mcl
Table of Contents
Mantelcellymfoom
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1 Verplicht aanvullend onderzoek
- laboratoriumonderzoek inclusief M-proteine
- PET-CT
- beenmergonderzoek
- aaIPI
- bij een aaIPI >1 wordt soms de therapie geintensiveerd
- criteria:
- stadium III/IV
- performance status >2
- verhoogd LDH
- IPI, verplicht als kwaliteitscriterium bij statusvoering
- kent dezelfde punten als aaIPI, plus
- >60 jaar oud
- >1 extranodale lokalisatie
- kent dezelfde punten als aaIPI, plus
1.1 PET-CT
- wordt verricht als eindevaluatie
- zo mogelijk ook voor start therapie (en voor start prednison)
- Deauville-score
- no uptake or no residual uptake (when used interim)
- slight uptake, but below blood pool (mediastinum)
- uptake above mediastinal, but below or equal to uptake in the liver
- uptake slightly to moderately higher than liver
- markedly increased uptake or any new lesion (on response evaluation)
- Deauville t/m 3 geldt als mCR bij eindevaluatie 1
2 Therapie
2.1 eerste lijn
- stadium I: 3x R-CHOP-14 + IF-RT (30 Gy)
- stadium >I:
- indolent MCL: expectatief
- Ki-67 < 30%, geen blastoide of pleiomorfe histologie, maximale tumor diameter < 3 cm, normaal LDH en beta2microglobuline, geen B-symptomen.2
- <65-70: R-CHOP alternerend met R-DHAP (totaal 6 kuren) gevolgd door autologe stamceltransplantatie3 en rituximab-onderhoud bij PR of CR, 1x per 2 maanden, gedurende 3 jaar4
- >65-70:
- 8x R-CHOP-21 gevolgd door 2 jaar rituximabonderhoud1
- BR zonder R-onderhoud
- indolent MCL: expectatief
2.2 recidief
2.2.1 algemeen
- symptomatische progressie pas indicatie therapie
- indien geschikte kandidaat bij respons op tweedelijns therapie: allogene stamceltransplantatie1
- recidiefkans 26%, NRM 28%, 5-jr OS 55% en 10-jr 45%
2.2.2 geaccepteerde opties voor de tweedelijn
- R-bendamustine
- R-FC
- gekenmerkt door hematotoxiciteit en hoge incidentie ernstige infecties
- response rate 75%, mediane duur 11 maanden7
- 4x R-FCM
- response rate 58%, PFS 8 maanden8
- ibrutinib 1dd 560 mg tot progressie
- bij voorkeur pas bij chemo-refractaire patiƫnten in 2e of latere lijn
- RR 72%, PFS 14 maanden9
2.2.3 andere opties, meestal voor 3e of latere lijn
2.2.4 palliatief
- chloorambucil lage dosis continu
- palliatieve lokale radiotherapie / steroiden
Footnotes:
Vaughn JE, Sorror ML, Storer BE, et al. Long-term sustained disease control in patients with mantle cell lymphoma with or without active disease after treatment with allogeneic hematopoietic cell transplantation after nonmyeloablative conditioning. Cancer. 2015;121(20):3709-16. Robinson S, Dreger P, Caballero D, et al. The EBMT/EMCL consensus project on the role of autologous and allogeneic stem cell transplantation in mantle cell lymphoma. Leukemia. 2015;29(2):464-73.
- Cohen. Deferred therapy is associated with improved overall survival in patients with newly diagnosed mantle cell lymphoma. Cancer. 2016.
- Cheah. Mantle Cell Lymphoma. J Clin Oncol. 2016;34(11):1256-69.
Hermine. Addition of high-dose cytarabine to immunochemotherapy before autologous stem-cell transplantation in patients aged 65 years or younger with mantle cell lymphoma (MCL Younger): a randomised, open-label, phase 3 trial of the European Mantle Cell Lymphoma Network. Lancet. 2016;388(10044):565-75.
Le Gouill. Rituximab after Autologous Stem-Cell Transplantation in Mantle-Cell Lymphoma. N Engl J Med. 2017;377(13):1250-60.
Czuczman MS, Kahanic S, Forero A, et al. Results of a phase II study of bendamustine and ofatumumab in untreated indolent B cell non-Hodgkin's lymphoma. Ann Hematol. 2015;94(4):633-41. 75% na R-CHOP Czuczman. Phase II study of bendamustine combined with rituximab in relapsed/refractory mantle cell lymphoma: efficacy, tolerability, and safety findings. Ann Hematol. 2015 Dec;94(12):2025-32.
Rummel M, Kaiser U, Balser C, et al. Bendamustine plus rituximab versus fludarabine plus rituximab for patients with relapsed indolent and mantle-cell lymphomas: a multicentre, randomised, open -label, non-inferiority phase 3 trial. Lancet Oncol. 2016;17(1):57-66.
Thomas DW, Owen RG, Johnson SA, et al. Superior quality and duration of responses among patients with mantle-cell lymphoma treated with fludarabine and cyclophosphamide with or without rituximab compared with prior responses to CHOP. Leuk Lymphoma. 2005;46(4):549-52.
Forstpointner R, Dreyling M, Repp R, et al. The addition of rituximab to a combination of fludarabine, cyclophosphamide, mitoxantrone (FCM) significantly increases the response rate and prolongs survival as compared with FCM alone in patients with relapsed and refractory follicular and mantle cell lymphomas: results of a prospective randomized study of the German Low-Grade Lymphoma Study Group. Blood. 2004;104(10):3064-71.
Dreyling M, Jurczak W, Jerkeman M, et al. Ibrutinib versus temsirolimus in patients with relapsed or refractory mantle-cell lymphoma: an international, randomised, open-label, phase 3 study. Lancet. 2016;387(10020):770-8.
Trneny M, Lamy T, Walewski J, et al. Lenalidomide versus investigator's choice in relapsed or refractory mantle cell lymphoma (MCL-002; SPRINT): a phase 2, randomised, multicentre trial. Lancet Oncol. 2016;17(3):319-31.
O'Connor OA, Moskowitz C, Portlock C, et al. Patients with chemotherapy-refractory mantle cell lymphoma experience high response rates and identical progression-free survivals compared with patients with relapsed disease following treatment with single agent bortezomib: results of a multicentre Phase 2 clinical trial. Br J Haematol. 2009;145(1):34-9