SMZL
MCID: SPL004
MIFTS: 47

Splenic Marginal Zone Lymphoma (SMZL) malady

Blood diseases, Cancer diseases, Rare diseases, Immune diseases categories

Summaries for Splenic Marginal Zone Lymphoma

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9Disease Ontology, 66Wikipedia, 34MalaCards
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Wikipedia:66 Splenic marginal zone lymphoma (SMZL) is a lymphoma made up of B-cells that replace the normal... more...

MalaCards: Splenic Marginal Zone Lymphoma, also known as splenic marginal zone b-cell lymphoma, is related to b-cell lymphomas and malt lymphoma. An important gene associated with Splenic Marginal Zone Lymphoma is AICDA (activation-induced cytidine deaminase), and among its related pathways are IL4-mediated signaling events and Hematopoietic cell lineage. The compounds calcitriol and zinc have been mentioned in the context of this disorder. Affiliated tissues include the spleen comprised of b-cells in place of white pulp, b cells and spleen, and related mouse phenotypes are digestive/alimentary and mortality/aging.

Disease Ontology:9 A marginal zone b-cell lymphocyte located in the spleen comprised of b-cells in place of white pulp.

Aliases & Classifications for Splenic Marginal Zone Lymphoma

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9Disease Ontology, 11DISEASES, 50Orphanet, 32LifeMap Discoveryā„¢, 63UMLS, 60SNOMED-CT via Orphanet, 27ICD10 via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases, Rare diseases
Anatomical: Blood diseases, Immune diseases


Characteristics (Orphanet epidemiological data):

50
splenic marginal zone lymphoma:
Age of onset: Adulthood


Aliases & Descriptions:

splenic marginal zone lymphoma 9 11 50 32
splenic marginal zone b-cell lymphoma 63
smzl 50


External Ids:

Disease Ontology9 DOID:0050750
SNOMED-CT via Orphanet60 128802003
ICD10 via Orphanet27 C83.0

Related Diseases for Splenic Marginal Zone Lymphoma

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18GeneCards, 19GeneDecks
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Graphical network of the top 20 diseases related to Splenic Marginal Zone Lymphoma:



Diseases related to splenic marginal zone lymphoma

Symptoms for Splenic Marginal Zone Lymphoma

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Drugs & Therapeutics for Splenic Marginal Zone Lymphoma

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 32LifeMap Discoveryā„¢
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Approved drugs:

Search CenterWatch for Splenic Marginal Zone Lymphoma

Drug clinical trials:

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Search NIH Clinical Center for Splenic Marginal Zone Lymphoma

Search CenterWatch for Splenic Marginal Zone Lymphoma

Cell-based therapeutics:


LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine
Read about Splenic Marginal Zone Lymphoma cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Splenic Marginal Zone Lymphoma:
Hematopoietic stem cells for treatment of hematologic malignancies
Embryonic/Adult Cultured Cells Related to Splenic Marginal Zone Lymphoma:
Bone marrow-derived hematopoietic stem cells

Genetic Tests for Splenic Marginal Zone Lymphoma

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Anatomical Context for Splenic Marginal Zone Lymphoma

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34MalaCards, 15FMA
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MalaCards organs/tissues related to Splenic Marginal Zone Lymphoma:

34
B cells, Spleen, Bone, Bone marrow, Lymph node, T cells, Brain

FMA organs/tissues related to Splenic Marginal Zone Lymphoma:

15
The spleen comprised of b-cells in place of white pulp

Animal Models for Splenic Marginal Zone Lymphoma or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Splenic Marginal Zone Lymphoma:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053818.5AICDA, FCER2, NFKB2, NOTCH2
2MP:00107688.0AICDA, GFI1, FCER2, NFKB2, NOTCH2
3MP:00053847.7NOTCH2, NFKB2, FCER2, GFI1, AICDA
4MP:00053976.8AICDA, CD5, NOTCH2, NFKB2, MALT1, FCER2
5MP:00053876.7NOTCH2, NFKB2, MALT1, FCER2, GFI1, AICDA

Publications for Splenic Marginal Zone Lymphoma

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53PubMed
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Articles related to Splenic Marginal Zone Lymphoma:

(show top 50)    (show all 167)
idTitleAuthorsYear
1
A peculiar case of splenic marginal zone lymphoma and review of literature. (24076596)
2013
2
Treatment of splenic marginal zone lymphoma with rituximab monotherapy: progress report and comparison with splenectomy. (23345547)
2013
3
Splenic marginal zone lymphoma: comprehensive analysis of gene expression and miRNA profiling. (23429603)
2013
4
Splenic marginal zone lymphoma with villous lymphocytes in patients with human immunodeficiency virus. (22646999)
2013
5
Clinical characteristics and outcome in dogs with splenic marginal zone lymphoma. (23734665)
2013
6
Immunoarchitectural patterns in splenic marginal zone lymphoma: correlations with chromosomal aberrations, IGHV mutations, and survival. A study of 76 cases. (23611359)
2013
7
Nuclear factor-I_B dysregulation in splenic marginal zone lymphoma: new therapeutic opportunities. (22556352)
2012
8
Whole-genome sequencing identifies recurrent somatic NOTCH2 mutations in splenic marginal zone lymphoma. (22891276)
2012
9
MicroRNA expression profile in splenic marginal zone lymphoma. (21902683)
2012
10
Dysregulation of global microRNA expression in splenic marginal zone lymphoma and influence of chronic hepatitis C virus infection. (22307176)
2012
11
Systemic mastocytosis associated with splenic marginal zone lymphoma with villous lymphocytes. (21785582)
2011
12
De novo large B-cell lymphoma in a parenchymal brain lesion: evidence of clonal evolution from splenic marginal zone lymphoma. (21205746)
2011
13
Immunogenetics features and genomic lesions in splenic marginal zone lymphoma. (21113978)
2010
14
Rituximab monotherapy for splenic marginal zone lymphoma with villous lymphocytes: report on long-term disease control for two patients with recurrence after splenectomy. (21308163)
2010
15
Higher expression levels of activation-induced cytidine deaminase distinguish hairy cell leukemia from hairy cell leukemia-variant and splenic marginal zone lymphoma. (20237507)
2010
16
Defining the borders of splenic marginal zone lymphoma: a multiparameter study. (20004934)
2010
17
Autoimmune hemolytic anemia as a risk factor of poor outcome in patients with splenic marginal zone lymphoma. (19343544)
2009
18
Splenectomy with chemotherapy vs surgery alone as initial treatment for splenic marginal zone lymphoma. (19705496)
2009
19
FOXP1 status in splenic marginal zone lymphoma: a fluorescence in situ hybridization and immunohistochemistry approach. (19760589)
2009
20
Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells. (18665362)
2009
21
Splenic marginal zone lymphoma: Clinical clustering of immunoglobulin heavy chain repertoires. (19250848)
2009
22
Pure red cell aplasia as first manifestation of splenic marginal zone lymphoma-successful treatment with rituximab: a case report. (19918496)
2009
23
High-grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis. (18671706)
2008
24
The prevalence of IG translocations and 7q32 deletions in splenic marginal zone lymphoma. (17989713)
2008
25
A unique case of IgD-only splenic marginal-zone lymphoma with mutated immunoglobulin genes: ontogenetic implications. (17420049)
2008
26
Splenic marginal zone lymphoma--a clinicopathological study in a series of 16 patients. (18854089)
2008
27
Composite splenic marginal zone lymphoma and classic Hodgkin lymphoma -- an unusual combination. (17988302)
2007
28
Establishment and characterization of the new splenic marginal zone lymphoma-derived cell line UCH1 carrying a complex rearrangement involving t(8;14) and chromosome 3. (17454636)
2007
29
Coexistence of splenic marginal zone lymphoma with hepatocellular carcinoma: a case report. (17284308)
2007
30
Composite splenic marginal zone lymphoma and mantle cell lymphoma arising from 2 independent B-cell clones. (17134739)
2007
31
Unusual gamma heavy chain disease protein in a patient with splenic marginal-zone lymphoma. (16536920)
2006
32
Intrasinusoidal bone marrow infiltration and splenic marginal zone lymphoma: a quantitative study. (16480431)
2006
33
Splenic marginal zone lymphoma-like features in API2-MALT1 transgenic mice that are exposed to antigenic stimulation. (17145608)
2006
34
Activity of rituximab monotherapy in refractory splenic marginal zone lymphoma complicated with autoimmune hemolytic anemia. (16796783)
2006
35
Splenic marginal zone lymphoma: a prognostic model for clinical use. (16493005)
2006
36
Regression of advanced non-splenic marginal zone lymphoma after treatment of hepatitis C virus infection. (16109616)
2005
37
Meningeal lymphomatosis as the first manifestation of splenic marginal zone lymphoma. (16105762)
2005
38
Deoxycoformycin (pentostatin) in the treatment of splenic marginal zone lymphoma (SMZL) with or without villous lymphocytes. (16000129)
2005
39
Constitutive expression of the AP-1 transcription factors c-jun, junD, junB, and c-fos and the marginal zone B-cell transcription factor Notch2 in splenic marginal zone lymphoma. (15507668)
2004
40
Splenic marginal zone lymphoma. (12945935)
2003
41
Splenic marginal zone lymphoma with villous lymphocytes shows on-going immunoglobulin gene mutations. (12547726)
2003
42
Successful rituximab therapy for hemolytic anemia associated with relapsed splenic marginal zone lymphoma with leukemic phase. (14959867)
2003
43
Small solitary littoral cell angioma associated with splenic marginal zone lymphoma and villous lymphocyte leukaemia in a patient with hepatitis C infection. (12405918)
2002
44
Splenic marginal zone lymphoma: clinical characteristics and prognostic factors in a series of 60 patients. (12176884)
2002
45
Simultaneous presence of t(2;8)(p12;q24) and t(14;18)(q32;q21) in a B-cell lymphoproliferative disorder with features suggestive of an aggressive variant of splenic marginal-zone lymphoma. (10942804)
2000
46
Incidence and subtype specificity of API2-MALT1 fusion translocations in extranodal, nodal, and splenic marginal zone lymphomas. (10751343)
2000
47
Absence of cyclin D1 protein expression in splenic marginal zone lymphoma. (9688179)
1998
48
Splenic marginal zone lymphoma. A distinct B-cell neoplasm. (8619426)
1996
49
Splenic marginal zone lymphoma. (8695824)
1996
50
Splenic marginal zone lymphoma: a distinctive type of low-grade B-cell lymphoma. A clinicopathological study of 13 cases. (7573673)
1995

Variations for Splenic Marginal Zone Lymphoma

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Expression for genes affiliated with Splenic Marginal Zone Lymphoma

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Splenic Marginal Zone Lymphoma

Search GEO for disease gene expression data for Splenic Marginal Zone Lymphoma.

Pathways for genes affiliated with Splenic Marginal Zone Lymphoma

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51PathCards, 39NCBI BioSystems Database, 31KEGG, 61Thomson Reuters, 13EMD Millipore, 54QIAGEN, 56Reactome, 55R&D Systems, 5Cell Signaling Technology
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Pathways related to Splenic Marginal Zone Lymphoma according to GeneCards/GeneDecks:

(show all 14)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6FCER2, AICDA
29.6CD5, FCER2
3
Show member pathways
Immune response IL 4 signaling pathway61
Signal transduction AKT signaling61
9.5NFKB2, FCER2
49.5NFKB2, FCER2
5
Show member pathways
RANKL/RANK Signaling Pathway39
Apoptosis and survival APRIL and BAFF signaling61
9.5FCER2, NFKB2
6
Show member pathways
9.5NOTCH2, FCER2
7
Show member pathways
9.5NOTCH2, FCER2
89.4NFKB2, NOTCH2
99.4NOTCH2, NFKB2
109.3MALT1, AICDA
119.1MALT1, NFKB2
12
Show member pathways
Immune response Role of TLRs 3 and 4 in cell antiviral response TICAM1 specific signaling pathways61
Immune response IL 1 signaling pathway61
9.1NFKB2, MALT1
13
Show member pathways
Immune response T cell receptor signaling pathway61
Immune response CD28 signaling61
Immune response ICOS pathway in T helper cell61
9.1MALT1, NFKB2
149.1NFKB2, MALT1

Compounds for genes affiliated with Splenic Marginal Zone Lymphoma

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46Novoseek, 62Tocris Bioscience, 25HMDB, 12DrugBank
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Compounds related to Splenic Marginal Zone Lymphoma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1calcitriol46 62 25 1212.4FCER2, GFI1
2zinc46 259.7FCER2, GFI1, AICDA
3paraffin468.7CD5, MALT1, FCER2

GO Terms for genes affiliated with Splenic Marginal Zone Lymphoma

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17Gene Ontology
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Cellular components related to Splenic Marginal Zone Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:0098979.6CD5, FCER2
2nucleusGO:0056347.3NOTCH2, NFKB2, MALT1, GFI1, AICDA

Biological processes related to Splenic Marginal Zone Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cellular response to lipopolysaccharideGO:0712229.4GFI1, AICDA
2Notch signaling pathwayGO:0072199.2NOTCH2, FCER2
3positive regulation of NF-kappaB transcription factor activityGO:0510929.1NFKB2, MALT1

Molecular functions related to Splenic Marginal Zone Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein bindingGO:0055157.2CD5, NOTCH2, NFKB2, MALT1, GFI1, AICDA

Products for genes affiliated with Splenic Marginal Zone Lymphoma

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  • Antibodies
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Sources for Splenic Marginal Zone Lymphoma

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet