SMZL
MCID: SPL004
MIFTS: 48

Splenic Marginal Zone Lymphoma (SMZL) malady

Blood, Cancer, Immune categories

Summaries for Splenic Marginal Zone Lymphoma

Sources:
8Disease Ontology, 64Wikipedia, 33MalaCards
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Wikipedia:64 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 burkitt's lymphoma. 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, bone marrow and whole blood, 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

Sources:
8Disease Ontology, 10DISEASES, 49Orphanet, 31LifeMap Discovery™, 61UMLS, 26ICD10 via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
splenic marginal zone lymphoma:
Age of onset: Adulthood


Aliases & Descriptions:

splenic marginal zone lymphoma 8 10 49 31
splenic marginal zone b-cell lymphoma 61
smzl 49


External Ids:

Disease Ontology8 DOID:0050750
ICD10 via Orphanet26 C83.0

Related Diseases for Splenic Marginal Zone Lymphoma

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Splenic Marginal Zone Lymphoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 60)
idRelated DiseaseScoreTop Affiliating Genes
1hepatitis c30.9AICDA
2burkitt's lymphoma30.5BCL6, GFI1, CD5, BTK, MALT1, AICDA
3bone lymphoma10.7
4macroglobulinemia10.7
5lymphoma, somatic10.7
6hepatitis a10.6
7autoimmune hemolytic anemia10.6
8mantle cell lymphoma10.6
9splenic disease10.6
10hepatitis c virus10.6
11composite lymphoma10.5
12liver lymphoma10.4
13t-cell leukemia10.4
14leukemia/lymphoma, t-cell10.4
15membranous glomerulonephritis10.3
16antiphospholipid syndrome10.3
17evans' syndrome10.3
18hepatitis e10.3
19visceral leishmaniasis10.3
20gastrointestinal lymphoma10.3
21gamma heavy chain disease10.3
22bladder lymphoma10.3
23hepatitis d10.3
24hypersplenism10.3
25cerebral lymphoma10.3
26pediatric lymphoma10.3
27anaplastic large cell lymphoma10.3
28systemic mastocytosis10.3
29catastrophic antiphospholipid syndrome10.3
30chromosome 7q deletion10.3
31waldenstrom macroglobulinemia10.3
32mastocytosis10.3
33splenomegaly10.3
34lymphoma, diffuse large cell10.3
35primary bone lymphoma10.3
36refractory anemia10.3
37colorectal cancer10.0GLI1
38myeloma10.0BCL6
39multiple myeloma10.0SDC1
40stomach cancer10.0GLI1
41central nervous system lymphoma10.0BCL6
42immunodeficiency with hyper igm type 110.0BTK, AICDA
43glioblastoma multiforme10.0GLI1
44common variable immune deficiency10.0BTK, AICDA
45lymphoma, malt, somatic10.0BCL6, CD5, MALT1
46pallister-hall syndrome10.0GLI1, SHH
47vacterl association10.0SHH
48plasmacytoma10.0AICDA, BTK, BCL6
49follicular lymphoma10.0AICDA, CD5, BCL6
50hepatocellular carcinoma10.0JUND, AICDA, SOX4

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

Drugs & Therapeutics for Splenic Marginal Zone Lymphoma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 31LifeMap Discovery™
See all sources

Approved drugs:

Search CenterWatch for Splenic Marginal Zone Lymphoma

Drug clinical trials:

Search ClinicalTrials for Splenic Marginal Zone Lymphoma

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

Anatomical Context for Splenic Marginal Zone Lymphoma

Sources:
14FMA, 33MalaCards
See all sources

MalaCards organs/tissues related to Splenic Marginal Zone Lymphoma:

33
Bone marrow, Whole blood, Lymph node, Spleen, Brain, Liver, T cells, B cells

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

Sources:
37MGI, 28inGenious Targeting Laboratory
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Publications for Splenic Marginal Zone Lymphoma

Sources:
51PubMed
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Articles related to Splenic Marginal Zone Lymphoma:

(show top 50)    (show all 200)
idTitleAuthorsYear
1
Pediatric Presentation of Splenic Marginal Zone Lymphoma on FDG PET/CT Scan. (23640226)
2014
2
A peculiar case of splenic marginal zone lymphoma and review of literature. (24076596)
2013
3
Clinical characteristics and outcome in dogs with splenic marginal zone lymphoma. (23734665)
2013
4
Laparoscopic splenectomy for treatment of splenic marginal zone lymphoma. (23840125)
2013
5
Risk stratification for Splenic Marginal Zone Lymphoma based on haemoglobin concentration, platelet count, high lactate dehydrogenase level and extrahilar lymphadenopathy: development and validation on 593 cases. (22924582)
2012
6
Survival outcomes with and without splenectomy in splenic marginal zone lymphoma. (22887176)
2012
7
An integrated genomic and expression analysis of 7q deletion in splenic marginal zone lymphoma. (23028731)
2012
8
Selection of antigen receptors in splenic marginal-zone lymphoma: further support from the analysis of the immunoglobulin light-chain gene repertoire. (22858907)
2012
9
High-throughput sequencing analysis of the chromosome 7q32 deletion reveals IRF5 as a potential tumour suppressor in splenic marginal-zone lymphoma. (22816737)
2012
10
The coding genome of splenic marginal zone lymphoma: activation of NOTCH2 and other pathways regulating marginal zone development. (22891273)
2012
11
De novo large B-cell lymphoma in a parenchymal brain lesion: evidence of clonal evolution from splenic marginal zone lymphoma. (21205746)
2011
12
Alteration of BIRC3 and multiple other NF-I_B pathway genes in splenic marginal zone lymphoma. (21881048)
2011
13
Splenic marginal zone lymphoma: hydra with many heads? (20378575)
2010
14
Splenectomy with chemotherapy vs surgery alone as initial treatment for splenic marginal zone lymphoma. (19705496)
2009
15
Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells. (18665362)
2009
16
Splenic marginal zone B-cell lymphoma in a HIV-positive patient: a case report. (18709501)
2009
17
Mutation analysis of IgVH genes in splenic marginal zone lymphomas: correlation with clinical characteristics and outcome. (19443409)
2009
18
Splenic marginal zone lymphoma--a clinicopathological study in a series of 16 patients. (18854089)
2008
19
Splenic marginal zone lymphoma: transformation to diffuse large B-cell lymphoma with isolated cerebral manifestation. (18802164)
2008
20
Re: Rituximab monotherapy is highly effective in splenic marginal zone lymphoma. (18271062)
2008
21
Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria. (18094718)
2008
22
Successful treatment of catastrophic antiphospholipid antibody syndrome (CAPS) associated with splenic marginal-zone lymphoma with low-molecular weight heparin, rituximab and bendamustine. (18480659)
2008
23
Composite splenic marginal zone lymphoma and mantle cell lymphoma arising from 2 independent B-cell clones. (17134739)
2007
24
Hepatitis C virus and splenic marginal zone lymphoma with villous lymphocytes: away from conventional therapy. (17852855)
2007
25
Acquired C1-esterase inhibitor deficiency and positive lupus anticoagulant accompanied by splenic marginal zone B-cell lymphoma. (17888223)
2007
26
Splenic marginal-zone lymphoma: one or more entities? A histologic, immunohistochemical, and molecular study of 42 cases. (17325486)
2007
27
Unusual gamma heavy chain disease protein in a patient with splenic marginal-zone lymphoma. (16536920)
2006
28
Intrasinusoidal bone marrow infiltration and splenic marginal zone lymphoma: a quantitative study. (16480431)
2006
29
Splenic marginal zone lymphoma: a prognostic model for clinical use. (16493005)
2006
30
Translocation t(9;14)(p13;q32) in cases of splenic marginal zone lymphoma. (16956840)
2006
31
Immunophenotypic profile and role of adhesion molecules in splenic marginal zone lymphoma with bone marrow involvement. (16321827)
2006
32
Meningeal lymphomatosis as the first manifestation of splenic marginal zone lymphoma. (16105762)
2005
33
Splenic marginal zone lymphoma: 7q abnormalities. (15998972)
2005
34
Images of interest. Gastrointestinal: white small bowel, macroglobulinemia and splenic marginal-zone lymphoma. (16174087)
2005
35
Recurrent genomic imbalances in B-cell splenic marginal-zone lymphoma revealed by comparative genomic hybridization. (15642391)
2005
36
Uncommon cytogenetic findings in a case of splenic marginal zone lymphoma with aggressive clinical course. (14734224)
2004
37
Successful rituximab therapy for hemolytic anemia associated with relapsed splenic marginal zone lymphoma with leukemic phase. (14959867)
2003
38
Small solitary littoral cell angioma associated with splenic marginal zone lymphoma and villous lymphocyte leukaemia in a patient with hepatitis C infection. (12405918)
2002
39
Splenic small B-cell lymphoma with predominant red pulp involvement: a diffuse variant of splenic marginal zone lymphoma? (11903595)
2002
40
Terminal plasmocytoid differentiation of malignant B cells induced by autotumor-reactive CD4(+) T cells in one case of splenic marginal zone B-cell lymphoma. (11783434)
2002
41
Analysis of the IgV(H) somatic mutations in splenic marginal zone lymphoma defines a group of unmutated cases with frequent 7q deletion and adverse clinical course. (11830479)
2002
42
No V(H) somatic hypermutation was detected in B-cells of a patient with macroglobulinemia due to splenic marginal zone lymphoma. (12512840)
2002
43
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
44
7q31-32 allelic loss is a frequent finding in splenic marginal zone lymphoma. (10329610)
1999
45
Primary nodal marginal zone lymphomas of splenic and MALT type. (9888704)
1999
46
Splenic involvement by blastic mantle cell lymphoma (large cell/anaplastic variant) mimicking splenic marginal zone lymphoma. (10589081)
1999
47
A marginal zone pattern may be found in different varieties of non-Hodgkin's lymphoma: the morphology and immunohistology of splenic involvement by B-cell lymphomas simulating splenic marginal zone lymphoma. (9777389)
1998
48
Splenic marginal zone lymphoma. (8695824)
1996
49
Splenic marginal zone cell lymphoma: report of an indolent variant without massive splenomegaly presumably representing an early phase of the disease. (7821915)
1995
50
Frequent p53 gene involvement in splenic B-cell leukemia/lymphomas of possible marginal zone origin. (8018922)
1994

Genetic Variations for Splenic Marginal Zone Lymphoma

Expression for genes affiliated with Splenic Marginal Zone Lymphoma

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
4Cell Signaling Technology, 53R&D Systems, 12EMD Millipore, 30KEGG, 38NCBI BioSystems Database
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Compounds for genes affiliated with Splenic Marginal Zone Lymphoma

Sources:
60Tocris Bioscience, 45Novoseek
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Compounds related to Splenic Marginal Zone Lymphoma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1sant-16010.3GLI1, SHH
220(s)-hydroxycholesterol6010.3SHH, GLI1
3cyclopamine45 6011.3GLI1, SHH
4ciliobrevin a6010.2SHH, GLI1
5ay 9944 dihydrochloride6010.0GLI1, SHH

GO Terms for genes affiliated with Splenic Marginal Zone Lymphoma

Sources:
16Gene Ontology
See all sources

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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Splenic Marginal Zone Lymphoma

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet