge. The etiopathogenesis of BIA-ALCL is likely to be multifactorial, with current evidence-based theories recognizing
the combination of chronic inflammation, biofilm formation, implant texturization, genetic predisposition and time. Breast implants with textured surfaces seem to be associated with nearly all cases of BIA-ALCL. Late onset, persistent seroma around breast implant represents the classical clinical presentation. Most of the patients present with localized disease, which confers an excellent prognosis. Complete surgical excision has a key role in the treatment.

• Keywords: anaplastični limfom velikih stanica; anaplastični limfom velikih stanica povezan s implantatima dojki; implantat dojki
• Keywords (english): anaplastic large cell lymphoma; breast implant-associated anaplastic large cell lymphoma; breast implant
• Language: croatian
• URN:NBN: urn:nbn:hr:105:681357
• 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: 2021-04-21 12:22:52