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-HodgkinÂ
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 mantellareÂ
- 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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