Difference between revisions of "Marginal zone lymphoma"

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Is there a regimen missing from this list?  Would you like to share a different dosage/schedule or an additional reference for a regimen?  Have you noticed an error?  Do you have an idea that will help the site grow to better meet your needs and the needs of many others?  You are [[How_to_contribute|invited to contribute to the site]].
 
Is there a regimen missing from this list?  Would you like to share a different dosage/schedule or an additional reference for a regimen?  Have you noticed an error?  Do you have an idea that will help the site grow to better meet your needs and the needs of many others?  You are [[How_to_contribute|invited to contribute to the site]].
  
{{TOC limit|limit=2}}
+
{{TOC limit|limit=3}}
 +
 
 +
=Untreated=
 +
 
 +
''Note: Untreated in this context 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 (Velcade)==
 
==Bortezomib (Velcade)==
  
 +
===Regimen, Troch, et al. 2009===
 
Level of Evidence:
 
Level of Evidence:
 
<span  
 
<span  
Line 15: Line 20:
 
border-style:solid;">Phase II</span>
 
border-style:solid;">Phase II</span>
  
===Regimen #1, Troch, et al. 2009===
 
 
*[[Bortezomib (Velcade)]] 1.5 mg/m2 IV bolus once daily on days 1, 4, 8, 11
 
*[[Bortezomib (Velcade)]] 1.5 mg/m2 IV bolus once daily on days 1, 4, 8, 11
  
Line 23: Line 27:
 
*[[Antiemesis|5-HT3 antagonist]] IV immediately before bortezomib
 
*[[Antiemesis|5-HT3 antagonist]] IV immediately before bortezomib
 
*500 mL NS after bortezomib
 
*500 mL NS after bortezomib
 
===Regimen #2, Conconi, et al. 2011===
 
*[[Bortezomib (Velcade)]] 1.3 mg/m2 IV once daily 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===
 
===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. [http://www.haematologica.org/content/94/5/738.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/19336742 PubMed]
 
# 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. [http://www.haematologica.org/content/94/5/738.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/19336742 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. [http://annonc.oxfordjournals.org/content/22/3/689.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/20810546 PubMed]
 
  
 
==Chlorambucil (Leukeran)==
 
==Chlorambucil (Leukeran)==
 
===Regimen #1, Leblond, et al. 2013===
 
===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
+
Level of Evidence:
 +
<span
 +
style="background:#00CD00;
 +
padding:3px 6px 3px 6px;
 +
border-color:black;
 +
border-width:2px;
 +
border-style:solid;">Phase III</span>
 +
 
 +
*[[Chlorambucil (Leukeran)]] 8 mg/m2 (6 mg/m2 per day if older than 75 years old) PO daily on days 1 to 10
  
 
'''28-day cycles x up to 12 cycles'''
 
'''28-day cycles x up to 12 cycles'''
Line 47: Line 50:
 
**[[Pentamidine (Nebupent)]] 300 mg inhaled once per month
 
**[[Pentamidine (Nebupent)]] 300 mg inhaled once per month
  
===Regimen #2, Zucca, et al. 2013 - IELSG-19===
+
===Regimen #2, Zucca, et al. 2013 (IELSG-19)===
 +
Level of Evidence:
 +
<span
 +
style="background:#00CD00;
 +
padding:3px 6px 3px 6px;
 +
border-color:black;
 +
border-width:2px;
 +
border-style:solid;">Phase III</span>
 +
 
 
====Induction phase====
 
====Induction phase====
*[[Chlorambucil (Leukeran)]] 6 mg/m2 PO daily on days 1-42
+
*[[Chlorambucil (Leukeran)]] 6 mg/m2 PO daily on days 1 to 42
  
 
'''42-day course of therapy'''
 
'''42-day course of therapy'''
  
 
====Stable disease & responders -> consolidation phase====
 
====Stable disease & responders -> consolidation phase====
*[[Chlorambucil (Leukeran)]] 6 mg/m2 PO daily on days 1-14
+
*[[Chlorambucil (Leukeran)]] 6 mg/m2 PO daily on days 1 to 14
  
 
'''28-day cycles x up to 4 cycles'''
 
'''28-day cycles x up to 4 cycles'''
Line 73: Line 84:
  
 
===Regimen===
 
===Regimen===
*[[Cladribine (Leustatin)]] 0.12 mg/kg IV over 2 hours once on days 1-5
+
*[[Cladribine (Leustatin)]] 0.12 mg/kg IV over 2 hours once on days 1 to 5
  
 
'''28-day cycles x 4 to 6 cycles'''
 
'''28-day cycles x 4 to 6 cycles'''
Line 87: Line 98:
  
 
==Fludarabine (Fludara)==
 
==Fludarabine (Fludara)==
 +
 
===Regimen===
 
===Regimen===
*[[Fludarabine (Fludara)]] 40 mg/m2 (30 mg/m2 per day if older than 75 years old) PO once daily on days 1-5
+
Level of Evidence:
 +
<span
 +
style="background:#00CD00;
 +
padding:3px 6px 3px 6px;
 +
border-color:black;
 +
border-width:2px;
 +
border-style:solid;">Phase III</span>
 +
 
 +
*[[Fludarabine (Fludara)]] 40 mg/m2 (30 mg/m2 per day if older than 75 years old) PO once daily on days 1 to 5
  
 
'''28-day cycles x up to 6 cycles'''
 
'''28-day cycles x up to 6 cycles'''
Line 107: Line 127:
  
 
===Regimen===
 
===Regimen===
 +
Level of Evidence:
 +
<span
 +
style="background:#ff0000;
 +
padding:3px 6px 3px 6px;
 +
border-color:black;
 +
border-width:2px;
 +
border-style:solid;">Retrospective</span>
 +
 
*[[Mitoxantrone (Novantrone)]] 8 mg/m2 IV once daily on days 1 & 2
 
*[[Mitoxantrone (Novantrone)]] 8 mg/m2 IV once daily 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
+
*[[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 to 5
*[[Prednisolone (Millipred)]] 25 mg/m2 PO/IV once daily on days 1-5
+
*[[Prednisolone (Millipred)]] 25 mg/m2 PO/IV once daily on days 1 to 5
  
 
'''28-day cycles x up to 8 cycles'''
 
'''28-day cycles x up to 8 cycles'''
Line 115: Line 143:
 
===References===
 
===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. [http://annonc.oxfordjournals.org/content/14/12/1758.long link to orginal article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/14630681 PubMed]
 
# 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. [http://annonc.oxfordjournals.org/content/14/12/1758.long link to orginal article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/14630681 PubMed]
 +
 +
==R-Flu==
 +
R-Flu: '''<u>R</u>'''ituximab, '''<u>Flu</u>'''darabine
 +
 +
===Regimen===
 +
*[[Rituximab (Rituxan)]] 375 mg/m2 IV once on day 1
 +
*[[Fludarabine (Fludara)]] 25 mg/m2 IV (or 40 mg/m2 PO) once daily on days 1 to 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 once daily on days 1 to 3
 +
 +
'''28-day cycles for 4-6 cycles'''
 +
 +
Supportive medications:
 +
*[[Trimethoprim/Sulfamethoxazole (Bactrim DS)|Trimethoprim/Sulfamethoxazole]] prophylaxis (dose/schedule not listed) per physician preference
 +
*No routine antiviral prophylaxis or G-CSF use
 +
 +
===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. [http://onlinelibrary.wiley.com/doi/10.1002/cncr.24605/full link to orginal article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/19672998 PubMed]
  
 
==Rituximab (Rituxan)==
 
==Rituximab (Rituxan)==
Line 133: Line 179:
 
# 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. [http://jco.ascopubs.org/content/23/9/1979.long link to original article] '''contains protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/15668468 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. [http://jco.ascopubs.org/content/23/9/1979.long link to original article] '''contains protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/15668468 PubMed]
  
==Rituximab (Rituxan) & Chlorambucil (Leukeran)==
+
==Rituximab & Chlorambucil==
===Regimen, Zucca, et al. 2013 - IELSG-19===
+
 
 +
===Regimen, Zucca, et al. 2013 (IELSG-19)===
 +
Level of Evidence:
 +
<span
 +
style="background:#00CD00;
 +
padding:3px 6px 3px 6px;
 +
border-color:black;
 +
border-width:2px;
 +
border-style:solid;">Phase III</span>
 +
 
 
====Induction phase====
 
====Induction phase====
 
*[[Rituximab (Rituxan)]] 375 mg/m2 IV once daily on days 1, 8, 15, 22
 
*[[Rituximab (Rituxan)]] 375 mg/m2 IV once daily on days 1, 8, 15, 22
*[[Chlorambucil (Leukeran)]] 6 mg/m2 PO daily on days 1-42
+
*[[Chlorambucil (Leukeran)]] 6 mg/m2 PO daily on days 1 to 42
  
 
====Stable disease & responders -> consolidation phase====
 
====Stable disease & responders -> consolidation phase====
 
*[[Rituximab (Rituxan)]] 375 mg/m2 IV once on day 1
 
*[[Rituximab (Rituxan)]] 375 mg/m2 IV once on day 1
*[[Chlorambucil (Leukeran)]] 6 mg/m2 PO daily on days 1-14
+
*[[Chlorambucil (Leukeran)]] 6 mg/m2 PO daily on days 1 to 14
  
 
'''28-day cycles x up to 4 cycles'''
 
'''28-day cycles x up to 4 cycles'''
Line 148: Line 203:
 
# 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. [http://jco.ascopubs.org/content/31/5/565.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/23295789 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. [http://jco.ascopubs.org/content/31/5/565.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/23295789 PubMed]
  
==Rituximab (Rituxan) & Fludarabine (Fludara) (R-Flu)==
+
=Relapsed/refractory=
R-Flu: '''<u>R</u>'''ituximab, '''<u>Flu</u>'''darabine
+
 
 +
==Bortezomib (Velcade)==
 +
 
 +
===Regimen #1, Troch, et al. 2009===
 +
Level of Evidence:
 +
<span
 +
style="background:#EEEE00;
 +
padding:3px 6px 3px 6px;
 +
border-color:black;
 +
border-width:2px;
 +
border-style:solid;">Phase II</span>
 +
 
 +
*[[Bortezomib (Velcade)]] 1.5 mg/m2 IV bolus once daily on days 1, 4, 8, 11
 +
 
 +
'''21-day cycles x up to 8 cycles'''
 +
 
 +
Supportive medications:
 +
*[[Antiemesis|5-HT3 antagonist]] IV immediately before bortezomib
 +
*500 mL NS after bortezomib
 +
 
 +
===Regimen #2, Conconi, et al. 2011===
 +
Level of Evidence:
 +
<span
 +
style="background:#EEEE00;
 +
padding:3px 6px 3px 6px;
 +
border-color:black;
 +
border-width:2px;
 +
border-style:solid;">Phase II</span>
  
===Regimen===
+
*[[Bortezomib (Velcade)]] 1.3 mg/m2 IV once daily on days 1, 4, 8, 11
*[[Rituximab (Rituxan)]] 375 mg/m2 IV once on day 1
 
*[[Fludarabine (Fludara)]] 25 mg/m2 IV (or 40 mg/m2 PO) once daily 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 once daily on days 1-3
 
  
'''28-day cycles for 4-6 cycles'''
+
'''21-day cycles x up to 6 cycles'''
  
 
Supportive medications:
 
Supportive medications:
*[[Trimethoprim/Sulfamethoxazole (Bactrim DS)|Trimethoprim/Sulfamethoxazole]] prophylaxis (dose/schedule not listed) per physician preference
+
*No routine growth factors, antibiotic, or antiviral prophylaxis was given
*No routine antiviral prophylaxis or G-CSF use
+
 
 +
===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. [http://www.haematologica.org/content/94/5/738.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/19336742 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. [http://annonc.oxfordjournals.org/content/22/3/689.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/20810546 PubMed]
 +
 
 +
==Rituximab (Rituxan)==
 +
 
 +
Level of Evidence:
 +
<span
 +
style="background:#EEEE00;
 +
padding:3px 6px 3px 6px;
 +
border-color:black;
 +
border-width:2px;
 +
border-style:solid;">Phase II</span>
 +
 
 +
===Regimen===
 +
*[[Rituximab (Rituxan)]] 375 mg/m2 IV once daily on days 1, 8, 15, 21
  
 
===References===
 
===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. [http://onlinelibrary.wiley.com/doi/10.1002/cncr.24605/full link to orginal article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/19672998 PubMed]
+
# 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. [http://bloodjournal.hematologylibrary.org/content/102/8/2741.long link to orginal article] '''contains protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/12842999 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. [http://jco.ascopubs.org/content/23/9/1979.long link to original article] '''contains protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/15668468 PubMed]

Revision as of 17:48, 18 June 2013

Use of this site is subject to you reading and agreeing with the terms set forth in the disclaimer.

Is there a regimen missing from this list? Would you like to share a different dosage/schedule or an additional reference for a regimen? Have you noticed an error? Do you have an idea that will help the site grow to better meet your needs and the needs of many others? You are invited to contribute to the site.


Untreated

Note: Untreated in this context 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 (Velcade)

Regimen, Troch, et al. 2009

Level of Evidence: Phase II

21-day cycles x up to 8 cycles

Supportive medications:

References

  1. 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

Chlorambucil (Leukeran)

Regimen #1, Leblond, et al. 2013

Level of Evidence: Phase III

28-day cycles x up to 12 cycles

Supportive medications:

Regimen #2, Zucca, et al. 2013 (IELSG-19)

Level of Evidence: Phase III

Induction phase

42-day course of therapy

Stable disease & responders -> consolidation phase

28-day cycles x up to 4 cycles

References

  1. 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
  2. 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 (Leustatin)

Level of Evidence: Phase II

Regimen

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

  1. 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 (Fludara)

Regimen

Level of Evidence: Phase III

  • Fludarabine (Fludara) 40 mg/m2 (30 mg/m2 per day if older than 75 years old) PO once daily on days 1 to 5

28-day cycles x up to 6 cycles

Supportive medications:

References

  1. 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

Level of Evidence: Retrospective

28-day cycles x up to 8 cycles

References

  1. 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 once on day 1
  • Fludarabine (Fludara) 25 mg/m2 IV (or 40 mg/m2 PO) once daily on days 1 to 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 once daily on days 1 to 3

28-day cycles for 4-6 cycles

Supportive medications:

References

  1. 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 (Rituxan)

Level of Evidence: Phase II

Regimen

References

  1. 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
  2. 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

Rituximab & Chlorambucil

Regimen, Zucca, et al. 2013 (IELSG-19)

Level of Evidence: Phase III

Induction phase

Stable disease & responders -> consolidation phase

28-day cycles x up to 4 cycles

References

  1. 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

Relapsed/refractory

Bortezomib (Velcade)

Regimen #1, Troch, et al. 2009

Level of Evidence: Phase II

21-day cycles x up to 8 cycles

Supportive medications:

Regimen #2, Conconi, et al. 2011

Level of Evidence: Phase II

21-day cycles x up to 6 cycles

Supportive medications:

  • No routine growth factors, antibiotic, or antiviral prophylaxis was given

References

  1. 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
  2. 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

Rituximab (Rituxan)

Level of Evidence: Phase II

Regimen

References

  1. 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
  2. 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