Linfoma follicolare
Table of Contents
Il linfoma (Follicular Lymphoma, FL) costituisce circa il 30% dei linfoma non-Hodgkin e comprende un gruppo di neoplasie che originano dalle cellule B del centro germinativo. Il linfoma follicolare viene inquadrato al livello istopatologico in base al modello di crescita, nodulare, (follicolare, appunto) o diffuso.
Epidemiologia
Il linfoma follicolare è in termini di prevalenza la seconda più frequente neoplasia linfoproliferativa del mondo occidentale, rappresentando circa il 20% di tutti i linfomi non-Hodgkin ed almeno il 70% delle forme indolenti. La sua incidenza ha mostrato un rapido incremento negli ultimi 10 anni da 2-3 casi su 100.000 abitanti negli anni. Il linfoma follicolare interessa principalmente individui adulti nella sesta decade di vita, con una lievissima preponderanza del sesso femminile. Raramente la neoplasia colpisce pazienti giovani.
Eziopatologia del linfoma follicolare
Non è nota l’eziologia, sebbene possa essere postulata un’associazione con l’esposizione di alcuni agenti carcinogeni ambientali (pesticidi, fumo di sigaretta), con agenti virali (virus di Epstein-Barr, harpes virusHHV8) o con stati di immunodeficienza primitiva o acquisita. Aspetto caratteristico della patologia è la presenza del riarrangiamento del gene BCL2, che codifica per una proteina con funzione antiapoptotica, dato che peraltro si riscontra fisiologicamente nelle cellule B di memoria nell’individuo adulto e la cui prevalenza tende ad aumentare con l’età. Lo svilupparsi di una seconda alterazione genica (hit) accanto alla presenza del riarrangiamento del BCL2 si suppone possa determinare la trasformazione in senso neoplastico della normale popolazione linfocitaria del centro germinativo, da un lato sostenuta da uno stimolo proliferativo di natura antigenica, dall’altro non contrastata per via della perdita del segnale pro-apoptotico.
Manifestazioni cliniche
Al momento della diagnosi la maggior parte dei pazienti si presenta con una malattia diffusa a molteplici sedi linfonodali, prevalentemente in assenza di una sintomatologia specificamente linfoma correlata. I linfonodi maggiormente interessati dalla neoplasia sono quelli delle stazioni superficiali, del mediastino e del retroperitoneo; spesso sono riscontrabili masse adenopatiche addominali di grandi dimensioni (bulky). Il linfoma follicolare si presenta al III o IV stadio secondo Anna Harbor in almeno il 60% dei casi.
Diagnosi
La diagnosi è posta all’esame istologico e immunoistochimico di uno più linfonodi dotati di caratteristiche cliniche sospette ed escissi chirurgicamente. In alternativa, in presenza di adenopatie profonde o difficili da raggiungere, può essere preso in esame un approccio agobioptico. La stadiazione -la cui accuratezza risulta di fondamentale importanza per poter pianificare un adeguato trattamento- viene eseguita attraverso la TC (tomografia computerizzata) di collo, torace, addome e pelvi, con mezzo di contrasto, eventualmente affiancata alla tomografia per emittenti di positroni (PET) con fluorodeossiglucosio (FDG). La PET può talora mettere in luce ipermetabolismo non immediatamente evidenti alla TC rappresentando inoltre uno strumento attraverso il quale stabilire il grado di risposta della malattia all’eventuale terapia messo in atto. La biopsia osteomidollare è sempre richiesta per completare la stadiazione.
Prognosi
La sopravvivenza mediana è di circa 10 anni, ma la gamma è ampia, da meno di un anno a più di 20 anni. Alcuni pazienti possono non necessitare mai di trattamento. Il tasso di sopravvivenza globale a 5 anni è del 72-77%. Il linfoma follicolare è attualmente considerata una patologia e raramente guaribile, spesso favorevolmente responsiva alla prima linea di trattamento, ma clinicamente caratterizzata dalle ricadute successive subentranti. La sopravvivenza globale si misura in anni, in maniera differente sulla base di parametri clinico-laboratoristici che entrando a far parte di modelli prognostici, codificati nel corso dell’ultimo decennio consentono di specificare i pazienti in funzione della prognosi.
Terapia
Se il linfoma è localizzato, deve essere trattato con la radioterapia. Nei linfomi avanzati, è indicata l’immunochemioterapia (rituximab con CHOP). Nel caso di recidive chemiosensibili, il trattamento deve essere intensificato.
Articoli correlati:
Linfoma a grandi cellule B primitivo del mediastino
Linfoma B diffuso a grandi cellule (DLBCL)
Linfomi, per guarirli si punta sull’immunoterapia
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