Mantle Cell Lymphoma is like Non hodgkin lymphoma with a twist
CD20 and CD 5 positive but no CD23 (CLL) nor CD 10 (follicular)
could be diffusely involving the Bowel (Lymphomatous polyposis)
and 20% CNS infiltrating as a late presentation
Cyclin D1 driven condition leading to incurable disease
with Median survival of 3-4 years
Now Ki-67 has been found to be prognotic
Treatment
1.R-CHOP
can be given
but remember here the Median survival is 20 months
It got to be followed by Maintenance Rituxan or transplant
so no good option, except may be in elderly and desperate
2. Most people would try R- HYPERCVAD, or R-Modified HYPERCVAD to make it palatable !
3. after HYPERCVAD comes
Rituxan-Fludarabine Mitoxantrone-Cytoxan similar to CLL.
Intereferon has been given in Maintenance setting, but perception is that Rituxan Maintenance is better..
4.Now comes Bendamustin and Rituxan superior to R-CHOP
CR 50% VS 27%
Overall Resp 94% Vs 85%
Nausea and hypersensitivity Alopecia,neuropathy, constipation
seems like for older patient R-HYPERCVAD folloed by Transplant
whereas Older patient will get more and more Bendamustine-Rituxan
5.Transplant in first remission beter than later!
6.New Active drug and valid option in refractory disease
-----Velcade RR 33% (prompting Velcade-Bendamustine trial)
-----Revlimid RR 28%
-----Temsorilimus response but not sustained
-----Ibrutinib is shaking things up now, at 560 mg, RR 65-75%, shaking things up so much that it is now being combined to -CHOP, Threatening to become standard therapy?
------And here come Idelalisib PI3K oral Inhibitor ?RR 52%
----------ABT-199 a BCL 2 inhibitor (watch for tumor lysis syndrome)
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THE WORLD OF ONCOLOGY IS BEING MOVED BY TARGET THERAPY
OH! DON'T FORGET REDUCED INTENSITY ALLOGENEIC TRANSPLANT IN THIS DISEASE, SOMEONE REPORTED 60-80% 2 YEARS SURVIVAL "IN SELECTED SERIES"
LOT OF EXCITING STUFF IN THIS DISEASE!
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