PCMZL
MCID: PRM129
MIFTS: 26

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 58 70
Pcmzl 58

Classifications:

Orphanet: 58  
Rare skin diseases
Rare haematological diseases


External Ids:

MESH via Orphanet 45 D018442
ICD10 via Orphanet 33 C83.0
UMLS via Orphanet 71 C1275321
Orphanet 58 ORPHA178536
UMLS 70 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 marginal zone b-cell lymphoma and b-cell lymphoma. An important gene associated with Primary Cutaneous Marginal Zone B-Cell Lymphoma is MALT1 (MALT1 Paracaspase), and among its related pathways/superpathways are T cell receptor signaling pathway and B Cell Receptor Signaling Pathway (sino). Affiliated tissues include bone marrow.

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 38)
# Related Disease Score Top Affiliating Genes
1 marginal zone b-cell lymphoma 30.8 MALT1 IRF4 BCL10
2 b-cell lymphoma 30.8 MALT1 IRF4 BCL10
3 lymphoma 30.6 MALT1 IRF4 BCL10
4 lymphoma, mucosa-associated lymphoid type 30.0 MALT1 IRF4 BCL10
5 diffuse large b-cell lymphoma 29.8 MALT1 IRF4 BCL10
6 follicular lymphoma 29.7 IRF4 BCL10
7 primary cutaneous b-cell lymphoma 10.6
8 annular erythema 10.3
9 anemia, autoimmune hemolytic 10.3
10 lymphoma, hodgkin, classic 10.3
11 nodal marginal zone lymphoma 10.3
12 igg4-related disease 10.3
13 folliculitis 10.3
14 hemolytic anemia 10.3
15 thyroiditis 10.3
16 amyloidosis 10.3
17 hypereosinophilic syndrome 10.3
18 chromosomal triplication 10.3
19 lupus erythematosus tumidus 10.3
20 primary cutaneous follicle center lymphoma 10.3
21 rosai-dorfman disease 10.3
22 posttransplant acute limbic encephalitis 10.3
23 primary cutaneous diffuse large b-cell lymphoma, leg type 10.3
24 primary cutaneous lymphoma 10.3
25 gallbladder lymphoma 9.9 MALT1 IRF4
26 leukocyte disease 9.8 MALT1 IRF4
27 orbit lymphoma 9.7 MALT1 BCL10
28 phaeohyphomycosis 9.7 MALT1 BCL10
29 alpha chain disease 9.7 MALT1 BCL10
30 plasma cell leukemia 9.7 IRF4 BCL10
31 immunodeficiency 11 9.7 MALT1 BCL10
32 orofaciodigital syndrome viii 9.7 MALT1 BCL10
33 sezary's disease 9.7 IRF4 BCL10
34 chronic mucocutaneous candidiasis 9.6 MALT1 BCL10
35 gastric lymphoma 9.5 MALT1 IRF4 BCL10
36 mantle cell lymphoma 9.4 MALT1 BCL10
37 lymphoma, non-hodgkin, familial 9.4 MALT1 IRF4 BCL10
38 combined immunodeficiency 9.4 MALT1 IRF4 BCL10

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

Search Clinical Trials , 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:

40
Bone Marrow

Publications for Primary Cutaneous Marginal Zone B-Cell Lymphoma

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

(show top 50) (show all 126)
# Title Authors PMID Year
1
Distribution of CD1a-positive cells is not different between pseudolymphomatous folliculitis and primary cutaneous marginal zone lymphoma. 61
33326629 2021
2
Dermoscopic and trichoscopic features of primary cutaneous lymphomas - systematic review. 61
33710688 2021
3
Small lymphocytic lymphoma mimicking primary cutaneous marginal zone lymphoma with colonization of germinal center follicles. 61
32506455 2021
4
Lack of clinical relevance of blood clonality in primary cutaneous marginal zone B-cell lymphoma. 61
33495148 2021
5
Primary cutaneous follicle center lymphoma in a 16-year-old girl. 61
33326136 2020
6
Darier sign in a teenager with primary cutaneous marginal zone lymphoma. 61
33336458 2020
7
How I Diagnose Primary Cutaneous Marginal Zone Lymphoma. 61
32808967 2020
8
Cutaneous B-cell lymphomas: 2021 update on diagnosis, risk-stratification, and management. 61
32815650 2020
9
Lupus Erythematosus Tumidus Mimicking Primary Cutaneous Marginal Zone B-cell Lymphoma. 61
32556349 2020
10
Primary cutaneous marginal zone lymphoma treated with doxycycline in a pediatric patient. 61
32323885 2020
11
Primary cutaneous marginal zone B-cell lymphoma with unusual manifestation and spontaneous regression. 61
32415050 2020
12
De-escalated radiotherapy for indolent primary cutaneous B-cell lymphoma. 61
31758232 2020
13
Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma. 61
32850331 2020
14
Complete resolution of primary cutaneous marginal zone B-cell lymphoma on the cheek of a 7-year-old boy with intralesional triamcinolone and tincture of time. 61
31667886 2020
15
[Localized urticarial rituximab-induced reaction in primary cutaneous marginal zone B-cell lymphoma]. 61
31635943 2020
16
Primary Cutaneous B-Cell Lymphomas: An Update. 61
32528871 2020
17
Prevalence of atopy and staphylococcal superantigen-specific immunoglobulin E (IgE) antibodies and total serum IgE in primary cutaneous T- and B-cell lymphoma. 61
31587349 2019
18
Class-switched Primary Cutaneous Marginal Zone Lymphomas Are Frequently IgG4-positive and Have Features Distinct From IgM-positive Cases. 61
31464711 2019
19
Large Cells With CD30 Expression and Hodgkin-like Features in Primary Cutaneous Marginal Zone B-Cell Lymphoma: A Study of 13 Cases. 61
31145160 2019
20
Clinical presentation, therapeutic approach and outcome of primary cutaneous marginal zone B-cell lymphoma presenting as AL amyloidoma of the skin. 61
30801660 2019
21
AL-Amyloidoma of the Skin-A Rare Manifestation of Primary Cutaneous Marginal Zone Lymphoma. 61
30839349 2019
22
Primary cutaneous marginal zone B-cell lymphoma of vulva in a pregnant woman. 61
30665994 2019
23
Radiation therapy in indolent primary cutaneous B cell lymphoma: a single institute experience. 61
30094511 2018
24
Paraproteinaemia in Primary Cutaneous Marginal Zone Lymphoma. 61
30085323 2018
25
[Primary cutaneous marginal zone B cell lymphoma: a clinicopathologic study of 11 cases]. 61
30423612 2018
26
Cutaneous B-cell lymphomas: 2019 update on diagnosis, risk stratification, and management. 61
30039522 2018
27
Outcomes of radiation therapy of indolent cutaneous B-cell lymphomas and literature review. 61
29578630 2018
28
IgG4 Expression in Primary Cutaneous Marginal Zone Lymphoma: A Multicenter Study. 61
28151793 2018
29
Panel Sequencing Shows Recurrent Genetic FAS Alterations in Primary Cutaneous Marginal Zone Lymphoma. 61
29481902 2018
30
Frequent immunoglobulin G4 expression in a common variant of primary cutaneous marginal zone B-cell lymphoma. 61
28980311 2018
31
Image Gallery: Metachronic occurrence of primary cutaneous marginal zone B-cell lymphoma and primary cutaneous Rosai-Dorfman disease in the same patient. 61
29313941 2017
32
Diagnostic value of STMN1, LMO2, HGAL, AID expression and 1p36 chromosomal abnormalities in primary cutaneous B cell lymphomas. 61
28594133 2017
33
Paediatric primary cutaneous marginal zone B-cell lymphoma: does it differ from its adult counterpart? 61
27501236 2017
34
B-Zell-Lymphome der Haut - Pathogenese, Diagnostik und Therapie. 61
27992139 2016
35
Cutaneous B-cell lymphomas - pathogenesis, diagnostic workup, and therapy. 61
27992127 2016
36
Primary cutaneous marginal-zone lymphoma. 61
28329553 2016
37
Primary Cutaneous B-Cell Lymphoma in Young Monozygotic Twins: A Case Report. 61
27358311 2016
38
Cutaneous B-cell lymphomas: 2016 update on diagnosis, risk-stratification, and management. 61
27650702 2016
39
Primary Cutaneous Marginal Zone B-Cell Lymphoma Presenting as a Small Mass on Temple Area. 61
27152562 2016
40
Primary Cutaneous B-Cell Lymphoma: Management and Patterns of Recurrence at the Multimodality Cutaneous Lymphoma Clinic of The Ohio State University. 61
26306900 2015
41
Frequency of primary cutaneous lymphoma variants in Austria: retrospective data from a dermatology referral centre between 2006 and 2013. 61
25600184 2015
42
A case report of primary cutaneous marginal zone lymphoma treated with intralesional steroids. 61
26437162 2015
43
Primary cutaneous marginal IgG4 lymphoma and Rosai-Dorfman's disease coexisting in several lesions of the same patient. 61
25590288 2015
44
Clinical features and treatment outcomes of primary cutaneous B-cell lymphoma: a single-center analysis in South Korea. 61
25552248 2015
45
Cutaneous B-cell lymphomas: 2015 update on diagnosis, risk-stratification, and management. 61
25535037 2015
46
PD-1, S-100 and CD1a expression in pseudolymphomatous folliculitis, primary cutaneous marginal zone B-cell lymphoma (MALT lymphoma) and cutaneous lymphoid hyperplasia. 61
25384543 2015
47
Rituximab plus liposomal pegylated doxorubicin in the treatment of primary cutaneous B-cell lymphomas. 61
24635751 2014
48
Primary cutaneous marginal zone lymphoma in children: a report of 3 cases and review of the literature. 61
24698939 2014
49
Rituximab in the treatment of primary cutaneous B-cell lymphoma: a review. 61
23540593 2014
50
High incidence of gastrointestinal tract disorders and autoimmunity in primary cutaneous marginal zone B-cell lymphomas. 61
24500411 2014

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
11.97 MALT1 IRF4 BCL10
2
Show member pathways
11.96 MALT1 BCL10
3
Show member pathways
11.94 MALT1 BCL10
4 11.9 MALT1 BCL10
5
Show member pathways
11.87 MALT1 BCL10
6
Show member pathways
11.83 MALT1 BCL10
7 11.72 MALT1 BCL10
8 11.69 MALT1 BCL10
9 11.65 MALT1 IRF4 BCL10
10
Show member pathways
11.64 MALT1 BCL10
11
Show member pathways
11.4 MALT1 BCL10
12 11.34 MALT1 BCL10
13
Show member pathways
11.19 MALT1 IRF4 BCL10
14 10.52 MALT1 BCL10

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 8.96 MALT1 BCL10
2 polkadots GO:0002096 8.62 MALT1 BCL10

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

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 regulation of apoptotic process GO:0042981 9.54 MALT1 BCL10
2 positive regulation of I-kappaB kinase/NF-kappaB signaling GO:0043123 9.52 MALT1 BCL10
3 T cell receptor signaling pathway GO:0050852 9.51 MALT1 BCL10
4 Fc-epsilon receptor signaling pathway GO:0038095 9.49 MALT1 BCL10
5 cellular response to lipopolysaccharide GO:0071222 9.48 MALT1 BCL10
6 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.46 MALT1 BCL10
7 stimulatory C-type lectin receptor signaling pathway GO:0002223 9.43 MALT1 BCL10
8 positive regulation of protein ubiquitination GO:0031398 9.4 MALT1 BCL10
9 positive regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0043280 9.37 MALT1 BCL10
10 lipopolysaccharide-mediated signaling pathway GO:0031663 9.32 MALT1 BCL10
11 positive regulation of interleukin-2 production GO:0032743 9.26 MALT1 IRF4
12 positive regulation of T cell activation GO:0050870 9.16 MALT1 BCL10
13 regulation of T cell receptor signaling pathway GO:0050856 8.96 MALT1 BCL10
14 response to fungus GO:0009620 8.62 MALT1 BCL10

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 IRF4 BCL10
2 protease binding GO:0002020 9.16 MALT1 BCL10
3 protein self-association GO:0043621 8.96 MALT1 BCL10
4 kinase activator activity GO:0019209 8.62 MALT1 BCL10

Sources for Primary Cutaneous Marginal Zone B-Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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