Linfomi non-Hodgkin
I linfomi non-Hodgkin (LNH) sono tumori clonali B dei linfociti B, T e natural killer (NK), i quali possono insorgere sia in linfociti immaturi o precursori, sia in linfociti maturi o periferici. I linfomi B e T linfocitari tendono a riprodurre le caratteristiche morfologiche, fenotipiche e, talora funzionali della tappa ontogenetica nella quale la neoplasia si è sviluppata e possono essere classificati sulla base della controparte cellulare normale da cui originano. I linfomi non-Hodgkin si differenziano dai linfomi di Hodgkin perché privi della cellula di Reed-Sternberg, una cellula gigante caratterizzata da due nuclei distinti, che è l’elemento distintivo dei linfomi del secondo tipo.
Incidenza dei linfomi non-Hodgkin
A livello mondiale, linfomi sono al sesto posto fra le neoplasie, per incidenza. Mentre la frequenza del linfoma di Hodgking è rimasta stabile, con un tipico andamento stagionale, i linfomi non-Hodgkin hanno riconosciuto nel corso degli ultimi vent’anni un aumento esponenziale: tale tendenza è destinata a mantenersi o, più probabilmente a subire un’ ulteriore progressione nel prossimo mezzo secolo. Al momento, il rapporto fra le due forme non-hodgkiniane e quelle hodgkiniane è di 10/1. Nel complesso i linfomi non- Hodgkin rappresentano circa il 4% di tutti i tumori, con un’ incidenza globale di circa 35 nuovi casi/100.000 abitanti/anno. Essi sono più frequenti nei paesi sviluppati, con un’ incidenza che aumenta in maniera esponenziale con l’età, anche se in modo differenziato per le varie categorie di linfoma. Infatti, in età pediatrica sono più frequenti i linfomi che derivano dai precursori, mentre le forme mature a decorso clinico indolente colpiscono in prevalenza gli adulti-anziani (5ª- 7ª), e quelli maturi aggressivi più spesso i giovani adulti (3ª-4ª) pur potendo interessare tutte le fasce di età. I linfomi che originano dai B linfociti rappresentano oltre il 90% di tutti i linfomi, con prevalenza delle forme dei linfociti B maturi. L’incidenza dei singoli tipi di linfomi non-Hodgkin varia nelle diverse aree geografiche: il linfoma follicolare ad esempio, è più frequente in Europa occidentale e negli Stati Uniti e meno frequente in Sud america, Africa e Asia.
Eziopatogenesi
Le neoplasie linfoidi, come tutti i tumori, insorgono come conseguenza delle interazioni tra ambiente e fattori genetici, anche se l’eziologia rimane a tutt’oggi sconosciuta per molte categorie. Tuttavia, batteri o virus, farmaci, agenti chimici, radiazioni ionizzanti e ultravioletti sono stati associati allo sviluppo dei linfomi. In particolare, è stato dimostrato che gli agenti infettivi svolgono un importante ruolo nello sviluppo di alcuni tipi di linfoma, sia B cheT.
Il virus di Epstein-Barr (EBV) è presente nel 100% dei casi di linfoma di Burkitt endemico africano e nel 15-35% delle forme sporadiche e HIV associate; esso inoltre è coinvolto nella patogenesi delle proliferazioni linfocitarie B dei soggetti con immunodepressione (congenita o acquisita a seguito di terapie immunosoppressive – per trapianti o malattie autoimmuni- o per aging del sistema immunitario come succede nei linfomi B a grandi cellule dell’età senile). Alcune malattie autoimmuni sono state infatti associate ad un aumentato incidenza del linfoma, come accade nella:
- Tiroide di hashimoto
- Sindrome di Sjögren
- Enteropatia da ipersensibilità al glutine
- Lupus eritematoso sistemico
- Artrite reumatoide
- Sindrome linfoproliferativa autoimmune.
Il virus dell’epatite C, particolarmente diffuso in italia, è stato implicato nell’ insorgenza dei linfomi da linfociti B maturi della zona marginale ad insorgenza splenica.
Ormai definita è ad esempio la correlazione patogenetica tra il linfoma di derivazione dai linfociti B maturi della zona marginale ad insorgenza extra-nodale e la prolungata infezione da Helicobacter pylori per la forma gastrica, a Borrelia burgdorferi e da Chlamydia psittaci rispettivamente per gli interessamenti cutaneo ed oculare. Campylocbater per la forma intestinale associata a malattia delle catene pesanti.
Classificazione clinica dei linfomi non-Hodgkin
LINFOMI B AGGRESSIVI
Nodali:
- Linfoma B diffuso a grandi cellule (DLBCL)
- Linfoma di Burkitt
- Linfoma primitivo del mediastino
- Linfoma mantellare
- Linfoma del testicolo
Extranodali:
- Linfoma del SNC
- Linfoma del testicolo
LINFOMI B INDOLENTI
Nodali:
- Linfoma follicolare
- Linfoma linfoplasmocitico
- Linfoma linfocitico
- Linfoma marginale nodale
- Linfoma marginale splenico
Extranodale:
- Linfoma marginale extranodale (MALT)
- Linfoma follicolare della cute
- Linfoma follicolare dell’intestino
LINFOMI DI DERIVAZIONE DEI LINFOCITI T
Nodali:
- Linfoma anaplastico a grandi cellule
- Linfoma anaplastico a grandi cellule (ALCL)
- Linfoma T periferico non altrimenti specificato
- Linfoma angioimmunoblastico
Extranodali:
- Micosi fungoide
- Sindrome di Sèzary
Articoli correlati:
(Bibl. Corso di malattie del sangue e degli organi linfopoietici, Sante Sura, Settembre 2015)
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