SMZL
MCID: SPL004
MIFTS: 47

Splenic Marginal Zone Lymphoma (SMZL) malady

Blood diseases, Cancer diseases, Rare diseases, Immune diseases categories
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Summaries for Splenic Marginal Zone Lymphoma

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Wikipedia:65 Splenic marginal zone lymphoma (SMZL) is a lymphoma made up of B-cells that replace the normal... more...

MalaCards based summary: Splenic Marginal Zone Lymphoma, also known as splenic marginal zone b-cell lymphoma, is related to malt lymphoma and b-cell lymphomas. 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: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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Splenic Marginal Zone Lymphoma, Aliases & Descriptions:

Name: Splenic Marginal Zone Lymphoma 30 8 10 48
Splenic Marginal Zone B-Cell Lymphoma 62
 
Smzl 48


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
splenic marginal zone lymphoma:
Age of onset: Adulthood


External Ids:

Disease Ontology8 DOID:0050750
ICD10 via Orphanet26 C83.0

Related Diseases for Splenic Marginal Zone Lymphoma

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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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Drug clinical trials:

Search ClinicalTrials for Splenic Marginal Zone Lymphoma

Search NIH Clinical Center 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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MalaCards organs/tissues related to Splenic Marginal Zone Lymphoma:

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

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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MGI Mouse Phenotypes related to Splenic Marginal Zone Lymphoma:

36
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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Articles related to Splenic Marginal Zone Lymphoma:

(show top 50)    (show all 179)
idTitleAuthorsYear
1
Splenic marginal-zone lymphoma: ontogeny and genetics. (24798744)
2014
2
Rare Presentation of Secondary Cutaneous Involvement by Splenic Marginal Zone Lymphoma: Report of a Case and Review of the Literature. (25238446)
2014
3
Splenic marginal zone lymphoma involving the breast: lessons in patient-centered care. (24521384)
2014
4
A peculiar case of splenic marginal zone lymphoma and review of literature. (24076596)
2013
5
Treatment of splenic marginal zone lymphoma with rituximab monotherapy: progress report and comparison with splenectomy. (23345547)
2013
6
Splenic marginal zone lymphoma: comprehensive analysis of gene expression and miRNA profiling. (23429603)
2013
7
Splenic marginal zone lymphoma with villous lymphocytes in patients with human immunodeficiency virus. (22646999)
2013
8
Clinical characteristics and outcome in dogs with splenic marginal zone lymphoma. (23734665)
2013
9
Immunoarchitectural patterns in splenic marginal zone lymphoma: correlations with chromosomal aberrations, IGHV mutations, and survival. A study of 76 cases. (23611359)
2013
10
Nuclear factor-I_B dysregulation in splenic marginal zone lymphoma: new therapeutic opportunities. (22556352)
2012
11
Whole-genome sequencing identifies recurrent somatic NOTCH2 mutations in splenic marginal zone lymphoma. (22891276)
2012
12
MicroRNA expression profile in splenic marginal zone lymphoma. (21902683)
2012
13
Systemic mastocytosis associated with splenic marginal zone lymphoma with villous lymphocytes. (21785582)
2011
14
De novo large B-cell lymphoma in a parenchymal brain lesion: evidence of clonal evolution from splenic marginal zone lymphoma. (21205746)
2011
15
Immunogenetics features and genomic lesions in splenic marginal zone lymphoma. (21113978)
2010
16
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
17
Higher expression levels of activation-induced cytidine deaminase distinguish hairy cell leukemia from hairy cell leukemia-variant and splenic marginal zone lymphoma. (20237507)
2010
18
Defining the borders of splenic marginal zone lymphoma: a multiparameter study. (20004934)
2010
19
Autoimmune hemolytic anemia as a risk factor of poor outcome in patients with splenic marginal zone lymphoma. (19343544)
2009
20
Splenectomy with chemotherapy vs surgery alone as initial treatment for splenic marginal zone lymphoma. (19705496)
2009
21
FOXP1 status in splenic marginal zone lymphoma: a fluorescence in situ hybridization and immunohistochemistry approach. (19760589)
2009
22
Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells. (18665362)
2009
23
Splenic marginal zone lymphoma: Clinical clustering of immunoglobulin heavy chain repertoires. (19250848)
2009
24
Pure red cell aplasia as first manifestation of splenic marginal zone lymphoma-successful treatment with rituximab: a case report. (19918496)
2009
25
High-grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis. (18671706)
2008
26
The prevalence of IG translocations and 7q32 deletions in splenic marginal zone lymphoma. (17989713)
2008
27
A unique case of IgD-only splenic marginal-zone lymphoma with mutated immunoglobulin genes: ontogenetic implications. (17420049)
2008
28
Composite splenic marginal zone lymphoma and classic Hodgkin lymphoma -- an unusual combination. (17988302)
2007
29
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
30
Coexistence of splenic marginal zone lymphoma with hepatocellular carcinoma: a case report. (17284308)
2007
31
Composite splenic marginal zone lymphoma and mantle cell lymphoma arising from 2 independent B-cell clones. (17134739)
2007
32
Unusual gamma heavy chain disease protein in a patient with splenic marginal-zone lymphoma. (16536920)
2006
33
Intrasinusoidal bone marrow infiltration and splenic marginal zone lymphoma: a quantitative study. (16480431)
2006
34
Splenic marginal zone lymphoma-like features in API2-MALT1 transgenic mice that are exposed to antigenic stimulation. (17145608)
2006
35
Activity of rituximab monotherapy in refractory splenic marginal zone lymphoma complicated with autoimmune hemolytic anemia. (16796783)
2006
36
Splenic marginal zone lymphoma: a prognostic model for clinical use. (16493005)
2006
37
Regression of advanced non-splenic marginal zone lymphoma after treatment of hepatitis C virus infection. (16109616)
2005
38
Meningeal lymphomatosis as the first manifestation of splenic marginal zone lymphoma. (16105762)
2005
39
Deoxycoformycin (pentostatin) in the treatment of splenic marginal zone lymphoma (SMZL) with or without villous lymphocytes. (16000129)
2005
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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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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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
29.6FCER2, CD5
39.5FCER2, NFKB2
4
Show member pathways
Immune response IL 4 signaling pathway60
Signal transduction AKT signaling60
9.5NFKB2, FCER2
5
Show member pathways
RANKL/RANK Signaling Pathway37
Apoptosis and survival APRIL and BAFF signaling60
9.5NFKB2, FCER2
6
Show member pathways
9.5NOTCH2, FCER2
7
Show member pathways
9.5NOTCH2, FCER2
89.4NOTCH2, NFKB2
99.4NOTCH2, NFKB2
109.3AICDA, MALT1
119.1MALT1, NFKB2
12
Show member pathways
Immune response Role of TLRs 3 and 4 in cell antiviral response TICAM1 specific signaling pathways60
Immune response IL 1 signaling pathway60
9.1MALT1, NFKB2
13
Show member pathways
Immune response T cell receptor signaling pathway60
Immune response CD28 signaling60
Immune response ICOS pathway in T helper cell60
9.1MALT1, NFKB2
149.1MALT1, NFKB2

Compounds for genes affiliated with Splenic Marginal Zone Lymphoma

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Sources:
44Novoseek, 61Tocris Bioscience, 24HMDB, 11DrugBank
See all sources

Compounds related to Splenic Marginal Zone Lymphoma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1calcitriol44 61 24 1112.4FCER2, GFI1
2zinc44 249.7FCER2, GFI1, AICDA
3paraffin448.7CD5, MALT1, FCER2

GO Terms for genes affiliated with Splenic Marginal Zone Lymphoma

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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
  • Proteins
  • Lysates

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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet