B-cell lymphoma of mucosa-associated lymphoid tissue

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Are you looking for a regimen but can't find it here? It is possible that we've moved it to the historical regimens page. For placebo or observational studies in this condition, please visit this page. If you still can't find it, please let us know so we can add it!
Note: these are regimens specific to extranodal marginal zone lymphomas (EMZL) of mucosa-associated lymphoid tissue (MALT lymphomas), please see the main MZL page for other regimens.
Last updated on 2024-09-06:25 regimens on this page
26 variants on this page
=Guidelines=
Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.
==EGILS==
*2011: Ruskoné-Fourmestraux et al. EGILS consensus report. Gastric extranodal marginal zone B-cell lymphoma of MALT EuroPMC
==ESMO==
*2020: Zucca et al. Marginal zone lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up EuroPMC
*2013: Dreyling et al. ESMO Consensus conferences: guidelines on malignant lymphoma. part 2: marginal zone lymphoma, mantle cell lymphoma, peripheral T-cell lymphoma EuroPMC
*2013: Zucca et al. Gastric marginal zone lymphoma of MALT type: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up EuroPMC
**2010: Zucca & Dreyling. Gastric marginal zone lymphoma of MALT type: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up EuroPMC
**2009: Zucca & Dreyling. Gastric marginal zone lymphoma of MALT type: ESMO Clinical Recommendations for diagnosis, treatment and follow-up EuroPMC
**2008: Zucca & Dreyling. Gastric marginal zone lymphoma of MALT type: ESMO clinical recommendations for diagnosis, treatment and follow-up EuroPMC
**2007: Zucca. Gastric marginal zone lymphoma of mucosa-associated lymphoid tissue type: ESMO clinical recommendations for diagnosis, treatment and follow-up EuroPMC
==French Intergroup==
*2017: Matysiak-Budnik et al. Gastrointestinal lymphomas: French Intergroup clinical practice recommendations for diagnosis, treatment and follow-up (SNFGE, FFCD, GERCOR, UNICANCER, SFCD, SFED, SFRO, SFH) EuroPMC
==NCCN==
*NCCN does not currently have guidelines at this granular level; please see NCCN Guidelines - B-cell Lymphomas.
=Gastric MALT lymphoma, H. Pylori eradication therapy=
==Amoxicillin & Omemprazole==
===Regimen===
Study 	Dates of enrollment	Evidence
Bayerdörffer et al. 1995	Not reported 	Non-randomized
====Antibiotic therapy====
*Amoxicillin 750 mg PO three times per day
*Omeprazole (Prilosec) 40 mg PO three times per day
14-day course
===References===
# Bayerdörffer E, Neubauer A, Rudolph B, Thiede C, Lehn N, Eidt S, Stolte M; MALT Lymphoma Study Group. Regression of primary gastric lymphoma of mucosa-associated lymphoid tissue type after cure of Helicobacter pylori infection. Lancet. 1995 Jun 24;345(8965):1591-4. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
## Update: Neubauer A, Thiede C, Morgner A, Alpen B, Ritter M, Neubauer B, Wündisch T, Ehninger G, Stolte M, Bayerdörffer E. Cure of Helicobacter pylori infection and duration of remission of low-grade gastric mucosa-associated lymphoid tissue lymphoma. J Natl Cancer Inst. 1997 Sep 17;89(18):1350-5. link to original article EuroPMC
## Update: Wündisch T, Thiede C, Morgner A, Dempfle A, Günther A, Liu H, Ye H, Du MQ, Kim TD, Bayerdörffer E, Stolte M, Neubauer A. Long-term follow-up of gastric MALT lymphoma after Helicobacter pylori eradication. J Clin Oncol. 2005 Nov 1;23(31):8018-24. Epub 2005 Oct 3. link to original article EuroPMC
==Amoxicillin, Clarithromycin, PPI, Bismuth==
===Regimen===
Study 	Dates of enrollment 	Evidence
Steinbach et al. 1999	Not reported to 1997-05	Non-randomized
====Antibiotic therapy====
*Amoxicillin 750 mg PO three times per day on days 1 to 21
*Clarithromycin (Biaxin) 500 mg PO three times per day on days 1 to 21
*One of the following PPIs:
**Omeprazole (Prilosec) 20 mg PO twice per day on days 1 to 21
**Lansoprazole (Prevacid) 30 mg PO twice per day on days 1 to 21
*Bismuth subsalicylate (Pepto-Bismol) 2 tabs PO four times per day on days 1 to 21
8-week cycle for 2 cycles
===References===
#Steinbach G, Ford R, Glober G, Sample D, Hagemeister FB, Lynch PM, McLaughlin PW, Rodriguez MA, Romaguera JE, Sarris AH, Younes A, Luthra R, Manning JT, Johnson CM, Lahoti S, Shen Y, Lee JE, Winn RJ, Genta RM, Graham DY, Cabanillas FF. Antibiotic treatment of gastric lymphoma of mucosa-associated lymphoid tissue: an uncontrolled trial. Ann Intern Med. 1999 Jul 20;131(2):88-95. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
==Ampicillin, Metronidazole, Bismuth==
===Regimen===
Study 	Dates of enrollment	Evidence
Wotherspoon et al. 1993	Not reported 	Pilot
====Antibiotic therapy====
*Ampicillin
*Metronidazole (Flagyl)
*Tripotassium dicitratobismuthate (De-Noltab)
===References===
# Wotherspoon AC, Doglioni C, Diss TC, Pan L, Moschini A, de Boni M, Isaacson PG. Regression of primary low-grade B-cell gastric lymphoma of mucosa-associated lymphoid tissue type after eradication of Helicobacter pylori. Lancet. 1993 Sep 4;342(8871):575-7. link to original article does not contain dosing details EuroPMC
==Ampicillin & Omeprazole==
===Regimen===
Study 	Dates of enrollment	Evidence
Wotherspoon et al. 1993	Not reported 	Pilot
====Antibiotic therapy====
*Ampicillin
*Omeprazole (Prilosec)
===References===
# Wotherspoon AC, Doglioni C, Diss TC, Pan L, Moschini A, de Boni M, Isaacson PG. Regression of primary low-grade B-cell gastric lymphoma of mucosa-associated lymphoid tissue type after eradication of Helicobacter pylori. Lancet. 1993 Sep 4;342(8871):575-7. link to original article does not contain dosing details EuroPMC
==Clarithromycin, Metronidzole, PPI==
===Regimen===
Study 	Dates of enrollment	Evidence
Bayerdörffer et al. 1995	Not reported 	Non-randomized
Note: This was second-line treatment.
====Antibiotic therapy====
*Clarithromycin (Biaxin) 250 mg PO twice per day
*Metronidazole (Flagyl) 400 mg PO three times per day
*Omeprazole (Prilosec) 40 mg PO twice per day
10-day course
===References===
# Bayerdörffer E, Neubauer A, Rudolph B, Thiede C, Lehn N, Eidt S, Stolte M; MALT Lymphoma Study Group. Regression of primary gastric lymphoma of mucosa-associated lymphoid tissue type after cure of Helicobacter pylori infection. Lancet. 1995 Jun 24;345(8965):1591-4. link to original article EuroPMC
## Update: Neubauer A, Thiede C, Morgner A, Alpen B, Ritter M, Neubauer B, Wündisch T, Ehninger G, Stolte M, Bayerdörffer E. Cure of Helicobacter pylori infection and duration of remission of low-grade gastric mucosa-associated lymphoid tissue lymphoma. J Natl Cancer Inst. 1997 Sep 17;89(18):1350-5. link to original article EuroPMC
## Update: Wündisch T, Thiede C, Morgner A, Dempfle A, Günther A, Liu H, Ye H, Du MQ, Kim TD, Bayerdörffer E, Stolte M, Neubauer A. Long-term follow-up of gastric MALT lymphoma after Helicobacter pylori eradication. J Clin Oncol. 2005 Nov 1;23(31):8018-24. Epub 2005 Oct 3. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
==Clarithromycin, Tetracycline, PPI, Bismuth==
===Regimen===
Study 	Dates of enrollment 	Evidence
Steinbach et al. 1999	Not reported to 1997-05	Non-randomized
====Antibiotic therapy====
*Clarithromycin (Biaxin) 500 mg PO three times per day on days 1 to 21
*Tetracycline (Sumycin) 500 mg PO four times per day on days 1 to 21
*One of the following PPIs:
**Omeprazole (Prilosec) 20 mg PO twice per day on days 1 to 21
**Lansoprazole (Prevacid) 30 mg PO twice per day on days 1 to 21
*Bismuth subsalicylate (Pepto-Bismol) 2 tabs PO four times per day on days 1 to 21
8-week cycle for 2 cycles
===References===
#Steinbach G, Ford R, Glober G, Sample D, Hagemeister FB, Lynch PM, McLaughlin PW, Rodriguez MA, Romaguera JE, Sarris AH, Younes A, Luthra R, Manning JT, Johnson CM, Lahoti S, Shen Y, Lee JE, Winn RJ, Genta RM, Graham DY, Cabanillas FF. Antibiotic treatment of gastric lymphoma of mucosa-associated lymphoid tissue: an uncontrolled trial. Ann Intern Med. 1999 Jul 20;131(2):88-95. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
==Metronidazole, Tetracycline, PPI, Bismuth==
===Regimen===
Study 	Dates of enrollment 	Evidence
Steinbach et al. 1999	Not reported to 1997-05	Non-randomized
====Antibiotic therapy====
*Metronidazole (Flagyl) 500 mg PO three times per day on days 1 to 21
*Tetracycline (Sumycin) 500 mg PO four times per day on days 1 to 21
*One of the following PPIs:
**Omeprazole (Prilosec) 20 mg PO twice per day on days 1 to 21
**Lansoprazole (Prevacid) 30 mg PO twice per day on days 1 to 21
*Bismuth subsalicylate (Pepto-Bismol) 2 tabs PO four times per day on days 1 to 21
8-week cycle for 2 cycles
===References===
#Steinbach G, Ford R, Glober G, Sample D, Hagemeister FB, Lynch PM, McLaughlin PW, Rodriguez MA, Romaguera JE, Sarris AH, Younes A, Luthra R, Manning JT, Johnson CM, Lahoti S, Shen Y, Lee JE, Winn RJ, Genta RM, Graham DY, Cabanillas FF. Antibiotic treatment of gastric lymphoma of mucosa-associated lymphoid tissue: an uncontrolled trial. Ann Intern Med. 1999 Jul 20;131(2):88-95. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
=Ocular adenexal MALT lymphoma, antibiotic therapy=
==Doxycycline monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence
Ferreri et al. 2006 	Not reported 	Phase 2, fewer than 20 patients in this subgroup
Ferreri et al. 2012 (IELSG 27)	2006-08 to 2010-07 	Phase 2
Note: This treatment was intended for patients with ocular adnexal marginal zone lymphoma (OAMZL), given the association with Chlamydia psittaci.
====Antibiotic therapy====
*Doxycycline 100 mg PO twice per day
3-week course
===References===
# Ferreri AJ, Ponzoni M, Guidoboni M, Resti AG, Politi LS, Cortelazzo S, Demeter J, Zallio F, Palmas A, Muti G, Dognini GP, Pasini E, Lettini AA, Sacchetti F, De Conciliis C, Doglioni C, Dolcetti R. Bacteria-eradicating therapy with doxycycline in ocular adnexal MALT lymphoma: a multicenter prospective trial. J Natl Cancer Inst. 2006 Oct 4;98(19):1375-82. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
# IELSG 27: Ferreri AJ, Govi S, Pasini E, Mappa S, Bertoni F, Zaja F, Montalbán C, Stelitano C, Cabrera ME, Giordano Resti A, Politi LS, Doglioni C, Cavalli F, Zucca E, Ponzoni M, Dolcetti R. Chlamydophila psittaci eradication with doxycycline as first-line targeted therapy for ocular adnexae lymphoma: final results of an international phase II trial. J Clin Oncol. 2012 Aug 20;30(24):2988-94. Epub 2012 Jul 16. Erratum in: J Clin Oncol. 2012 Nov 1;30(31):3903. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC NCT01010295
=Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, first-line therapy=
Note: This implies chemotherapy-naive. Some patients in the following studies, especially those with gastric MALT, received H. pylori eradication therapy or radiation prior to chemotherapy.
==Bortezomib monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence
Troch et al. 2009 (Velcade-MALT-Lymphoma)	Not reported 	Phase 2
====Targeted therapy====
*Bortezomib (Velcade) 1.5 mg/m2 IV bolus once per day on days 1, 4, 8, 11
====Supportive therapy====
*5-HT3 antagonist IV immediately prior to bortezomib
*500 mL NS, given after bortezomib
21-day cycle for up to 8 cycles
===References===
# Velcade-MALT-Lymphoma: 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. Epub 2009 Mar 31. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article EuroPMC NCT00373906
==Bendamustine & Rituximab (BR)==
BR: Bendamustine, Rituximab
===Regimen===
Study 	Dates of enrollment	Evidence	Comparator	Comparative Efficacy
Salar et al. 2014 (GELTAMO MALT2008-01)	2009-2011 	Phase 2 	 	
Note: treatment in MALT2008-01 was response adapted; patients with CR after 3 cycles received a total of 4 cycles, whereas patients with PR after 3 cycles received a total of 6 cycles.
====Chemotherapy====
*Bendamustine 90 mg/m2 IV once per day on days 1 & 2
====Targeted therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once on day 1
====Supportive therapy====
*Antiemetics, antipyretics, and antibiotics according to local standard of care
*Prophylactic use of G-CSF allowed according ASCO guidelines (2006)
28-day cycle for up to 8 cycles (see note)
===References===
# GELTAMO MALT2008-01: Salar A, Domingo-Domenech E, Panizo C, Nicolás C, Bargay J, Muntañola A, Canales M, Bello JL, Sancho JM, Tomás JF, Rodríguez MJ, Peñalver FJ, Grande C, Sánchez-Blanco JJ, Palomera L, Arranz R, Conde E, García M, García JF, Caballero D, Montalbán C; Grupo Español de Linfomas/Trasplante de Médula Ósea (GELTAMO). First-line response-adapted treatment with the combination of bendamustine and rituximab in patients with mucosa-associated lymphoid tissue lymphoma (MALT2008-01): a multicentre, single-arm, phase 2 trial. Lancet Haematol. 2014 Dec;1(3):e104-11. Epub 2014 Nov 19. link to original article dosing details in abstract have been reviewed by our editors EuroPMC NCT01015248
## Update: Salar A, Domingo-Domenech E, Panizo C, Nicolás C, Bargay J, Muntañola A, Canales M, Bello JL, Sancho JM, Tomás JF, Rodríguez MJ, Peñalver J, Grande C, Sánchez-Blanco JJ, Palomera L, Arranz R, Conde E, García M, García JF, Caballero D, Montalbán C. Long-term results of a phase 2 study of rituximab and bendamustine for mucosa-associated lymphoid tissue lymphoma. Blood. 2017 Oct 12;130(15):1772-1774. Epub 2017 Aug 11. link to original article EuroPMC
==Chlorambucil monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence 	Comparator 	Comparative Efficacy
Zucca et al. 2013 (IELSG-19)	2003-2005 	Phase 3 (E-de-esc)	1. Chlorambucil & Rituximab	Seems to have shorter PFS1 (primary endpoint)
Zucca et al. 2013 (IELSG-19)	2003-2005 	Phase 3 (E-de-esc)	2. Rituximab 	Did not meet primary endpoint of EFS1
1Reported efficacy is based on the 2017 update.
====Chemotherapy====
*Chlorambucil (Leukeran) 6 mg/m2 PO once per day
6-week course
====Subsequent treatment====
*IELSG-19, patients with SD or better: Chlorambucil consolidation
===References===
# IELSG-19: 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. Epub 2013 Jan 7. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC NCT00210353
## Update: Zucca E, Conconi A, Martinelli G, Bouabdallah R, Tucci A, Vitolo U, Martelli M, Pettengell R, Salles G, Sebban C, Guillermo AL, Pinotti G, Devizzi L, Morschhauser F, Tilly H, Torri V, Hohaus S, Ferreri AJM, Zachée P, Bosly A, Haioun C, Stelitano C, Bellei M, Ponzoni M, Moreau A, Jack A, Campo E, Mazzucchelli L, Cavalli F, Johnson P, Thieblemont C. Final results of the IELSG-19 randomized trial of mucosa-associated lymphoid tissue lymphoma: improved event-free and progression-free survival with rituximab plus chlorambucil versus either chlorambucil or rituximab monotherapy. J Clin Oncol. 2017 Jun 10;35(17):1905-1912. Epub 2017 Mar 29. link to original article EuroPMC
==Chlorambucil & Rituximab (RClb)==
===Regimen===
Study 	Dates of enrollment	Evidence 	Comparator 	Comparative Efficacy
Zucca et al. 2013 (IELSG-19)	2003-2005 	Phase 3 (E-esc)	1. Chlorambucil2. Rituximab	Seems to have longer PFS1 (primary endpoint)(HR 0.62, 95% CI 0.42-0.93)
1Reported efficacy is based on the 2017 update.
====Chemotherapy====
*Chlorambucil (Leukeran) 6 mg/m2 PO once per day
====Targeted therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once per day on days 1, 8, 15, 22
6-week course
====Subsequent treatment====
*IELSG-19, patients with SD or better: Chlorambucil & rituximab consolidation
===References===
# IELSG-19: 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. Epub 2013 Jan 7. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC NCT00210353
## Update: Zucca E, Conconi A, Martinelli G, Bouabdallah R, Tucci A, Vitolo U, Martelli M, Pettengell R, Salles G, Sebban C, Guillermo AL, Pinotti G, Devizzi L, Morschhauser F, Tilly H, Torri V, Hohaus S, Ferreri AJM, Zachée P, Bosly A, Haioun C, Stelitano C, Bellei M, Ponzoni M, Moreau A, Jack A, Campo E, Mazzucchelli L, Cavalli F, Johnson P, Thieblemont C. Final results of the IELSG-19 randomized trial of mucosa-associated lymphoid tissue lymphoma: improved event-free and progression-free survival with rituximab plus chlorambucil versus either chlorambucil or rituximab monotherapy. J Clin Oncol. 2017 Jun 10;35(17):1905-1912. Epub 2017 Mar 29. link to original article EuroPMC
==Cladribine monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence
Jäger et al. 2002	Not reported 	Phase 2
====Chemotherapy====
*Cladribine (Leustatin) 0.12 mg/kg IV over 2 hours once per day on days 1 to 5
28-day cycle for 4 to 6 cycles
====Dose and schedule modifications====
*"In case of a persisting nadir of the WBC count less than or equal to 4.0 x 109/L (or ANC less than or equal to 1500/μL) and/or the platelets less than or equal to 100 x 109/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."
===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 dosing details in manuscript have been reviewed by our editors EuroPMC
==Cladribine & Rituximab==
===Regimen===
Study 	Dates of enrollment	Evidence
Troch et al. 2012 (AGMT MALT)	2008-2010 	Phase 2
====Chemotherapy====
*Cladribine (Leustatin) 0.1 mg/kg SC once per day on days 1 to 4
====Targeted therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once on day 1
====Supportive therapy====
*Acetaminophen (Tylenol) 1000 mg PO once on day 1, prior to rituximab
*Antihistamine IV once on day 1, prior to rituximab
*One of the following:
**Ondansetron (Zofran) IV once per day on days 1 to 4, prior to cladribine
**Tropisetron (Navoban) IV once per day on days 1 to 4, prior to cladribine
21-day cycle for up to 6 cycles
===References===
# AGMT MALT: Troch M, Kiesewetter B, Willenbacher W, Willenbacher E, Zebisch A, Linkesch W, Fridrik M, Müllauer L, Greil R, Raderer M. Rituximab plus subcutaneous cladribine in patients with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue: a phase II study by the Arbeitsgemeinschaft Medikamentose Tumortherapie. Haematologica. 2013 Feb;98(2):264-8. Epub 2012 Sep 14. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article EuroPMC NCT00656812
==Fludarabine and Rituximab (FR)==
FR: Fludarabine & Rituximab
===Regimen===
Study 	Dates of enrollment	Evidence	Efficacy
Salar et al. 2009	Not reported 	Phase 2 	ORR: 100%
====Chemotherapy====
*Fludarabine (Fludara) 25 mg/m2 IV (or 40 mg/m2 PO) once per day on days 1 to 5
**Patients with gastric lymphoma received the cycle 1 dose intravenously to guarantee drug absorption
**Patients greater than 70 years old: same dose, but only once per day on days 1 to 3
====Targeted therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once on day 1
====Supportive therapy====
*Trimethoprim/Sulfamethoxazole prophylaxis (dose/schedule not listed) per physician preference
*No routine antiviral prophylaxis or G-CSF use
28-day cycle for 4 to 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. link to orginal article dosing details in manuscript have been reviewed by our editors EuroPMC
==Lenalidomide monotherapy==
===Regimen===
Study 	Evidence
Kiesewetter et al. 2012 (LEN-MALT)	Phase 2, fewer than 20 patients reported
====Targeted therapy====
*Lenalidomide (Revlimid) 25 mg PO once per day on days 1 to 21
====Supportive therapy====
*Aspirin 100 mg PO once per day
28-day cycle for up to 6 cycles
===References===
# LEN-MALT: Kiesewetter B, Troch M, Dolak W, Müllauer L, Lukas J, Zielinski CC, Raderer M. A phase II study of lenalidomide in patients with extranodal marginal zone B-cell lymphoma of the mucosa associated lymphoid tissue (MALT lymphoma). Haematologica. 2013 Mar;98(3):353-6. Epub 2012 Aug 16. link to orginal article dosing details in manuscript have been reviewed by our editors link to PMC article EuroPMC NCT00923663
==Lenalidomide & Rituximab (R2)==
===Regimen===
Study 	Dates of enrollment	Evidence
Kiesewetter et al. 2016 (AGMT MALT-2)	Not reported 	Phase 2
====Targeted therapy====
*Lenalidomide (Revlimid) 20 mg PO once per day on days 1 to 21
*Rituximab (Rituxan) 375 mg/m2 IV once on day 1
28-day cycle for up to 8 to 12 cycles
===References===
# AGMT MALT-2: Kiesewetter B, Willenbacher E, Willenbacher W, Egle A, Neumeister P, Voskova D, Mayerhoefer ME, Simonitsch-Klupp I, Melchardt T, Greil R, Raderer M; AGMT Investigators. A phase 2 study of rituximab plus lenalidomide for mucosa-associated lymphoid tissue lymphoma. Blood. 2017 Jan 19;129(3):383-385. Epub 2016 Nov 22. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC NCT01611259
==MCP==
MCP: Mitoxantrone, Chlorambucil, Prednisone
===Regimen===
Study 	Dates of enrollment	Evidence
Wöhrer et al. 2003	Not reported 	Retrospective
Note: the chlorambucil dose is written in the reference as "3 x 3 mg/m2"; total dose per day is 9 mg/m2.
====Chemotherapy====
*Mitoxantrone (Novantrone) 8 mg/m2 IV once per day on days 1 & 2
*Chlorambucil (Leukeran) 3 mg/m2 PO three times per day on days 1 to 5
====Glucocorticoid therapy====
*Prednisone (Sterapred) 25 mg/m2 PO once per day on days 1 to 5
28-day cycle for up to 8 cycles
===References===
# Retrospective: 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 dosing details in manuscript have been reviewed by our editors EuroPMC
==Rituximab monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence 	Comparator 	Comparative Efficacy
Conconi et al. 2003 (IELSG 6)	2000-2001 	Phase 2 	 	
Martinelli et al. 2005 	Not reported 	Phase 2 	 	
Zucca et al. 2013 (IELSG-19) 	2003-2005 	Phase 3 (E-switch-ooc)	1. Chlorambucil 	Did not meet primary endpoint of EFS
Zucca et al. 2013 (IELSG-19) 	2003-2005 	Phase 3 (E-switch-ooc)	2. Chlorambucil & Rituximab	Seems to have shorter PFS (primary endpoint)
====Targeted therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once per day on days 1, 8, 15, 22
====Supportive therapy====
*Acetaminophen (Tylenol) 650 mg PO once per day on days 1, 8, 15, 22; 30 minutes prior to rituximab
*Diphenhydramine (Benadryl) 50 mg PO once per day on days 1, 8, 15, 22; 30 minutes prior to rituximab
4-week course
===References===
# IELSG 6: 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 dosing details in manuscript have been reviewed by our editors EuroPMC content property of HemOnc.org
# 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. Epub 2005 Jan 24. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
# IELSG-19: 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. Epub 2013 Jan 7. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC NCT00210353
## Update: Zucca E, Conconi A, Martinelli G, Bouabdallah R, Tucci A, Vitolo U, Martelli M, Pettengell R, Salles G, Sebban C, Guillermo AL, Pinotti G, Devizzi L, Morschhauser F, Tilly H, Torri V, Hohaus S, Ferreri AJM, Zachée P, Bosly A, Haioun C, Stelitano C, Bellei M, Ponzoni M, Moreau A, Jack A, Campo E, Mazzucchelli L, Cavalli F, Johnson P, Thieblemont C. Final results of the IELSG-19 randomized trial of mucosa-associated lymphoid tissue lymphoma: improved event-free and progression-free survival with rituximab plus chlorambucil versus either chlorambucil or rituximab monotherapy. J Clin Oncol. 2017 Jun 10;35(17):1905-1912. Epub 2017 Mar 29. link to original article EuroPMC
=Consolidation after first-line therapy=
==Chlorambucil & Rituximab (RClb)==
===Regimen===
Study 	Dates of enrollment	Evidence
Zucca et al. 2013 (IELSG-19)	2003-2005 	Non-randomized part of phase 3 RCT
====Preceding treatment====
*Chlorambucil & Rituximab induction
====Chemotherapy====
*Chlorambucil (Leukeran) 6 mg/m2 PO once per day on days 1 to 14
====Targeted therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once on day 1
28-day cycle for up to 4 cycles
===References===
# IELSG-19: 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. Epub 2013 Jan 7. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC NCT00210353
## Update: Zucca E, Conconi A, Martinelli G, Bouabdallah R, Tucci A, Vitolo U, Martelli M, Pettengell R, Salles G, Sebban C, Guillermo AL, Pinotti G, Devizzi L, Morschhauser F, Tilly H, Torri V, Hohaus S, Ferreri AJM, Zachée P, Bosly A, Haioun C, Stelitano C, Bellei M, Ponzoni M, Moreau A, Jack A, Campo E, Mazzucchelli L, Cavalli F, Johnson P, Thieblemont C. Final results of the IELSG-19 randomized trial of mucosa-associated lymphoid tissue lymphoma: improved event-free and progression-free survival with rituximab plus chlorambucil versus either chlorambucil or rituximab monotherapy. J Clin Oncol. 2017 Jun 10;35(17):1905-1912. Epub 2017 Mar 29. link to original article EuroPMC
=Relapsed or refractory=
==Bortezomib monotherapy==
===Regimen variant #1, 1.3 mg/m2===
Study 	Dates of enrollment	Evidence
Conconi et al. 2010 (IELSG 25A)	2005-2009 	Phase 2
====Targeted therapy====
*Bortezomib (Velcade) 1.3 mg/m2 IV once per day on days 1, 4, 8, 11
====Supportive therapy====
*No routine growth factors, antibiotic, or antiviral prophylaxis was given
21-day cycle for up to 6 cycles
===Regimen variant #2, 1.5 mg/m2===
Study 	Dates of enrollment	Evidence
Troch et al. 2009 (Velcade-MALT-Lymphoma)	Not reported 	Phase 2, fewer than 20 pts
====Targeted therapy====
*Bortezomib (Velcade) 1.5 mg/m2 IV bolus once per day on days 1, 4, 8, 11
====Supportive therapy====
*5-HT3 antagonist IV immediately prior to bortezomib
*500 mL NS, given after bortezomib
21-day cycle for up to 8 cycles
===References===
# Velcade-MALT-Lymphoma: 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. Epub 2009 Mar 31. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article EuroPMC NCT00373906
# IELSG 25A: 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. 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. Epub 2010 Sep 1. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC NCT00210327
==Cladribine & Rituximab==
===Regimen===
Study 	Dates of enrollment	Evidence
Troch et al. 2012 (AGMT MALT)	2008-2010 	Phase 2, fewer than 20 patients reported
====Chemotherapy====
*Cladribine (Leustatin) 0.1 mg/kg SC once per day on days 1 to 4
====Targeted therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once on day 1
====Supportive therapy====
*Acetaminophen (Tylenol) 1000 mg PO once on day 1, prior to rituximab
*Antihistamine IV once on day 1, prior to rituximab
*One of the following:
**Ondansetron (Zofran) IV once per day on days 1 to 4, prior to cladribine
**Tropisetron (Navoban) IV once per day on days 1 to 4, prior to cladribine
21-day cycle for up to 6 cycles
===References===
# AGMT MALT: Troch M, Kiesewetter B, Willenbacher W, Willenbacher E, Zebisch A, Linkesch W, Fridrik M, Müllauer L, Greil R, Raderer M. Rituximab plus subcutaneous cladribine in patients with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue: a phase II study by the Arbeitsgemeinschaft Medikamentose Tumortherapie. Haematologica. 2013 Feb;98(2):264-8. Epub 2012 Sep 14. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article EuroPMC NCT00656812
==Clarithromycin monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence
Ferreri et al. 2015 (HD-K)	2012-02 to 2013-10 	Phase 2
====Antibiotic therapy====
*Clarithromycin (Biaxin) 2000 mg PO once per day on days 1 to 14
21-day cycle for 4 cycles
===References===
# HD-K: Ferreri AJ, Sassone M, Kiesewetter B, Govi S, Scarfò L, Donadoni G, Raderer M. High-dose clarithromycin is an active monotherapy for patients with relapsed/refractory extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT): the HD-K phase II trial. Ann Oncol. 2015 Aug;26(8):1760-5. Epub 2015 May 1. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC NCT01516606
==Lenalidomide monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence
Kiesewetter et al. 2012 (LEN-MALT)	Not reported 	Phase 2, fewer than 20 patients reported
====Targeted therapy====
*Lenalidomide (Revlimid) 25 mg PO once per day on days 1 to 21
====Supportive therapy====
*Aspirin 100 mg PO once per day
28-day cycle for up to 6 cycles
===References===
# LEN-MALT: Kiesewetter B, Troch M, Dolak W, Müllauer L, Lukas J, Zielinski CC, Raderer M. A phase II study of lenalidomide in patients with extranodal marginal zone B-cell lymphoma of the mucosa associated lymphoid tissue (MALT lymphoma). Haematologica. 2013 Mar;98(3):353-6. Epub 2012 Aug 16. link to orginal article dosing details in manuscript have been reviewed by our editors link to PMC article EuroPMC NCT00923663
==Rituximab monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence
Conconi et al. 2003 (IELSG 6)	2000-2001 	Phase 2
Martinelli et al. 2005 	Not reported 	Phase 2
====Targeted therapy====
*Rituximab (Rituxan) 375 mg/m2 IV once per day on days 1, 8, 15, 22
28-day course
===References===
# IELSG 6: 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. Epub 2003 Jul 3. link to orginal article dosing details in manuscript have been reviewed by our editors EuroPMC
# 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. Epub 2005 Jan 24. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
=Prognosis=
==MALT-IPI (2017)==
===Risk factors===
*Stage III or IV
*Older than 70 years old years
*LDH greater than the upper limit of normal
===Calculation===
*Low risk: zero risk factors present
*Intermediate risk: one risk factor present
*High risk: two or more risk factors present
</div>
===References===
# Thieblemont C, Cascione L, Conconi A, Kiesewetter B, Raderer M, Gaidano G, Martelli M, Laszlo D, Coiffier B, Lopez Guillermo A, Torri V, Cavalli F, Johnson PW, Zucca E. A MALT lymphoma prognostic index. Blood. 2017 Sep 21;130(12):1409-1417. Epub 2017 Jul 18. link to original article EuroPMC
</div>
=Response criteria=
==NCI Sponsored International Working Group Criteria (1999)==
# Cheson BD, Horning SJ, Coiffier B, Shipp MA, Fisher RI, Connors JM, Lister TA, Vose J, Grillo-López A, Hagenbeek A, Cabanillas F, Klippensten D, Hiddemann W, Castellino R, Harris NL, Armitage JO, Carter W, Hoppe R, Canellos GP. Report of an international workshop to standardize response criteria for non-Hodgkin's lymphomas. NCI Sponsored International Working Group. J Clin Oncol. 1999 Apr;17(4):1244. Review. Erratum in: J Clin Oncol 2000 Jun;18(11):2351. link to original article EuroPMC
Category:MALT lymphoma regimens
Category:Disease-specific pages
Category:Marginal zone lymphomas
Category:B-cell lymphomas