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Types of Lymphoma >
Marginal Zone
Lymphomas
Last update:
06/16/2010
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TOPICS:
Overview
| Non-Gastric MZL | Treatment | Clinical Trials |
Prognosis
| Resources
& Research News
Subtypes: MALT | Nodal | Pulmonary
(BALT)
| Splenic | Cutaneous
(skin)
TOPIC
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Review
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Prognosis
Therapies: ASCO |
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Marginal Zone Lymphomas:
Marginal Zone Lymphomas are a related cancers of the
lymphatic system - a complex
system of lymph organs, including the bone marrow, thymus, spleen, and
the lymph nodes.
What is lymphoma?
Briefly, lymphomas result when
mutations occurs to an immune cell (a lymphocyte),
which alters the behavior of the cells, leading to the abnormal production of proteins that prevents the cells from dying
when they should, or causes sustained rapid cell division that
produces more of its kind.
... The malignant cells then may accumulate
to form tumors that may enlarge the lymph nodes or spread to other
areas of the lymphatic system,
such as the spleen or bone marrow. Lymphoma can also spread or first appear outside the lymphatic system
- and is called extranodal
disease.
The word "marginal zone" describes the cell
type. B-cells arise from the bone marrow and mature or
differentiate into many cell types that tend to migrate to different
areas of the body in order to defend against pathogens (viruses,
bacteria, etc.).
"The marginal zone of the B follicle
represents a well-defined compartment of the B-cell area, a distinct
cellular composition from that of the follicle center (follicular
b-cells), from which it also differs in its functional role in the
immune response."
Incidence:
Marginal Zone NHL is a relatively uncommon type of b-cell lymphoma,
comprising approximately 2-4% of all cases.
There are about 61,000 new
cases of NHL diagnosed annually. Therefore we calculate that there are
approximately 1,000 to 2,300 new cases of marginal zone NHL diagnosed
annually.
Infection-associated,
antigen-driven:
Infections strongly
associated with MZL include:
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H. pylori,
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C. jejuni,
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B. burgdorferi,
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C. psittaci, and
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Hepatitis
C Virus (HCV) |
Source: Infection-associated lymphomas derived from marginal zone
B cells: a model of antigen-driven lymphoproliferation. Blood. 2006
Apr 15;107(8):3034-44. Epub 2006 Jan 5. Review.
PMID:
16397126 | Related
articles
Categories of
marginal zone lymphomas:
"Extranodal Marginal Zone Cell Lymphoma (MZCL) of MALT-type
share similar features with nodal and splenic MZCL regarding
morphology - cell appearance - and immunophenotype - cell expressions that indicate
the maturation stage of the
malignant cell.
At the genetic level, recent
cytogenetic studies have shown that t(11;18) is a recurring
abnormality in extranodal MALT-type Marginal Zone Cell Lymphoma but has hitherto never been
reported in nodal or splenic MZCL. Source: DoctorsDoctor
Common Subtypes:
There are three distinct forms (Harris et al, 1999)
of marginal zone lymphomas:
Click the links above to go to the section on this
page.
Monitoring MZL with PET?
Recommended Resources:
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Non-gastric MZL
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Non-gastric Marginal Zone
Lymphomas
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Nongastric marginal zone B-cell lymphoma: Analysis of 247
cases.
Am J Hematol. 2007 Jan 31;
PMID:
17266060 |
"Non-gastric marginal zone B-cell lymphoma (NG-MZL) is a
relatively uncommon indolent lymphoma.
From 1990 to 2005, a total of 247 patients with histologically (by
cell type)
confirmed
NG-MZL were analyzed.
Ann Arbor stage I/II disease was present in 78% (167 out of
215).
One hundred eighty-six patients out of two hundred eight were
categorized into
the low/low-intermediate risk group (89%) according to International
Prognostic Index (IPI).
Eighty percent (172/215) were in low risk group according to
Follicular
Lymphoma International Prognostic Index (FLIPI).
Complete and partial remissions (CR and PR) were achieved in 140
(92.7%) and
8 (5.3%) of the 151 stage I/II patients.
Especially, radiation containing treatment achieved 96% CR
rate (108 out of 113).
In 38 patients with stage III/IV, CR and PR were achieved in
17
(44.7%) and 11 (26.3%), respectively.
The estimated five-year overall survival (OS) and progression-free
survival
(PFS) were 93.8% and 70.1%, respectively.
Although anthracycline-containing regimen could achieve higher CR
rate, it
did not improve PFS.
Stage III/IV, low hemoglobin, poor performance status,
high/high-intermediate IPI, poor risk FLIPI, and nodal MZL were poor
prognostic factors for PFS.
NG-MZL is an indolent disease. FLIPI has strong power to predict the
prognosis of NG-MZL.
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Treatment
Also see:
Treatment of non-follicular
NHL - PDF
Questions for your doctor
Patients
Against Lymphoma
General, Treatment & Side Effects, and Tests
Treatment Overview
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Factors that determine
treatment timing and approach:
The characteristics of the lymphoma at
diagnosis as determined by the pathology report, and it's actual
clinical behavior, and other factors determine the type of treatment
and the timing of treatment you and your doctor will consider.
Resources & News
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Marginal zone lymphomas -
Factors that affect outcome
interscience.wiley
Factors analyzed included age;
gender; presence of B symptoms; performance score; clinical
stage; serum 2-microglobulin, lactate dehydrogenase, albumin,
and hemoglobin levels; and presence of autoimmune disorder.
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Ongoing monoclonal
B-cell proliferation is not common in gastric B-cell lymphoma
after combined radiochemotherapy. J Clin Oncol. 2004 Aug
1;22(15):3039-45 PMID: 15284253
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Autologous bone
marrow transplantation for marginal zone non-Hodgkin's lymphoma. Leuk Lymphoma. 2004 Feb;45(2):315-20 PMID:
15101717 | Related
abstracts
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H-Pylori:
tests for and treatment of for MALT PAL
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Clinical activity of
rituximab in extranodal marginal zone B-cell lymphoma of MALT
type. Blood. 2003 Jul 3 PMID: 12842999
PubMed
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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. PMID: 12228207 PubMed
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The primary gastric
lymphoma: therapeutic strategies Romeo
Giuli MD
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Conservative treatment of primary gastric low-grade B-cell
lymphoma of mucosa-associated lymphoid tissue: predictive factors
of response and outcome. Am J Gastroenterol. 2002 Feb;97(2):292-7. PMID: 11866264
PubMed
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Clinical Trials
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Clinical Trials
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ClinicalTrials.gov
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Prognosis
Also see Prognostic
Indicators
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Prognosis
"Few
studies have investigated the relation between the location of MALT
lymphomas and their prognosis. In contrast to the results referred by
Thieblemont et al.,7 we found that the tendency to
progress or relapse seemed to be:
more
frequent in gastrointestinal than in non-gastrointestinal MALT
lymphomas (22% vs 10%).
The longer
time to progression showed by Thieblemont et al. for
gastrointestinal lymphomas has not been confirmed in our
study.
Although
our data showed no significant differences in either DFS (disease
free survival) or OS (overall survival) between the two groups of
patients, the slight survival advantage for non-gastrointestinal
MALT lymphomas could be explained by their local involvement and
good performance status at diagnosis.
It is
possible that the single center nature of our study and the
inclusion of only patients with unequivocal histologic criteria of
MALT lymphoma, could have had some influence on the results." source:
haematologica
Abstracts
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Splenic marginal zone lymphoma: a prognostic model for
clinical use.
Blood. 2006 Feb 21; PMID:
16493005
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[1125] Gene Expression Profiling Analysis in Splenic
Marginal Zone Lymphoma Allows To Predict Survival and Histological
Transformation. Session Type: Poster Session 279-I ASH
2004
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Treatment outcome of mucosa-associated lymphoid
tissue/marginal zone non-Hodgkin's lymphoma.
Int J Radiat Oncol
Biol Phys. 2002 Mar 15;52(4):1058-66.
PMID: 11958902 PubMed
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Predictive value of
endoscopic ultrasonography for regression of gastric low grade and
high grade MALT lymphomas after eradication of Helicobacter
pylori.
Gut. 2001 Apr;48(4):454-60. PMID: 11247887 PubMed
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Immunological and molecular analysis of B lymphocytes in
low-grade MALT lymphoma of the stomach. Are there any useful
markers for predicting outcome after Helicobacter pylori
eradication? J Gastroenterol. 2002;37(6):428-33. PMID: 12108676
PubMed
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Comparative study of marginal zone lymphoma involving bone
marrow. Am J Clin Pathol. 2002 May;117(5):698-708. PMID: 12090417 PubMed
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Research News
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Research News
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Nongastric marginal zone B-cell lymphoma: Analysis of 247
cases.
Am J Hematol. 2007 Jan 31; PMID:
17266060
Nongastric marginal zone B-cell lymphoma (NG-MZL) is a relatively
uncommon
indolent lymphoma.
From 1990 to 2005, a total of 247 patients with histologically
confirmed
NG-MZL were analyzed.
Ann Arbor stage I/II disease was present in 78% (167 out of
215).
One hundred eighty-six patients out of two hundred eight were
categorized into
the low/low-intermediate risk group (89%) according to
International
Prognostic Index (IPI).
Eighty percent (172/215) were in low risk group according to
Follicular
Lymphoma International Prognostic Index (FLIPI).
Complete and partial remissions (CR and PR) were achieved in 140
(92.7%) and
8 (5.3%) of the 151 stage I/II patients.
Especially, radiation containing treatment achieved 96% CR rate
(108 out of
113).
In 38 patients with stage III/IV, CR and PR were achieved in 17
(44.7%) and
11 (26.3%), respectively.
The estimated five-year overall survival (OS) and progression-free
survival
(PFS) were 93.8% and 70.1%, respectively.
Although anthracycline-containing regimen could achieve higher CR
rate, it
did not improve PFS.
Stage III/IV, low hemoglobin, poor performance status,
high/high-intermediate IPI, poor risk FLIPI, and nodal MZL were
poor
prognostic factors for PFS.
NG-MZL is an indolent disease. FLIPI has strong power to predict
the
prognosis of NG-MZL. Am. J. Hematol., 2007. (c) 2007 Wiley-Liss,
Inc.
PMID: 17266060
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Rituxan® Effective in Treatment of Splenic Marginal Zone Lymphoma and Marginal Zone Lymphoma
cancerconsultants.com
" A complete or partial disappearance of detectable cancer was experienced by 88% of patients treated with Rituxan alone, 83% of patients treated with Rituxan and chemotherapy, and 55% of patients treated with chemotherapy alone. The proportion of patients who survived for three years or longer was 95% among patients treated with Rituxan alone, 100% among patients treated with Rituxan and chemotherapy, and 55% among patients treated with chemotherapy alone."
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Marginal Zone Lymphomas & Hepatitis C: Splenic and nodal
marginal zone lymphomas are indolent disorders at high hepatitis C
virus seroprevalence with distinct presenting features but similar
morphologic and phenotypic profiles.
Cancer. 2004 Jan 1; 100(1):
107-15. PMID: 14692030
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articles
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IELSG phase II study of rituximab in MALT lymphoma: final
results Year: 2002 Abstract No: 1067
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Stage I and II MALT
lymphoma: results of treatment with radiotherapy.
Int J Radiat Oncol Biol Phys. 2001 Aug 1;50(5):1258-64. PMID:
11483337 PubMed
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Mucosa-associated
lymphoid tissue lymphoma with initial supradiaphragmatic
presentation: natural history and patterns of disease progression.
Int J Radiat Oncol Biol Phys. 2000 Sep 1;48(2):399-403. PMID:
10974453 PubMed
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Molecular
follow-up in gastric mucosa-associated lymphoid tissue lymphomas:
early analysis of the LY03 cooperative trial. Blood. 2002 Apr
1;99(7):2541-4. PMID: 11895791 PubMed
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Of interest to pts with MALT: Randomized trial of endoscopy with testing for Helicobacter pylori compared with non-invasive H pylori testing alone in the management of dyspepsia
BMJ 2002;324:999 (27 April)
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The Modified International Prognostic Index Can
Predict The Outcome of Localized Primary Intestinal Lymphoma of
Both Extranodal Marginal Zone B-Cell and Diffuse Larege B-Cell
Histologies International Extranodal Lymphoma Study Group (IELSG)
British Journal of Haematology, 2002, 118, 218–228 PDF
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Subtype of Marginal Zone Lymphomas
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MALT
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We moved this most common type of Marginal Zone Lymphoma
to the MALT page
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Nodal
(Monocytoid B-cell Lymphoma)
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"Malignant
monocytoid B-cell proliferations in a lymph node have been classified
as monocytoid B-cell lymphomas (MBCL), which are now called nodal
marginal-zone B-cell lymphoma (MZL) in the World Health Organization
(WHO) classification." 1
"Splenic and nodal marginal zone lymphomas are typical low-grade lymphomas with an indolent course. A subset of patients, however, presents with more
aggressive disease and have a shorter survival. Clinical and biological prognostic factors identified in reported series are
heterogeneous (varied).
The role played by hepatitis C virus (HCV) in marginal zone lymphomas is not fully elucidated, but there is demonstration that eradication of HCV infection in splenic lymphoma with villous lymphocytes causes regression of the lymphoma.
The optimal treatment has not yet been identified. Retrospective series, however, show that splenectomy is a good option if symptoms from the presence of spleen enlargement or cytopenias need to be treated. The utility of purine analogs and of anti-CD20 immunotherapy needs to be clarified in prospective trials."
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Nodal monocytoid B-cell lymphoma (nodal marginal-zone B-cell
lymphoma). Semin Hematol. 1999 Apr;36(2):128-38.
Review. PMID:
10319381 PubMed
Technical:
Marginal zone-related neoplasms of splenic and nodal origin.
Haematologica. 2003 Jan;88(1):80-93.
Review. PMID:
12551831 | Related
articles
Full text PDF
"The marginal zone is an anatomically distinct B-cell
compartment of lymphoid tissue with an abundant antigenic influx.
Among marginal zone-derived lymphomas the WHO classification
listed, in addition to extranodal marginal zone B-cell lymphoma of
MALT type, two other marginal zone B-cell neoplasms: splenic
marginal zone B-cell lymphoma (+/- villous lymphocytes) and nodal
marginal zone B-cell lymphoma (+/- monocytoid B cells).
State-of-the-Art Therapeutics: Marginal-Zone Lymphoma
abstract only
jco.ascopubs.org
"These data have determined unique approach among all other
lymphoma subtypes: the possibility of treating a subset of
patients with antibiotics alone as first line of treatment.
Indeed, there is compelling evidence that histologic regressions
can be achieved in most gastric MALT lymphomas by eradicating
Helicobacter pylori infection. However, molecular follow-up
studies showed the persistence of the malignant clone in half of
the cases in histologic remission after antibiotic treatment and
transient, either histologic or molecular, relapses have been
reported, too.
Hence, a careful long-term follow-up is mandatory after antibiotic
treatment. Radiotherapy, chemotherapy, anti-CD20 monoclonal
antibodies are effective alternative therapies. The precise role
of surgical resection should be redefined in view of the
encouraging results of conservative approaches.
Differently from EMZL, both SMLZ and NMZL often present with
disseminated disease at diagnosis. The therapeutic approach
comprises splenectomy, for SMZL, and chemotherapy, but with no
consensus about the best treatment. "
Monocytoid
B cell lymphoma: clinical and prognostic features of 21
patients.
The prognosis of MBCL was comparable with that of
other low grade malignant lymphomas.
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Pulmonary
(Lung) MALT Lymphoma (BALT)
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TOPIC SEARCH: ASCO
| PubMed
| WEB
In
"a retrospective study of the pathological features in 69 primary
pulmonary non-Hodgkin's lymphomas which have previously been
clinically reviewed. The tumors consisted of 61 (88%) low-grade and
eight (12%) high-grade malignant lymphomas. Fifty-four of the
low-grade malignant lymphomas were MALT lymphomas." [2]
Nearly half of the patients at diagnosis have no
symptoms, and are identified incidentally on the basis of a
radiological exams. Symptoms are usually non specific, such as cough,
mild shortness of breath, chest pain and occasionally coughing of
blood. [1]
"Clinical data suggest that limited surgery or
non-aggressive chemotherapy can provide long-term survival in patients
with such slowly developing neoplasms." [1]
"The role played by hepatitis C virus (HCV) in marginal zone
lymphomas is not fully elucidated, but there is demonstration that
eradication of HCV infection in splenic lymphoma with villous
lymphocytes causes regression of the lymphoma. The optimal treatment
has not yet been identified. Retrospective series, however, show that
splenectomy is a good option if symptoms from the presence of spleen
enlargement or cytopenias need to be treated." 5
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Primary pulmonary non-Hodgkin's lymphomas.
Histopathology.
1995 Jun;26(6):529-37. PMID: 7665143 PubMed
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Primary pulmonary lymphoma.
Eur Respir J. 2002
Sep;20(3):750-62.
PMID: 12358356 PubMed
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Complete Pathologic Remission of BALT Lymphoma with
Antibiotics, Case Report ASCO
2002
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Marginal zone-related neoplasms of splenic and nodal origin.
Haematologica. 2003 Jan;88(1):80-93. Review. PMID:
12551831 | Related
articles
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Marginal Zone B-Cell Lymphoma of Bronchus-Associated Lymphoid
Tissue (BALT): Imaging Findings in 21 Patients chestjournal.org
Marginal zone B-cell lymphomas of BALT manifest diverse
patterns of lung abnormality at CT, but single or multiple
nodule(s) or area(s) of consolidation are the main patterns that
occur in a majority (76%) of patients. Most lesions show
heterogeneous but identifiable FDG uptake at PET.
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Low-grade B-cell bronchial associated lymphoid tissue (BALT) lymphoma.
Cancer Invest. 2002;20(7-8):1059-68. Review.
PMID: 12449739
Low-grade B-cell bronchial associated lymphoid tissue (BALT) lymphoma is a distinct subgroup of non-Hodgkin's lymphoma. Chronic antigen stimulation, triggered by autoimmune process or persistent infection may precede the development of BALT lymphoma. The lymphoma cells originate from the marginal zone and by invading the bronchial epithelial tissue, give rise to the lymphoepithelial lesion. BALT lymphoma shares the morphologic, immunophenotypic, and cytogenetic characteristics of other mucosa associated lymphoid tissue lymphomas.
A majority of the patients are asymptomatic and pulmonary lesions are incidentally discovered on a routine chest radiograph. However, the clinical and radiographic features of BALT lymphoma are nonspecific. The disease is often localized at the time of diagnosis and responds favorably to local treatment, but the optimal management is not clearly defined. Overall, BALT lymphoma has a favorable prognosis and is associated with long-term survival.
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Splenic Marginal Zone
Lymphoma (SMZL)
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SMZL
B-cell stage
Mature (peripheral) neoplasms
Also see: Splenic
Lymphoma with Villous Lymphocytes
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TOPIC
SEARCH:
PubMed Diagnosis
| Review
| Therapies
| Prognosis
| Other
A recently described primary Splenic lymphoproliferative disorder
that mainly affects older individuals.
Splenic Marginal Zone Lymphoma is an indolent (slow
growing) b-cell lymphoma. It typically presents with an enlarged
spleen (splenomegaly). "Splenic lymphomas present with a massive
splenomegaly (enlarged spleen) sometimes with mesenteric lymph nodes
or hepatic involvement, but without peripheral lymph nodes; bone
marrow and blood are often involved." 2
Treatment: Splenectomy
is often indicated and can result in remissions. Treatment is similar
to other indolent lymphomas - not typically initiated until the
patient is symptomatic.
"Rituximab with or without chemotherapy was found to have
major activity in patients with SMZL. These results may be associated
with high levels of cellular CD20 antigen sites. Rituximab should be
the treatment of choice, at least in older patients with SMZL who have
comorbid diseases." [1]
About:
Splenic marginal zone lymphoma: clinical characteristics and
prognostic factors in a series of 60 patients - full text
bloodjournal.org
Splenic marginal zone lymphoma - Review article bloodjournal.org
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Resources:
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Outcomes in patients with splenic marginal zone lymphoma and
marginal zone lymphoma treated with rituximab with or without
chemotherapy or chemotherapy alone. Cancer. 2006 May 12; PMID:
16700034 | Related
articles
"CONCLUSIONS: Rituximab with or without chemotherapy was
found to have major activity in patients with SMZL. These results
may be associated with high levels of cellular CD20 antigen sites.
Rituximab should be the treatment of choice, at least in older
patients with SMZL who have comorbid diseases."
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Comparative study of marginal zone lymphoma involving bone
marrow.
Am J Clin Pathol. 2002 May;117(5):698-708.
PMID: 12090417 PubMed
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Splenic Marginal Zone Lymphoma Associated With Hepatitis B
Virus Infection:
A Case Report ispub.com
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Cutaneous
Marginal Zone
Lymphoma (PCMZL or MZCL)
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PCMZL or MZCL
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TOPIC SEARCH: ASCO
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PubMed
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WEB
Primary cutaneous marginal zone B-cell lymphoma (PCMZL)
is a low-grade (slow growing) b-cell lymphoma that originates in the
skin, with no evidence of extracutaneous disease.1
"Cutaneous marginal zone B-cell lymphoma is
a recently proposed entity and constitutes a cutaneous counterpart of
extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid
tissue (MALT)." 2
MZCL appears to be a well recognizable entity,
clinically, histologically and immunophenotypically. Although
prognosis is generally good, the disease has potential for skin as
well as extracutaneous recurrences. Large cell transformation and head
and neck presentation may be associated with a worse prognosis.3
Primary cutaneous B-cell lymphomas have been
associated with Borrelia burgdorferi, the spirochete responsible for
Lyme disease. 4 This
association warrants discussion of antibiotics as initial
treatment. See Related
PubMed articles
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Primary cutaneous marginal zone B-cell lymphoma: a molecular
and clinicopathologic study of 24 asian cases.
Am J Surg Pathol. 2003 Aug;27(8):1061-9. PMID:
12883238
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Primary cutaneous marginal zone B-cell lymphoma: a clinical,
histopathological, immunophenotypic and molecular genetic study of
22 cases. Br J Dermatol. 2002 Dec;147(6):1147-58. PMID:
12452864
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Eradication of Borrelia burgdorferi infection in primary
marginal zone B-cell lymphoma of the skin. Hum Pathol. 2000
Feb;31(2):263-8. PMID:
10685647
Primary cutaneous marginal zone B-cell lymphoma. Am J Clin
Pathol. 2006 Jun;125 Suppl:S38-49. PMID: 16830956
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