PCMZL
MCID: PRM129
MIFTS: 32

Primary Cutaneous Marginal Zone B-Cell Lymphoma (PCMZL)

Categories: Blood diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Primary Cutaneous Marginal Zone B-Cell Lymphoma

MalaCards integrated aliases for Primary Cutaneous Marginal Zone B-Cell Lymphoma:

Name: Primary Cutaneous Marginal Zone B-Cell Lymphoma 59 73
Pcmzl 59

Classifications:



External Ids:

Orphanet 59 ORPHA178536
MESH via Orphanet 45 D018442
UMLS via Orphanet 74 C1275321
ICD10 via Orphanet 34 C83.0
UMLS 73 C1275321

Summaries for Primary Cutaneous Marginal Zone B-Cell Lymphoma

MalaCards based summary : Primary Cutaneous Marginal Zone B-Cell Lymphoma, also known as pcmzl, is related to lymphoma and b-cell lymphomas. An important gene associated with Primary Cutaneous Marginal Zone B-Cell Lymphoma is MALT1 (MALT1 Paracaspase), and among its related pathways/superpathways are Immune response NFAT in immune response and CLEC7A (Dectin-1) signaling. The drugs Anti-Infective Agents and Interferon-gamma have been mentioned in the context of this disorder. Affiliated tissues include b cells, bone and skin.

Related Diseases for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Diseases related to Primary Cutaneous Marginal Zone B-Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 12)
# Related Disease Score Top Affiliating Genes
1 lymphoma 31.2 BCL10 MALT1
2 b-cell lymphomas 30.9 BCL10 IRF4 MALT1
3 marginal zone b-cell lymphoma 30.9 BCL10 IRF4 MALT1
4 lymphoma, mucosa-associated lymphoid type 29.7 BCL10 IRF4 MALT1
5 folliculitis 10.2
6 rosai-dorfman disease 10.2
7 primary effusion lymphoma 9.9 IRF4 MALT1
8 leukocyte disease 9.9 IRF4 MALT1
9 orofaciodigital syndrome viii 9.8 BCL10 MALT1
10 diffuse large b-cell lymphoma 9.8 IRF4 MALT1
11 follicular lymphoma 9.8 BCL10 IRF4
12 lymphoma, non-hodgkin, familial 9.6 BCL10 IRF4 MALT1

Graphical network of the top 20 diseases related to Primary Cutaneous Marginal Zone B-Cell Lymphoma:



Diseases related to Primary Cutaneous Marginal Zone B-Cell Lymphoma

Symptoms & Phenotypes for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Drugs & Therapeutics for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Drugs for Primary Cutaneous Marginal Zone B-Cell Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Anti-Infective Agents Phase 2
2 Interferon-gamma Phase 2
3 interferons Phase 2
4 Antiviral Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Study to Evaluate the Safety and Efficacy of Adeno-IFN Gamma in Cutaneous B-cell Lymphoma Completed NCT00394693 Phase 2

Search NIH Clinical Center for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Genetic Tests for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Anatomical Context for Primary Cutaneous Marginal Zone B-Cell Lymphoma

MalaCards organs/tissues related to Primary Cutaneous Marginal Zone B-Cell Lymphoma:

41
B Cells, Bone, Skin, Bone Marrow

Publications for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Articles related to Primary Cutaneous Marginal Zone B-Cell Lymphoma:

(show all 39)
# Title Authors Year
1
Image Gallery: Metachronic occurrence of primary cutaneous marginal zone B-cell lymphoma and primary cutaneous Rosai-Dorfman disease in the same patient. ( 29313941 )
2017
2
Frequent immunoglobulin G4 expression in a common variant of primary cutaneous marginal zone B-cell lymphoma. ( 28980311 )
2017
3
Primary Cutaneous Marginal Zone B-Cell Lymphoma Presenting as a Small Mass on Temple Area. ( 27152562 )
2016
4
Paediatric primary cutaneous marginal zone B-cell lymphoma: Does it differ from the adult counterpart? ( 27501236 )
2016
5
Primary Cutaneous Marginal Zone B Cell Lymphoma of the Eyelid Skin: Diagnostic Clues and Distinction from Other Ocular Adnexal Disease. ( 26545575 )
2015
6
PD-1, S-100, and CD1a Expression in Pseudolymphomatous Folliculitis, Primary Cutaneous Marginal Zone B-cell Lymphoma (MALT lymphoma), and Cutaneous Lymphoid Hyperplasia. ( 25384543 )
2014
7
Primary cutaneous marginal zone B-cell lymphoma: response to treatment and disease-free survival in a series of 137 patients. ( 23796549 )
2013
8
Is bone marrow biopsy always indicated in patients with primary cutaneous marginal zone B-cell lymphoma? ( 23954046 )
2013
9
Primary cutaneous marginal zone lymphoma (PCMZL) presenting with heterochronous biclonal lesions. ( 24152529 )
2013
10
Bi-clonal, multifocal primary cutaneous marginal zone B-cell lymphoma: report of a case and review of the literature. ( 22809282 )
2012
11
Letter: Primary cutaneous marginal zone B cell lymphoma of the face: a challenging diagnosis. ( 22398233 )
2012
12
Recurrent localized primary cutaneous marginal-zone B cell lymphoma. ( 22031653 )
2011
13
Primary cutaneous marginal zone B cell lymphoma in monozygotic twins. ( 22707632 )
2011
14
Primary cutaneous marginal zone B-cell lymphoma with anetoderma: spontaneous involution plus de novo clonal expansion. ( 21219395 )
2011
15
Pediatric primary cutaneous marginal zone B-cell lymphoma treated with intralesional rituximab. ( 20542839 )
2010
16
CD123-positive plasmacytoid dendritic cells in primary cutaneous marginal zone B-cell lymphoma: A crucial role and a new lymphoma paradigm. ( 19940749 )
2010
17
Primary cutaneous marginal zone B-cell lymphoma with amyloid deposition: report of two cases with review of literature. ( 20507739 )
2010
18
Red nose: primary cutaneous marginal zone B-cell lymphoma. ( 19945162 )
2010
19
Primary cutaneous marginal zone B-cell lymphoma: An atypical case. ( 21199631 )
2010
20
CD123-positive plasmacytoid dendritic cells in primary cutaneous marginal zone B-cell lymphoma: diagnostic and pathogenetic implications. ( 19718787 )
2009
21
Primary cutaneous marginal zone B-cell lymphoma: clinical and histological aspects. ( 19771768 )
2009
22
Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathological study of cases from Asia, Germany, and the United States. ( 18820662 )
2008
23
Primary cutaneous marginal zone B-cell lymphoma may exhibit both the t(14;18)(q32;q21) IGH/BCL2 and the t(14;18)(q32;q21) IGH/MALT1 translocation: an indicator for clonal transformation towards higher-grade B-cell lymphoma? ( 17519619 )
2007
24
Primary cutaneous marginal zone B-cell lymphoma infiltrating the frontal bone. ( 17324846 )
2007
25
Borrelia burgdorferi-associated primary cutaneous marginal-zone B-cell lymphoma: a case report. ( 17823520 )
2007
26
Primary cutaneous marginal zone B-cell lymphoma in a patient with chronic lymphocytic leukaemia. ( 17697078 )
2007
27
Topical mechlorethamine and clobetasol in multifocal primary cutaneous marginal zone-B cell lymphoma. ( 16704661 )
2006
28
Primary cutaneous marginal zone B-cell lymphoma of MALT type in a child. ( 17163787 )
2006
29
Intra-lesional low-dose interferon alpha2a therapy for primary cutaneous marginal zone B-cell lymphoma. ( 16753871 )
2006
30
Aberrant nuclear BCL10 expression and lack of t(11;18)(q21;q21) in primary cutaneous marginal zone B-cell lymphoma. ( 16784987 )
2006
31
Primary cutaneous marginal zone B-cell lymphoma (PCMZL-MALT). ( 16870546 )
2006
32
Primary cutaneous marginal zone B-cell lymphoma. ( 16830956 )
2006
33
Primary cutaneous marginal zone B-cell lymphoma: clinical and therapeutic features in 50 cases. ( 16172311 )
2005
34
Primary cutaneous marginal zone B-cell lymphoma. ( 16280304 )
2005
35
Absence of MALT1 translocations in primary cutaneous marginal zone B-cell lymphoma. ( 15377491 )
2004
36
Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathologic study of 24 asian cases. ( 12883238 )
2003
37
Primary cutaneous marginal zone B-cell lymphoma: a clinical, histopathological, immunophenotypic and molecular genetic study of 22 cases. ( 12452864 )
2002
38
Primary cutaneous marginal zone B-cell lymphoma: a report of 9 cases. ( 10426886 )
1999
39
Primary cutaneous marginal zone B-cell lymphoma: a recently described entity of low-grade malignant cutaneous B-cell lymphoma. ( 9351568 )
1997

Variations for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Expression for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Search GEO for disease gene expression data for Primary Cutaneous Marginal Zone B-Cell Lymphoma.

Pathways for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Pathways related to Primary Cutaneous Marginal Zone B-Cell Lymphoma according to GeneCards Suite gene sharing:

(show all 14)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.09 BCL10 MALT1
2
Show member pathways
11.94 BCL10 MALT1
3
Show member pathways
11.89 BCL10 MALT1
4 11.85 BCL10 IRF4 MALT1
5
Show member pathways
11.84 BCL10 MALT1
6 11.72 BCL10 MALT1
7 11.69 BCL10 MALT1
8
Show member pathways
11.64 BCL10 MALT1
9
Show member pathways
11.54 BCL10 IRF4 MALT1
10
Show member pathways
11.4 BCL10 MALT1
11 11.3 BCL10 MALT1
12
Show member pathways
11.17 BCL10 IRF4 MALT1
13 11.08 BCL10 MALT1
14 10.62 BCL10 MALT1

GO Terms for Primary Cutaneous Marginal Zone B-Cell Lymphoma

Cellular components related to Primary Cutaneous Marginal Zone B-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 CBM complex GO:0032449 8.62 BCL10 MALT1

Biological processes related to Primary Cutaneous Marginal Zone B-Cell Lymphoma according to GeneCards Suite gene sharing:

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 regulation of apoptotic process GO:0042981 9.52 BCL10 MALT1
2 positive regulation of I-kappaB kinase/NF-kappaB signaling GO:0043123 9.51 BCL10 MALT1
3 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.49 BCL10 MALT1
4 T cell receptor signaling pathway GO:0050852 9.48 BCL10 MALT1
5 Fc-epsilon receptor signaling pathway GO:0038095 9.46 BCL10 MALT1
6 protein complex oligomerization GO:0051259 9.43 BCL10 MALT1
7 positive regulation of protein ubiquitination GO:0031398 9.4 BCL10 MALT1
8 stimulatory C-type lectin receptor signaling pathway GO:0002223 9.37 BCL10 MALT1
9 lipopolysaccharide-mediated signaling pathway GO:0031663 9.32 BCL10 MALT1
10 positive regulation of T cell activation GO:0050870 9.26 BCL10 MALT1
11 response to molecule of bacterial origin GO:0002237 9.16 BCL10 MALT1
12 response to fungus GO:0009620 8.96 BCL10 MALT1
13 regulation of T cell receptor signaling pathway GO:0050856 8.62 BCL10 MALT1

Molecular functions related to Primary Cutaneous Marginal Zone B-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transcription factor binding GO:0008134 9.26 BCL10 IRF4
2 protease binding GO:0002020 9.16 BCL10 MALT1
3 protein self-association GO:0043621 8.96 BCL10 MALT1
4 kinase activator activity GO:0019209 8.62 BCL10 MALT1

Sources for Primary Cutaneous Marginal Zone B-Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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