Linfoma mantellare
Table of Contents
Il linfoma mantellare (Mantle-cell-lynphoma-MCL)è una neoplasia ematologica relativamente rara, rappresentando circa il 6% di tutti i linfominon Hodgkincon un’incidenza di circa 300 nuovi casi diagnosticati in Italia ogni anno. L’età di insorgenza del linfoma mantellare è 65 anni con un rapporto M:F da 3 a 4:1. Il linfoma mantellare ha un decorso clinico aggressivo ma negli ultimi anni la risposta clinica e la sopravvivenza sono migliorate grazie ai progressi in campo biologico, biotraslocazionale e clinico.
Manifestazioni cliniche del linfoma mantellare
Il linfoma mantellare solitamente si presenta alla diagnosi già instadio avanzato(III, IV soprattutto con interessamento midollare) con estesocoinvolgimento extranodale(tratto gastroentrico e reni) linfonodale, meno frequentemente con splenomegalia e interessamento del sangue periferico. Nel tratto gastrointestinale la presentazione assume una forma clinica definita“poliposi linfomatoide” nella quale si repertano endoscopicamente numerose formazioni polipoidi a carico della mucosa dovuti alla crescita linfomatosa nella lamina propria estesa lungo i segmenti delcolon, tenue e digiuno,tali da mimare, in assenza di conferma istologica, i comunipolipidel tratto gastroenterico; l’anemiae la pancitopenia non sono rare, così come i sintomi sistemici e il rialzo di alcuni indicatori di attività tumorali quali:LDH VES e PCR.Meno frequentemente sono coinvolti lacute, il polmone e la mammella.L’interessamento del sistema nervoso centrale è riscontrato in circa il 20% dei pazienti in fase di ricaduta della malattia.
Indagini strumentali
La diagnosi viene posta attraverso labiopsia linfonodaledeve essere completata da una precisa valutazione dell’estensione della malattia.
Le indagini strumentali e essenziali per eseguire una corretta stadiazione sono:
- TC- total body
- PET -FDG
- Biopsia osteomidollare.
A completamento sono inoltre necessarie:
- Colonscopia
- Gastroscopia con biopsie multiple.
Analisi di laboratorio
All’esame emocromocitometrico si può dimostrare una lieve anemia normocromica e normocitica. Spesso concomita con unalinfocitosi monoclonale, espressione leucemica della malattia. La VES è costantemente elevata. L’ LDH è elevato nella maggior parte dei casi.
Istopatologia
Il linfoma mantellare deriva dailinfociti B periferici della zona mantellare del follicolo linfoide.Tale neoplasia si compone di linfociti di aspetto monomorfo, piccolo/medi, con nucleo a profilo irregolare che, in una fase iniziale di espansione, mostra una crescita a manicotto attorno a centri germinativi reattivi (cosiddetta crescitamantle zone), che poi diviene diffusa con la progressione del processo. Laddove le cellule abbiano dimensioni medio/grandi e/o aspetto polimorfo con elevato numero di mitosi, si configurano le varietàblastoideepolimorfa,entrambe caratterizzate da particolare aggressività clinica. Al contrario è stata più recentemente riconosciuta una varietà a piccole cellule. Questa varietà, pur essendo a tutti gli effetti un NCL, riconosce un andamento clinico più favorevole: identificare tale categoria di pazienti è cruciale per evitare incauti trattamenti o , favorire un atteggiamento inizialmente solo osservativo.
Prognosi
La prognosi complessiva è infausta, con unasopravvivenza media di 3-4 anni alla diagnosi. Il trattamento di prima linea nei pazienti affetti da linfoma mantellare differisce in base all’età e alle condizioni cliniche generali. I pazienti giovani, in buone condizioni generali, sono candidati a ricevere untrattamento immunochemioterapico,contenente antraciclina seguito dalla somministrazione dicitarabinaad alte dosi per arrivare al trapianto autologo.
Terapia
Ilpaziente con età inferiore ai 65 annie in buone condizioni cliniche viene avviato in prima linea 4-6 cicli di chemioterapia contenente citarabina (DHAP) seguiti dal trapianto autologo, al fine di garantire il miglior risultato in termini di risposta completa e di sopravvivenza. Nei pazienti con età maggiore di 65 anni si utilizzanochemioterapiequali CHPO obendamustinain associazione all’ anticorpo monoclonalerituximab.L’obiettivo del trattamento è quello di ridurre il più possibile la massa neoplastica, al fine di migliorare l’aspettativa di vita.
(Bibl. Corso di malattie del sangue e degli organi linfopoietici, Sante Sura, Settembre 2015)
ARTICOLI CORRELATI:
Linfoma B diffuso a grandi cellule (DLBCL)
Linfoma a grandi cellule B primitivo del mediastino
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