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Guidelines at Diagnosis | About Clinical Trials
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evidence-based support and information |
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Recommended Expert Overview:
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. * Incidence of marginal zone lymphoma in the United States, 2001–2009 with a focus on primary anatomic site http://bit.ly/2sHpSnt
Marginal Zone Lymphomas are
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H. pylori (gastric MALT)
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Helicobacter heilmannii (immunoproliferative small intestinal disease) |
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Hepatitis C Virus (Nodal MZL) |
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Campylobacter jejuni (cutaneous MALT) |
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B. burgdorferi from tick bite (cutaneous MALT - also Mantle Cell lymphoma) |
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C. psittaci from birds (orbital MALT) |
Sources :
Infection-associated lymphomas derived from marginal zone B cells: a model of antigen-driven lymphoproliferation. Blood. 2006 Review. PMID: 16397126 | Related articles
Hematology, 2012 Zinzani:
The many faces of marginal zone lymphoma http://bit.ly/1iGgRlcRoggero E, Zucca E, Mainetti C, et al.
Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin.
Hum Pathol. 2000;31:263–268. [PubMed]
Clinical Trials and Observations: Borrelia infection and risk of non-Hodgkin lymphoma http://1.usa.gov/1eViXJf
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Jan 2017: Ibrutinib - First Drug Approved for Marginal Zone Lymphoma http://wb.md/2u10bCf
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Incidence of marginal zone lymphoma in the United States, 2001–2009 with a focus on primary anatomic site http://bit.ly/2sHpSnt |
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Mediterr J Hematol Infect Dis. 2010: Hepatitis C Virus Infection and Lymphoma http://1.usa.gov/neu1Kz snip: "If HCV infection has been inferred to be a factor in the development of NHL on the basis of case-control epidemiological studies as previously discussed, strong line of evidence also arose from response of so called HCV-associated lymphoma to antiviral therapy. Of the 9 IFN-treated patients, 7 achieved a complete hematological remission, defined by the absence of abnormal lymphocytosis and the resolution of the splenomegaly, after HCV RNA load became undetectable. The remaining 2 patients experienced a partial or a complete response after addition of ribavirin and the loss of detectable HCV RNA. Conversely, none of 6 similarly treated HCV-negative SLVL patients responded to therapy thereby suggesting that the observed response rate was not due to the effect of interferon itself. __________________ |
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Hepatitis C in Hematological Patients http://1.usa.gov/oQExvz
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Cancer: Prognostic value of the Follicular Lymphoma International Prognostic Index (FLIPI) score in marginal zone lymphoma: An analysis of clinical presentation and outcome in 144 patients. with comments. |
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ASCO 2012 Marginal Zone Lymphoma abstracts
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MALT - MedWire:
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MALT - Alimentary Pharmacology and Therapeutics:
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Marginal Zone - Annals of Hematology:
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"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
There are three distinct forms (Harris et al, 1999) of marginal zone lymphomas:
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Extranodal
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Uncommon subtypes:
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Non-Hodgkins Lymphomas
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Technical: Nodal Marginal Zone Lymphoma - Differential Diagnosis: surgpathcriteria.stanford.edu/ |
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Marginal Zone NHL, overview - DoctorsDoctor | infobiogen |
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Lymphomas outside the lymphatic system
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Overview |
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Cutaneous |
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Hepatic |
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Conjunctival/Ocular/Orbital |
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Osteo |
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Oral |
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Treatment, age and gender specific:
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Nongastric marginal zone B-cell lymphoma: Analysis of 247 cases.
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"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.
Monitoring MZL with PET?
See PET
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.
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See Factors that determine treatment timing and approach |
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See Flow chart for frontline indolent NHL |
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Your age and treatment priorities |
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Non-gastric Marginal Zone B-cell Lymphoma in Korea: Clinical Features, Treatment, and Prognostic Factors http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2932933/ |
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Marginal zone lymphomas - Factors that affect outcome interscience.wiley
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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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Find trials in the ClinicalTrials.gov registry |
Also see Prognostic Indicators
"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
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Splenic marginal zone lymphoma: a prognostic model for clinical use.
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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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2018: PI3K Inhibition With Copanlisib in Relapsed or Refractory Indolent Lymphoma (including Marginal Zone lymphoma) http://bit.ly/2F6hggE |
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Clarithromycin as a “repurposing drug” against MALT lymphoma - Ferreri - 2017 - British Journal of Haematology - Wiley Online Library http://bit.ly/2wkWlCm
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Added June 2015:
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Nongastric marginal zone B-cell lymphoma: Analysis of 247 cases.
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Rituxan® Effective in Treatment of Splenic Marginal Zone Lymphoma and Marginal Zone Lymphoma cancerconsultants.com
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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 | Related 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 | PDF Help |
We moved this most common type of Marginal Zone Lymphoma
to the MALT page
"There is currently no consensus on how to treat NMZL patients. Usually, guidelines for follicular lymphoma or chronic lymphocytic leukemia/small lymphocytic lymphoma are adopted.
Different studies have reported different (combinations of) treatments, including watchful waiting, surgery, radiotherapy, different chemotherapeutic regimens, rituximab, and autologous stem cell transplantation. However, no optimal treatment strategy can be deduced from these studies. Complete response is achieved in 55–74% of patients."".... Nodal marginal zone lymphoma remains an enigmatic entity with accompanying difficulties in diagnosis and a lack of knowledge of prognosis and treatment. Because of its rarity, it is hard to obtain large study groups. Also, because NMZL is frequently a diagnosis of exclusion, the series that have been studied might contain a somewhat heterogeneous group of low-grade B-cell lymphomas. Nevertheless, progress is being made; recent studies have identified positive markers for MZL (i.e. MNDA and IRTA1), and gene expression studies have identified a specific gene expression profile that separates NMZL from other lymphoma types.
Source: Haematologica, 2013: Recognizing nodal marginal zone lymphoma: recent advances and pitfalls. A systematic review http://1.usa.gov/1kDQVXp
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Treatment, age and gender specific:
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TOPIC SEARCH: PubMed
Diagnosis:
As of this writing, there is a need for less invasive procedures to diagnose lesions that present in the lung .... which may be obtained by
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video-assisted thoracoscopic (VATS) mayoclinic.org |
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open thoracotomy nlm.nih.gov |
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ultrasound-guided percutaneous biopsy ncbi.nlm.nih.gov |
Source
A favorable prognosis:
Most patients diagnosed with BALT are without symptoms and pulmonary lesions are incidentally discovered on a routine chest radiograph, which cannot be distinguished from other conditions by imaging features.
"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." 6
Recent Reports:
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2015: Pulmonary MALT lymphoma: A case report and review of the literature http://1.usa.gov/1RREdYA SNIP: Chemotherapy with chlorambucil currently appears to be the optimal treatment option for cases with disseminated disease (5). The therapeutic role of rituximab remains unclear, since thus far there is only a limited number of case reports with conflicting results (14,16–17). However, as pMALToma cells express the CD20 antigen, the therapeutic possibility of rituximab appears to be promising, either in combination with chemotherapy or as a single agent. Recently, Zinzani et al (12) reported an observational retrospective study on homogeneous clinical data from 17 patients with pMALToma; all the patients were treated with fludarabine and mitoxantrone-containing regimens. The immunochemotherapy mentioned above achieved a high response rate: 82.3% of the patients achieved a complete response and 11.8% a partial response. Furthermore, a remarkable progression-free survival (71%) and overall survival (100%) were reported at 14 years. The approach to the case presented here was treatment with chlorambucil combined with prednisolone and vincristine, which was effective in achieving a complete remission. |
Treatment:
"The optimal management of BALT lymphoma with regard to surgery, chemotherapy, and radiation therapy alone or in combination, as well as abstention from therapy, has yet to be clearly determined.
From the perspective of MZL, localized PMZL, which is limited to one side of the lung, may be a marker of favorable response to local radiation or operation [9, 11, 14].
Additionally, advanced or disseminated P-MZL, involving bilateral lung or extra-pulmonary sites, could be controlled via chemotherapy. However, in the lung, even though the lesions were localized, radiation and surgical excision of segments or lobes should be carefully considered due to surgical complications, reductions in organ function, and a favorable clinical course of MZL itself.In our series, we noted no difference between chemotherapy and operation in TTP and OS. This was the case even in the patients with single-lobe or unilateral P-MZL. Thus, in patients for whom surgery is not necessary for diagnosis, the operation might not be the first choice of PMZL treatment to preserve lung function and avoid the risks of surgery like any other primary pulmonary NHL [15, 16].
In another study, watchful vigilance until patients developed symptoms made no difference in terms of the efficacy of TTP treatment [17]. "Watchful waiting" might constitute another treatment option for asymptomatic patients. In order to assess the efficacy of this approach, a prospective multi-center randomized study will be necessary." 8 ncbi.nlm.nih.gov
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."
SMZL
B-cell stage
Mature (peripheral) neoplasms
Also see: Splenic Lymphoma with Villous Lymphocytes
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."
"Many cases show a protracted uncomplicated evolution, a good response to splenectomy or chemotherapy, or even an unmodified clinical picture in the absence of any kind of treatment. " 2
"Splenectomy will rapidly improve performance status and correct anemia, thrombocytopenia, and neutropenia within 6 months of the procedure.[19] This improvement is maintained for years, with a median period of freedom from treatment of 8 years, even if bone marrow and blood lymphocytosis persist, suggesting a partial response. Adjuvant chemotherapy following splenectomy provides an increased remission rate without modifying relapse-free and overall survival." 6
Antiviral therapy can lead to clearance of HCV RNA in 75% of patients and to concomitant clinical remission in SMZL associated with active HCV infection. 6
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2017, Splenic marginal zone lymphoma: from genetics to management
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ASH Paper: Outcomes In Splenic Marginal Zone Lymphoma: Analysis Of 108 Patients Treated In British Columbia http://bit.ly/1h7ozoK |
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Leuk Lymphoma 2013:
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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]"French authors reported on a series of 18 patients with splenic lymphoma with villous lymphocytes associated with type II cryoglobulinemia and HCV infection,8 some of whom were responsive to antiviral treatment. " [4]
According to NCCN guidelines (2011), the appropriate treatment (when indicated) depends in part on test results for Hepatitis C and guidance from a Hepatology consult if Hep C positive. Otherwise, except for splenectomy or Rituxan mono-therapy as first treatment, SMZL is treated like follicular lymphoma.
Source Department of Hematology, Ha'Emek Medical Center, Afula, Israel.
AbstractSplenic marginal zone lymphoma (SMZL) is an uncommon indolent B-cell lymphoma causing marked splenic enlargement with CD20-rich lymphoma cells infiltrating blood and bone marrow. In the pre-rituximab era, the treatment of choice for patients with symptomatic splenomegaly or threatening cytopenia was splenectomy, since chemotherapy had limited efficacy.
Responses to splenectomy occurred in approximately 90% of patients. However, SMZL patients are often elderly and poor surgical risks.
Since approval of rituximab, treatment of such patients with the anti-CD20 antibody both alone or in combination with chemotherapy has shown remarkable responses. In retrospective series of rituximab monotherapy totaling 52 patients, including both chemotherapy-naive and -refractory patients, overall responses of 88% to 100% were noted with marked and prompt regression of splenomegaly and improvement of cytopenias.
Sustained responses occurred both with and without rituximab maintenance in 60% to 88% of patients at 3 years. Relapsed patients responded to second courses of rituximab monotherapy.
Overall survival was comparable to that reported following splenectomy. Rituximab in combination with purine nucleosides may provide further improvement in progression-free survival; however, confirmatory prospective trials are necessary.
These results suggest that splenectomy should no longer be considered as initial therapy for SMZL but rather as palliative therapy for patients not responsive to immunotherapy with or without chemotherapy.
PMID:20350661
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 (what it expresses on the cell surface).
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
TOPIC SEARCH: PubMed: Diagnosis | Review | Therapies | Prognosis