Sono decenni che milioni di scienziati in tutto il mondo si ingegnano per cercare di fare quel passo decisivo: trovare la famigerata “cura per il cancro”. Negli ultimi mesi un team della Pennsylvania University, diretto da Carl June, ci è andato davvero molto vicino, sfruttando un lato ancora inesplorato dell’immunoterapia: la terapia genica. Si chiama CAR Therapy, scopriamo insieme di cosa si tratta.

Il dottor June ha aperto uno scenario nuovo nel campo della lotta ai tumori: se potessimo insegnare alle cellule del nostro sistema immunitario a riconoscere specifiche molecole espresse da ciascun istotipo umorale, otterremmo un miglioramento della prognosi dei pazienti oncologici? La risposta, stando ai risultati dei suoi studi, è sì. CAR è l’acronimo di Chimeric Antigenic Receptor: un recettore chimerico sintetizzato in laboratorio, che questi ricercatori hanno inserito nel genoma dei linfociti T di pazienti affetti da Leucemia Linfoblastica Acuta, il tumore più comunemente diagnosticato nei bambini: rappresenta infatti circa il 25% delle diagnosi di tumore tra i bambini sotto i 15 anni (questo non rende, ovviamente, gli adulti immuni da tale patologia). La leucemia linfoblastica acuta è un tipo di tumore in cui il midollo osseo produce linfociti immaturi, in quantità eccessive, è rapidamente progressiva ed ovviamente ha i caratteri di malignità. Se non viene trattata può risultare fatale nel giro di pochi mesi; è pertanto fondamentale che i pazienti inizino il trattamento subito dopo la diagnosi. Nei pazienti con leucemia linfoblastica acuta refrattaria a terapia le cellule leucemiche sono ancora presenti nel midollo osseo anche in seguito al trattamento, aumentando considerevolmente il rischio di recidive.

Un vetrino che mostra i linfociti alterati in un paziente affetto da Leucemia Linfoblastica Acuta (ALL)
Un vetrino che mostra i linfociti alterati in un paziente affetto da Leucemia Linfoblastica Acuta (ALL)

Il trial sperimentale prevede il prelievo dei linfociti T del paziente leucemico e la coltivazione dei suddetti in laboratorio per poterli armare della proteina CAR. Per poter “insegnare” ai linfociti ad esprimere CAR è stato necessario l’utilizzo dei Lentivirus, la famiglia che comprende l’HIV. Perché utilizzare un virus potenzialmente così pericoloso? E’ semplice, i Lentivirus sono celebri per la loro capacità di invadere la cellula “ospite” ed integrare il proprio genoma nel genoma dell’ospite, per poi manipolarlo ed indurre la cellula a sintetizzare ciò di cui il virus ha bisogno per infettare il resto dell’organismo. Per rendere il tutto scevro da pericolosi effetti collaterali da infezione, i Lentivirus utilizzati per il trial sono stati opportunamente privati di tutte le componenti con potenziale patogeno, in modo da rendere il virus innocuo: hanno fatto di un virus un vettore ideale per il trasporto del genoma contenente le istruzioni per l’espressione della proteina CAR. I linfociti T modificati prendono il nome di CTL019. Dopo circa dieci giorni di espansione, i CTL019 vengono congelati: nel frattempo, il paziente è sottoposto ad una chemioterapia per abbassare il numero di linfociti T presenti nel sangue, così da far spazio a quelli modificati in laboratorio. Dopodichè si possono trasfondere i CTL019 nel sangue del paziente che entrano in contatto con le cellule tumorali presenti nel sangue e nel midollo osseo e cominciano, da un lato a distruggere le cellule tumorali, dall’altro a riprodursi in maniera esponenziale.

Il fattore davvero limitante, attualmente sono i costi: si parla di circa 600.000 dollari per un ciclo terapeutico completo; questo perché ovviamente il tutto richiede tecniche laboratoristiche avanzate, ma soprattutto perché l’intero processo è ad personam, ogni procedimento va fatto sui linfociti T del paziente, questo non consente una standardizzazione della procedura. Ciononostante la casa farmaceutica Novartis ha investito miliardi di dollari in questo progetto sperimentale, e sembra che nel 2016 sarà approvato come approccio terapeutico standard nella cura della patologia (questo ovviamente farebbe crollare il prezzo esorbitante). E’ stato dichiarato dalla casa farmaceutica, inoltre, la loro intenzione di continuare ad investire per cercare di estendere questo tipo di terapia anche alla cura dei tumori solidi.

Ma cos’ha di diverso dalle precedenti terapie? I linfociti T modificati non interagiscono con le cellule che non sono tumorali, e dunque limitano gli effetti collaterali causati dalle terapie standard. Le cellule tumorali esprimono la proteina CD19 che i linfociti T modificati in laboratorio sono in grado di riconoscere e attaccare con precisione. Si scatena così una vera e propria caccia all’uomo, perché i CTL019 reintrodotti nel sangue del paziente vanno immediatamente incontro ad una proliferazione massiva, per cui si otterrà un vero e proprio esercito con un comune target: la proteina CD19. Di conseguenza gli effetti collaterali, a lungo termine, sembrano essere del tutto trascurabili, quindi un paziente (il più delle volte pediatrico) con un’aspettativa di vita di pochi mesi, grazie a questa terapia, può aspirare ad avere una lunga vita, assolutamente normale.

Ma adesso vediamo i risultati di questi trial clinici. Gli studi del dottor June cominciarono negli anni ’80; però è stato possibile somministrare il trattamento sperimentale ad un paziente solo nel 2010, trent’anni dopo. E’ stato un ex soldato di 60 anni affetto da leucemia in fase terminale. Sono passati solo quattro anni da quando Bill, il paziente numero uno, ed Emily (una bambina di nove anni che vive da due anni completamente guarita dalla Leucemia Linfoblastica Acuta), la prima bambina a sperimentare la terapia, sono stati curati. I dati epidemiologici dimostrano che nell’ambito delle Leucemie Acute si è avuto tra il 90 ed il 100% di remissione della patologia (ricordiamo che prima della terapia CAR la prognosi era estremamente infausta), si sono registrati casi di recidive ma i risultati sono stati così sorprendentemente positivi che si è parlato di “rivoluzione scientifica”. Diverso è il caso del trattamento delle Leucemie Croniche, secondo i più recenti studi, in questi pazienti si è registrato il 50% di remissione della patologia, risultati non incredibili come nel trattamento delle Acute, ma che lasciano comunque speranza nettamente più concreta rispetto alle terapie standard.

In questi mesi abbiamo assistito a passi da gigante nell’ambito della terapia oncologica, questa nuova scoperta potrebbe cambiare per sempre il destino di moltissimi pazienti terminali, offrendo una prognosi completamente diversa ed una qualità di vita nettamente superiore a quella dei pazienti trattati con le terapie attuali.

Non abbiamo ancora vinto la guerra, ma in questi mesi è stato scritto un altro, importantissimo, capitolo della storia della medicina.

Vicepresidente | Nata a Napoli il 25 Giugno 1992. Ho frequentato l'università degli studi della Campania "Luigi Vanvitelli" laureandomi in Medicina e Chirurgia nel 2017. Attualmente sono una specializzanda in Ostetricia e Ginecologia presso l'Università degli studi di Torino. Mi occupo di coordinare la Redazione de "La Medicina In Uno Scatto", di cui sono anche Vicepresidente.

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