SMZL
MCID: SPL004
MIFTS: 53

Splenic Marginal Zone Lymphoma (SMZL) malady

Blood diseases, Cancer diseases, Immune diseases categories

Summaries for Splenic Marginal Zone Lymphoma

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8Disease Ontology, 63Wikipedia, 32MalaCards
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Wikipedia:63 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 hepatitis c and myeloma. An important gene associated with Splenic Marginal Zone Lymphoma is GFI1 (growth factor independent 1 transcription repressor), and among its related pathways are Wnt / Hedgehog / Notch and Toll-Like receptor Signaling Pathways. The compounds sant-1 and 20(s)-hydroxycholesterol 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 endocrine/exocrine gland and tumorigenesis.

Disease Ontology:8 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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8Disease Ontology, 10DISEASES, 48Orphanet, 30LifeMap Discovery™, 60UMLS, 57SNOMED-CT via Orphanet, 26ICD10 via Orphanet
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Classifications:

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


Characteristics (Orphanet epidemiological data):

48
splenic marginal zone lymphoma:
Age of onset: Adulthood


Aliases & Descriptions:

splenic marginal zone lymphoma 8 10 48 30
splenic marginal zone b-cell lymphoma 60
smzl 48


External Ids:

Disease Ontology8 DOID:0050750
SNOMED-CT via Orphanet57 128802003
ICD10 via Orphanet26 C83.0

Related Diseases for Splenic Marginal Zone Lymphoma

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Splenic Marginal Zone Lymphoma:



Diseases related to splenic marginal zone lymphoma

Clinical Features for Splenic Marginal Zone Lymphoma

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Drugs & Therapeutics for Splenic Marginal Zone Lymphoma

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 30LifeMap Discovery™
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Approved drugs:

Search CenterWatch for Splenic Marginal Zone Lymphoma

Drug clinical trials:

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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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32MalaCards, 14FMA
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MalaCards organs/tissues related to Splenic Marginal Zone Lymphoma:

32
B cells, Spleen, Bone, Bone marrow, Lymph node, T cells, Brain, Liver

FMA organs/tissues related to Splenic Marginal Zone Lymphoma:

14
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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36MGI
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MGI Mouse Phenotypes related to Splenic Marginal Zone Lymphoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537910.3JUND, SOX4
2MP:000200610.1AICDA, BTK, NOTCH2, SDC1
3MP:00028739.8AICDA, SOX4, GLI1, CD5, IRF5, SHH
4MP:00053819.7NOTCH2, GLI1, AICDA, SHH, SOX4, BTK
5MP:00053889.7SOX4, SHH, GLI1, NOTCH2, BCL6, CAV2
6MP:00053979.4BCL6, JUND, SOX4, IRF5, SHH, AICDA
7MP:00107689.3JUND, NOTCH2, BCL6, SDC1, BTK, GLI1
8MP:00053879.2BTK, MALT1, GFI1, SDC1, BCL6, CD5
9MP:00053789.1SHH, GLI1, BTK, SOX4, CAV2, JUND
10MP:00053849.0MALT1, GFI1, CD5, BTK, GLI1, BCL6

Publications for Splenic Marginal Zone Lymphoma

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50PubMed
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Articles related to Splenic Marginal Zone Lymphoma:

(show top 50)    (show all 200)
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
Clinical characteristics and outcome in dogs with splenic marginal zone lymphoma. (23734665)
2013
4
Immunoarchitectural patterns in splenic marginal zone lymphoma: correlations with chromosomal aberrations, IGHV mutations, and survival. A study of 76 cases. (23611359)
2013
5
Simplification of risk stratification for splenic marginal zone lymphoma: a point-based score for practical use. (23799931)
2013
6
Platelet satellitism and dual surface immunoglobulin light-chain expression in circulating splenic marginal zone lymphoma cells. (21889383)
2013
7
Comparative outcomes of rituximab-based systemic therapy and splenectomy in splenic marginal zone lymphoma. (24057925)
2013
8
Dysregulation of global microRNA expression in splenic marginal zone lymphoma and influence of chronic hepatitis C virus infection. (22307176)
2012
9
Follicular lymphoma with prominent Dutcher body formation: a pathologic study of 3 cases in comparison with nodal or splenic lymphoplasmacytic lymphoma and marginal zone lymphoma. (22607703)
2012
10
Selection of antigen receptors in splenic marginal-zone lymphoma: further support from the analysis of the immunoglobulin light-chain gene repertoire. (22858907)
2012
11
High-throughput sequencing analysis of the chromosome 7q32 deletion reveals IRF5 as a potential tumour suppressor in splenic marginal-zone lymphoma. (22816737)
2012
12
Rituximab, used alone or in combination, is superior to other treatment modalities in splenic marginal zone lymphoma. (23016878)
2012
13
Splenic marginal zone lymphoma: villous, not necessarily villainous. (22489358)
2012
14
Splenic marginal zone B-cell lymphoma: a distinct clinicopathological and molecular entity. Recent advances in ontogeny and classification. (21760505)
2011
15
CD138 expression helps distinguishing WaldenstrAPm's macroglobulinemia (WM) from splenic marginal zone lymphoma (SMZL). (21454203)
2011
16
Absence of mutations of the histone methyltransferase gene EZH2 in splenic b-cell marginal zone lymphoma. (21087793)
2011
17
Immunogenetics features and genomic lesions in splenic marginal zone lymphoma. (21113978)
2010
18
Efficacy of rituximab in autoimmune hemolytic anemia associated with splenic marginal zone lymphoma. (19578848)
2010
19
Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab. (20350661)
2010
20
Splenic marginal zone lymphoma: hydra with many heads? (20378575)
2010
21
FOXP1 status in splenic marginal zone lymphoma: a fluorescence in situ hybridization and immunohistochemistry approach. (19760589)
2009
22
Successful treatment with a rituximab-based regimen of a splenic marginal zone lymphoma with villous lymphocytes in a very frail patient on maintenance dialysis. (18500519)
2009
23
Rapid improvement of massive bloody pleural effusion after splenectomy for splenic marginal zone lymphoma. (21687049)
2009
24
A unique case of IgD-only splenic marginal-zone lymphoma with mutated immunoglobulin genes: ontogenetic implications. (17420049)
2008
25
Splenic marginal zone lymphoma--a clinicopathological study in a series of 16 patients. (18854089)
2008
26
Splenic marginal zone lymphoma: transformation to diffuse large B-cell lymphoma with isolated cerebral manifestation. (18802164)
2008
27
Progressive respiratory distress in a patient with splenic marginal zone lymphoma. (18591858)
2008
28
Splenic marginal zone lymphoma-like features in API2-MALT1 transgenic mice that are exposed to antigenic stimulation. (17145608)
2006
29
Multiple autoimmune phenomena in splenic marginal zone lymphoma. (16886284)
2006
30
Outcomes in patients with splenic marginal zone lymphoma and marginal zone lymphoma treated with rituximab with or without chemotherapy or chemotherapy alone. (16700034)
2006
31
Splenic marginal zone lymphoma with predominant red pulp involvement lacking p53 overexpression. (17071813)
2006
32
Acadesine induces apoptosis in B cells from mantle cell lymphoma and splenic marginal zone lymphoma. (15549145)
2005
33
Images of interest. Gastrointestinal: white small bowel, macroglobulinemia and splenic marginal-zone lymphoma. (16174087)
2005
34
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
35
Systematic screening for HCV infection should be performed in patients with splenic marginal zone lymphoma. (14687039)
2004
36
CD27 distinguishes two phases in bone marrow infiltration of splenic marginal zone lymphoma. (15049905)
2004
37
Splenic marginal zone lymphoma with villous lymphocytes shows on-going immunoglobulin gene mutations. (12547726)
2003
38
Splenic marginal zone lymphoma. (12446449)
2003
39
Patterns of bone marrow involvement in 58 patients presenting primary splenic marginal zone lymphoma with or without circulating villous lymphocytes. (12877667)
2003
40
Splenic marginal zone lymphomas presenting with splenomegaly and typical immunophenotype are characterized by allelic loss in 7q31-32. (14681321)
2003
41
Splenic marginal zone lymphoma: clinical characteristics and prognostic factors in a series of 60 patients. (12176884)
2002
42
Spontaneous regression of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma presenting with recurrent angio-oedema. (12354812)
2002
43
Treatment of splenic marginal zone B-cell lymphoma: an analysis of 81 patients. (12141954)
2002
44
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
45
Incidence and subtype specificity of API2-MALT1 fusion translocations in extranodal, nodal, and splenic marginal zone lymphomas. (10751343)
2000
46
The relationship between splenic marginal zone B-cell lymphoma and chronic liver disease associated with hepatitis C virus infection. (10638021)
1999
47
7q31-32 allelic loss is a frequent finding in splenic marginal zone lymphoma. (10329610)
1999
48
Incidental early detection of a splenic marginal zone lymphoma by polymerase chain reaction analysis of paraffin-embedded tissue. (9448023)
1998
49
Splenic marginal zone cell lymphoma. An immunophenotypic and molecular study of five cases. (8602607)
1996
50
Splenic marginal zone cell lymphoma: report of an indolent variant without massive splenomegaly presumably representing an early phase of the disease. (7821915)
1995

Genetic Variations for Splenic Marginal Zone Lymphoma

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Expression for genes affiliated with Splenic Marginal Zone Lymphoma

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1BioGPS, 15Gene 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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4Cell Signaling Technology, 52R&D Systems, 12EMD Millipore, 29KEGG, 37NCBI BioSystems Database
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Compounds for genes affiliated with Splenic Marginal Zone Lymphoma

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59Tocris Bioscience, 44Novoseek
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Compounds related to Splenic Marginal Zone Lymphoma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1sant-15910.3GLI1, SHH
220(s)-hydroxycholesterol5910.3SHH, GLI1
3cyclopamine44 5911.3GLI1, SHH
4ciliobrevin a5910.2SHH, GLI1
5ay 9944 dihydrochloride5910.0GLI1, SHH

GO Terms for genes affiliated with Splenic Marginal Zone Lymphoma

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16Gene Ontology
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Cellular components related to Splenic Marginal Zone Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1membrane raftGO:04512110.1BTK, SHH, CAV2
2protein complexGO:0432349.8SDC1, MALT1, CAV2, JUND
3nucleusGO:0056348.8SHH, IRF5, SOX4, CAV2, JUND, AICDA

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

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1ventral midline developmentGO:00741810.5GLI1, SHH
2smoothened signaling pathway involved in regulation of cerebellar granule cell precursor cell proliferationGO:02193810.4GLI1, SHH
3limb bud formationGO:06017410.4SHH, SOX4
4spinal cord motor neuron differentiationGO:02152210.3SHH, SOX4
5positive regulation of smoothened signaling pathwayGO:04588010.3SHH, GLI1
6positive regulation of Wnt receptor signaling pathwayGO:03017710.3SHH, SOX4
7canonical Wnt receptor signaling pathwayGO:06007010.2SOX4, SHH, SDC1
8osteoblast developmentGO:00207610.1JUND, SHH
9positive regulation of transcription from RNA polymerase II promoterGO:0459449.6GLI1, SHH, IRF5, SOX4, JUND

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

idNameGO IDScoreTop Affiliating Genes
1protein bindingGO:0055158.7SDC1, JUND, CAV2, SOX4, IRF5, SHH

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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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet