Korelacija histoloških i imunohistoloških svojstava T-staničnoga limfoma kože (Mycosis fungoides) s kliničkom slikom, stadijem i ishodom bolesti u desetogodišnjem razdoblju [Correlation of histological and immunohistochemical properties of T-cell skin lymphoma (mycosis fungoides) with the clinical picture, stage and patient outcome in ten-year period]

Jerković Gulin, Sandra (2018) Korelacija histoloških i imunohistoloških svojstava T-staničnoga limfoma kože (Mycosis fungoides) s kliničkom slikom, stadijem i ishodom bolesti u desetogodišnjem razdoblju [Correlation of histological and immunohistochemical properties of T-cell skin lymphoma (mycosis fungoides) with the clinical picture, stage and patient outcome in ten-year period]. PhD thesis, Sveučilište u Zagrebu.

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Abstract

The aim of this study was to identify predictors of disease progression and death in patients with early stage mycosis fungoides (MF). Eighty-three patients diagnosed with early-stage MF at the Departments of Dermatovenerology and Pathology, UHC Zagreb between 01/2003 and 12/2012 were included in this retrospective-prospective study. Following parameters were analyzed: clinical picture, response to therapy, disease progression, lichenoid dermal lymphocyte infiltrate, „guardian“ lymphocytes, atypical lymphocytes and loss of surface CD2, CD3, CD5 and/or CD7 markers on T lymphocytes. Patients with initial stage IIA had inferior overall and progression-free survival than those with initial stage IB and IA. Patients with plaques also had inferior survivial than those with patches. Worse overall and progression-free survival was noted in patients with a premycotic stage lasting 48 months or less in comparison to those lasting more than 48 months. The same was seen in patients with more than 30 „guardian“ lymphocytes/100 keratinocytes (compared to those with 30 and less). Patients with more than 50% atypical lymphocytes had a faster progression rate than those with 50% or fewer atypical lymphocytes. Patients with loss of CD7 have faster progression than those without it. Initial disease stage IIA, presence of plaques in the initial clinical presentation, skin lesion encompassing >10% of total body surface, enlarged lymph nodes, disease progression, disease without response to therapy, dense lichenoid dermal infiltrate of lymphocytes and >30 „guardian“ lymphocytes/100 keratinocytes increase the risk for mortality within five years of diagnosis. Response to therapy statistically significantly affects the progression of the disease in the first five years after the diagnosis. Disease unresponsive to therapy increases the risk of progression within five years of diagnosis, while a complete response reduces this risk. This study identified potential new prognostic factors for early stage MF. Larger studies are needed to confirm these results that could improve our ability to identify patients with increased risk of disease progression and poor prognosis.

Abstract in Croatian

Cilj ovog istraživanja bio je odrediti prediktore progresije bolesti i smrtnog ishoda u bolesnika s ranim stadijem mycosis fungoides (MF). U ovu retrospektivno-prospektivnu studiju bila su uključena 83 bolesnika kojima je dijagnosticiran rani stadij MF u Klinici za dermatologiju i venerologiju i Klinici za patologiju i citologiju Kliničkog bolničkog centra Zagreb u razdoblju od siječnja 2003. do prosinca 2012. Analizirani su sljedeći parametri: klinička slika, terapijski odgovor, progresija bolesti, lihenoidni dermalni infiltrat limfocita, limfociti „čuvari“, količina atipije limfocita i gubitak površinskih markera T-limfocita CD2, CD3, CD5 i/ili CD7. Ukupno preživljenje i preživljenje bez progresije bolesti bolesnika s početnim stadijem IIA bilo je lošije, nego onih sa stadijem IB i IA. Bolesnici s plakovima u početnoj kliničkoj slici su također imali lošije preživljenje, nego oni s patchevima. Lošije preživljenje i bržu progresiju bolesti su imali bolesnici u kojih je premikotički stadij trajao 48 mjeseci i kraće u odnosu na one u kojih je trajao više od 48 mjeseci. Bolesnici s više od 30 limfocita „čuvara“ na 100 keratinocita imaju lošije preživljenje i bržu progresiju bolesti od onih s 30 i manje limfocita „čuvara“ na 100 keratinocita. Bolesnici s više od 50% atipičnih limfocita imaju bržu progresiju od onih s 50% i manje atipičnih limfocita. Bolesnici koji imaju gubitak CD7 imaju bržu progresiju od onih bez gubitka CD7. Početni stadij bolesti IIA, prisutnost plakova u početnoj kliničkoj slici, proširenost kožnih lezija >10%, povećani limfni čvorovi, progresija bolesti, bolest bez odgovora na terapiju, gust lihenoidni dermalni infiltrat i >30 limfocita „čuvara“ na 100 keratinocita povećavaju rizik od smrtnog ishoda u prvih pet godina nakon postavljanja dijagnoze. Odgovor na terapiju statistički značajno utječe na progresiju bolesti u prvih 5 godina nakon postavljanja dijagnoze. Bolest bez odgovora na terapiju povećava rizik od progresije bolesti unutar 5 godina od postavljanja dijagnoze dok bolest s potpunim odgovorom na terapiju smanjuje taj rizik. Ovim istraživanjem su otkriveni potencijalni novi prognostički faktori za rani stadij MF. Veće studije su potrebne kako bi potvrdile ove rezultate koji bi mogli pomoći u identificiranju bolesnika s povećanim rizikom za progresiju bolesti i lošijom prognozom.

Item Type: Thesis (PhD)
Mentors:
Mentor
Čeović, Romana
Departments: Izvan medicinskog fakulteta
Depositing User: Anja Majstorović
University: Sveučilište u Zagrebu
Institution: Medicinski fakultet
Number of Pages: 76
Status: Unpublished
Creators:
CreatorsEmail
Jerković Gulin, SandraUNSPECIFIED
Date: 19 March 2018
Date Deposited: 16 Jan 2019 08:39
Last Modified: 17 Jan 2019 12:06
Subjects: /
Related URLs:
    URI: http://medlib.mef.hr/id/eprint/3042

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