Marginal zone lymphoma
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Bortezomib monotherapy
Level of Evidence: Phase II
Regimen #1, Troch, et al. 2009
- Bortezomib (Velcade) 1.5 mg/m2 IV bolus on days 1, 4, 8, 11
21-day cycles x up to 8 cycles
Supportive medications:
- 5-HT3 antagonist IV immediately before bortezomib
- 500 mL NS after bortezomib
Regimen #2, Conconi, et al. 2011
- Bortezomib (Velcade) 1.3 mg/m2 IV on days 1, 4, 8, 11
21-day cycles x up to 6 cycles
Supportive medications:
- No routine growth factors, antibiotic, or antiviral prophylaxis was given
References
- Troch M, Jonak C, Müllauer L, Püspök A, Formanek M, Hauff W, Zielinski CC, Chott A, Raderer M. A phase II study of bortezomib in patients with MALT lymphoma. Haematologica. 2009 May;94(5):738-42. doi:10.3324/haematol.2008.001537. Epub 2009 Mar 31. link to original article contains verified protocol PubMed
- Conconi A, Martinelli G, Lopez-Guillermo A, Zinzani PL, Ferreri AJ, Rigacci L, Devizzi L, Vitolo U, Luminari S, Cavalli F, Zucca E; International Extranodal Lymphoma Study Group (IELSG). Clinical activity of bortezomib in relapsed/refractory MALT lymphomas: results of a phase II study of the International Extranodal Lymphoma Study Group (IELSG). Ann Oncol. 2011 Mar;22(3):689-95. link to original article contains verified protocol PubMed
Chlorambucil monotherapy
Regimen #1, Leblond, et al. 2013
- Chlorambucil (Leukeran) 8 mg/m2 (6 mg/m2 per day if older than 75 years old) PO daily on days 1-10
28-day cycles x up to 12 cycles
Supportive medications:
- Recommended PCP prophylaxis with one of the following:
- Trimethoprim/Sulfamethoxazole (Bactrim SS) 1 tablet PO daily
- Pentamidine (Nebupent) 300 mg inhaled monthly
Regimen #2, Zucca, et al. 2013 (IELSG-19)
- Chlorambucil (Leukeran) 6 mg/m2 PO daily
42-days continuously followed by re-assessment; SD & responders -> 14-days out of 28-day cycles up to 4 more cycles
References
- Leblond V, Johnson S, Chevret S, Copplestone A, Rule S, Tournilhac O, Seymour JF, Patmore RD, Wright D, Morel P, Dilhuydy MS, Willoughby S, Dartigeas C, Malphettes M, Royer B, Ewings M, Pratt G, Lejeune J, Nguyen-Khac F, Choquet S, Owen RG. Results of a randomized trial of chlorambucil versus fludarabine for patients with untreated waldenstrom macroglobulinemia, marginal zone lymphoma, or lymphoplasmacytic lymphoma. J Clin Oncol. 2013 Jan 20;31(3):301-7. doi:10.1200/JCO.2012.44.7920. Epub 2012 Dec 10. link to original article contains verified protocol PubMed
- Zucca E, Conconi A, Laszlo D, López-Guillermo A, Bouabdallah R, Coiffier B, Sebban C, Jardin F, Vitolo U, Morschhauser F, Pileri SA, Copie-Bergman C, Campo E, Jack A, Floriani I, Johnson P, Martelli M, Cavalli F, Martinelli G, Thieblemont C. Addition of Rituximab to Chlorambucil Produces Superior Event-Free Survival in the Treatment of Patients With Extranodal Marginal-Zone B-Cell Lymphoma: 5-Year Analysis of the IELSG-19 Randomized Study. J Clin Oncol. 2013 Feb 10;31(5):565-72. doi: 10.1200/JCO.2011.40.6272. Epub 2013 Jan 7. link to original article contains verified protocol PubMed
Cladribine monotherapy
Level of Evidence: Phase II
Regimen
- Cladribine (Leustatin) 0.12 mg/kg IV over 2 hours on days 1 to 5
28-day cycles x 4 to 6 cycles
Dose reductions:
- "In case of a persisting nadir of the WBCs ≤ 4.0 × 10^9/L (or neutrophils ≤ 1.5 × 10^9/L) and/or the platelets ≤ 100 × 10^9/L, the next treatment cycle was delayed by 1 week until achieving normal values and then treatment was administered at a reduced dose of 0.1 mg/kg body weight."
Supportive medications:
- Not described
References
- Jäger G, Neumeister P, Brezinschek R, Hinterleitner T, Fiebiger W, Penz M, Neumann HJ, Mlineritsch B, DeSantis M, Quehenberger F, Chott A, Beham-Schmid C, Höfler G, Linkesch W, Raderer M. Treatment of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type with cladribine: a phase II study. J Clin Oncol. 2002 Sep 15;20(18):3872-7. link to original article contains verified protocol PubMed
Fludarabine monotherapy
Regimen
- Fludarabine (Fludara) 40 mg/m2 (30 mg/m2 per day if older than 75 years old) PO daily on days 1-5
28-day cycles x up to 6 cycles
Supportive medications:
- Recommended PCP prophylaxis with one of the following:
- Trimethoprim/Sulfamethoxazole (Bactrim SS) 1 tablet PO daily
- Pentamidine (Nebupent) 300 mg inhaled monthly
- Herpes zoster prophylaxis with one of the following:
- Valacyclovir (Valtrex) 500 mg PO daily
- Acyclovir (Zovirax) 200-400 mg PO BID
References
- Leblond V, Johnson S, Chevret S, Copplestone A, Rule S, Tournilhac O, Seymour JF, Patmore RD, Wright D, Morel P, Dilhuydy MS, Willoughby S, Dartigeas C, Malphettes M, Royer B, Ewings M, Pratt G, Lejeune J, Nguyen-Khac F, Choquet S, Owen RG. Results of a randomized trial of chlorambucil versus fludarabine for patients with untreated waldenstrom macroglobulinemia, marginal zone lymphoma, or lymphoplasmacytic lymphoma. J Clin Oncol. 2013 Jan 20;31(3):301-7. doi:10.1200/JCO.2012.44.7920. Epub 2012 Dec 10. link to original article contains verified protocol PubMed
MCP
MCP: Mitoxantrone, Chlorambucil, Prednisolone
Regimen
- Mitoxantrone (Novantrone) 8 mg/m2 IV on days 1 & 2
- Chlorambucil (Leukeran) 3 mg/m2 PO TID (written in the reference as "3 x 3 mg/m2"; total dose per day is 9 mg/m2) on days 1-5
- Prednisolone (Millipred) 25 mg/m2 PO/IV on days 1-5
28-day cycles x up to 8 cycles
References
- Wöhrer S, Drach J, Hejna M, Scheithauer W, Dirisamer A, Püspök A, Chott A, Raderer M. Treatment of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) with mitoxantrone, chlorambucil and prednisone (MCP). Ann Oncol. 2003 Dec;14(12):1758-61. link to orginal article contains verified protocol PubMed
R-Flu
R-Flu: Rituximab, Fludarabine
Regimen
- Rituximab (Rituxan) 375 mg/m2 IV on day 1
- Fludarabine (Fludara) 25 mg/m2 IV (or 40 mg/m2 PO) on days 1-5
- Patients with gastric lymphoma received the cycle 1 dose intravenously to guarantee drug absorption
- Patients >70 years old could receive fludarabine at the above dose, but only on days 1-3
28-day cycles for 4-6 cycles
References
- Salar A, Domingo-Domenech E, Estany C, Canales MA, Gallardo F, Servitje O, Fraile G, Montalbán C. Combination therapy with rituximab and intravenous or oral fludarabine in the first-line, systemic treatment of patients with extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type. Cancer. 2009 Nov 15;115(22):5210-7. doi: 10.1002/cncr.24605. link to orginal article contains verified protocol PubMed
Rituximab monotherapy
Level of Evidence: Phase II
Regimen
- Rituximab (Rituxan) 375 mg/m2 IV on days 1, 8, 15, 21
References
- Conconi A, Martinelli G, Thiéblemont C, Ferreri AJ, Devizzi L, Peccatori F, Ponzoni M, Pedrinis E, Dell'Oro S, Pruneri G, Filipazzi V, Dietrich PY, Gianni AM, Coiffier B, Cavalli F, Zucca E. Clinical activity of rituximab in extranodal marginal zone B-cell lymphoma of MALT type. Blood. 2003 Oct 15;102(8):2741-5. link to orginal article contains protocol PubMed
- Martinelli G, Laszlo D, Ferreri AJ, Pruneri G, Ponzoni M, Conconi A, Crosta C, Pedrinis E, Bertoni F, Calabrese L, Zucca E. Clinical activity of rituximab in gastric marginal zone non-Hodgkin's lymphoma resistant to or not eligible for anti-Helicobacter pylori therapy. J Clin Oncol. 2005 Mar 20;23(9):1979-83. link to original article contains protocol PubMed