SDRPL
MCID: SPL060
MIFTS: 30

Splenic Diffuse Red Pulp Small B-Cell Lymphoma (SDRPL)

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

MalaCards integrated aliases for Splenic Diffuse Red Pulp Small B-Cell Lymphoma:

Name: Splenic Diffuse Red Pulp Small B-Cell Lymphoma 58 71
Splenic Diffuse Red Pulp Lymphoma 58
Sdrpl 58

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 via Orphanet 33 C83.0
Orphanet 58 ORPHA300869
UMLS 71 C2699508

Summaries for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

MalaCards based summary : Splenic Diffuse Red Pulp Small B-Cell Lymphoma, also known as splenic diffuse red pulp lymphoma, is related to b-cell lymphoma and hairy cell leukemia. An important gene associated with Splenic Diffuse Red Pulp Small B-Cell Lymphoma is BCOR (BCL6 Corepressor), and among its related pathways/superpathways are GPCR Pathway and PI3K-Akt signaling pathway. Affiliated tissues include b cells, bone and t cells, and related phenotypes are cardiovascular system and embryo

Related Diseases for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Diseases related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 56)
# Related Disease Score Top Affiliating Genes
1 b-cell lymphoma 30.8 TP53 CCND3 CCNA2
2 hairy cell leukemia 10.6
3 marginal zone b-cell lymphoma 10.5
4 splenic marginal zone lymphoma 10.5
5 lymphoma 10.5
6 splenomegaly 10.5
7 triiodothyronine receptor auxiliary protein 10.3
8 prolymphocytic leukemia 10.3
9 thrombocytopenia 10.3
10 b cell prolymphocytic leukemia 10.3
11 posttransplant acute limbic encephalitis 10.3
12 parameningeal embryonal rhabdomyosarcoma 10.1 TP53 BCOR
13 bone osteosarcoma 10.1 TP53 BCOR
14 apocrine adenosis of breast 10.1 TP53 CCNA2
15 thyroid lymphoma 10.1 TP53 CCNA2
16 monoclonal gammopathy of uncertain significance 10.0 TP53 CCND3
17 rhabdomyosarcoma 2 9.9 TP53 BCOR
18 skin squamous cell carcinoma 9.8 TP53 MAP2K1
19 invasive bladder transitional cell carcinoma 9.8 TP53 NOTCH1
20 bone squamous cell carcinoma 9.8 TP53 NOTCH1
21 uterine adnexa cancer 9.8 TP53 NOTCH1
22 richter's syndrome 9.8 TP53 NOTCH1
23 tonsil cancer 9.7 TP53 NOTCH1
24 lung adenoma 9.7 TP53 NOTCH1
25 oropharynx cancer 9.7 TP53 NOTCH1
26 pre-malignant neoplasm 9.7 TP53 NOTCH1
27 basal cell nevus syndrome 9.7 TP53 NOTCH1
28 retinal cancer 9.7 TP53 CCND3 CCNA2
29 ewing sarcoma 9.7 TP53 CCND3 BCOR
30 lymphoblastic lymphoma 9.7 TP53 NOTCH1
31 breast adenocarcinoma 9.6 TP53 CCND3 CCNA2
32 brain glioma 9.6 TP53 NOTCH1
33 burkitt lymphoma 9.6 TP53 CCND3 CCNA2
34 adenocarcinoma 9.6 TP53 MAP2K1 CCNA2
35 bile duct adenocarcinoma 9.6 TP53 NOTCH1
36 myeloid leukemia 9.5 TP53 MAP2K1 CCNA2
37 tongue disease 9.5 TP53 NOTCH1
38 infratentorial cancer 9.5 TP53 NOTCH1
39 adenoid cystic carcinoma 9.4 TP53 NOTCH1 BCOR
40 mantle cell lymphoma 9.4 TP53 NOTCH1 CCND3
41 aplastic anemia 9.3 TP53 NOTCH1 CCND3
42 hematologic cancer 9.3 TP53 NOTCH1 CCNA2
43 myeloma, multiple 9.3 TP53 NOTCH1 CCND3
44 lymphoma, non-hodgkin, familial 9.2 TP53 NOTCH1 CCND3
45 t-cell leukemia 9.2 TP53 NOTCH1 CCND3
46 retinoblastoma 9.1 TP53 CCND3 CCNA2
47 squamous cell carcinoma, head and neck 9.0 TP53 NOTCH1 CCND3 CCNA2
48 leukemia, chronic lymphocytic 9.0 TP53 NOTCH1 CCND3 CCNA2
49 acute promyelocytic leukemia 9.0 NOTCH1 MAP2K1 BCOR
50 medulloblastoma 9.0 TP53 NOTCH1 MAP2K1 BCOR

Graphical network of the top 20 diseases related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma:



Diseases related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Symptoms & Phenotypes for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

MGI Mouse Phenotypes related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.65 BCOR CCND3 MAP2K1 NOTCH1 TP53
2 embryo MP:0005380 9.55 BCOR CCNA2 MAP2K1 NOTCH1 TP53
3 mortality/aging MP:0010768 9.43 BCOR CCNA2 CCND3 MAP2K1 NOTCH1 TP53
4 neoplasm MP:0002006 8.92 CCND3 MAP2K1 NOTCH1 TP53

Drugs & Therapeutics for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Search Clinical Trials , NIH Clinical Center for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Genetic Tests for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Anatomical Context for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

MalaCards organs/tissues related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma:

40
B Cells, Bone, T Cells, Breast, Myeloid, Spleen, Bone Marrow

Publications for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Articles related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma:

(show all 32)
# Title Authors PMID Year
1
Hairy cell leukemia: 2020 update on diagnosis, risk stratification, and treatment. 61
31591741 2019
2
Clinicopathological features of splenic tumours of lymphoid tissue. 61
30087035 2018
3
Development of B-cell prolymphocytic leukemia in a patient with splenic diffuse red pulp small B-cell lymphoma. 61
29199492 2018
4
New generation sequencing of targeted genes in the classical and the variant form of hairy cell leukemia highlights mutations in epigenetic regulation genes. 61
29989027 2018
5
Toll-like receptor expression and function differ between splenic marginal zone B cell lymphoma and splenic diffuse red pulp B cell lymphoma. 61
29805758 2018
6
A rare case of splenic diffuse red pulp small B-cell lymphoma (SDRPL): a review of the literature on primary splenic lymphoma with hairy cells. 61
29662866 2018
7
[Splenic diffuse red pulp small B-cell lymphoma diagnosed by splenectomy initially mimicking hairy cell leukemia-Japanese variant]. 61
29618685 2018
8
Hairy cell leukemia 2018: Update on diagnosis, risk-stratification, and treatment. 61
29110361 2017
9
[Tumors of lymphoid and hematopoietic tissue of spleen: a clinicopathologic analysis of 53 cases]. 61
29136691 2017
10
Exome sequencing identifies recurrent BCOR alterations and the absence of KLF2, TNFAIP3 and MYD88 mutations in splenic diffuse red pulp small B-cell lymphoma. 61
28751561 2017
11
Splenic diffuse red pulp lymphoma has a distinct pattern of somatic mutations amongst B-cell malignancies. 61
27347751 2017
12
Cyclin A2 as a potential differential marker of splenic diffuse red pulp small B-cell lymphoma: a report of the first case. 61
27761608 2017
13
Splenic diffuse red pulp small B-cell lymphoma displays increased expression of cyclin D3 and recurrent CCND3 mutations. 61
28069605 2017
14
A Single-center Experience in Splenic Diffuse Red Pulp Lymphoma Diagnosis. 61
27131623 2016
15
Splenic diffuse red-pulp small B-cell lymphoma associated with hepatitis B virus: a report of two cases. 61
27581334 2016
16
CD27-positive hairy cell leukemia-Japanese variant. 61
26868143 2016
17
NOTCH1, TP53, and MAP2K1 Mutations in Splenic Diffuse Red Pulp Small B-cell Lymphoma Are Associated With Progressive Disease. 61
26426381 2016
18
Bone marrow and splenic histology in hairy cell leukaemia. 61
26614898 2015
19
New insights in the management of patients with hairy cell leukemia. 61
26154707 2015
20
[Chronic B-cell lymphoproliferative disorders with hairy cells]. 61
25858127 2015
21
A comprehensive immunophenotypic marker analysis of hairy cell leukemia in paraffin-embedded bone marrow trephine biopsies--a tissue microarray study. 61
24903677 2015
22
Monoclonal antibody HBME-1 reacts with a minor subset of B cells with villous surface and can be useful in the diagnosis of hairy cell leukemia and other indolent lymphoproliferations of villous B lymphocytes. 61
24092261 2013
23
Annexin A1 expression in a splenic diffuse red pulp small B-cell lymphoma: report of the first case. 61
23795986 2013
24
Splenic B-cell lymphomas with more than 55% prolymphocytes in blood: evidence for prolymphocytoid transformation. 61
22520947 2012
25
Bone marrow histopathology in the diagnostic evaluation of splenic marginal-zone and splenic diffuse red pulp small B-cell lymphoma: a reliable substitute for spleen histopathology? 61
23073320 2012
26
Malignant lymphoma of the spleen in Japan: a clinicopathological analysis of 115 cases. 61
22924843 2012
27
Concurrent splenic diffuse red pulp small B-cell lymphoma and benign clonal proliferation of T-cell large granular lymphocytes. 61
22385871 2012
28
Splenic diffuse red pulp small-B cell lymphoma: toward the emergence of a new lymphoma entity. 61
22541613 2012
29
Splenic marginal zone B-cell lymphoma: a distinct clinicopathological and molecular entity. Recent advances in ontogeny and classification. 61
21760505 2011
30
Relevance of a scoring system including CD11c expression in the identification of splenic diffuse red pulp small B-cell lymphoma (SRPL). 61
20677173 2011
31
Splenic diffuse red pulp small B-cell lymphoma: revision of a series of cases reveals characteristic clinico-pathological features. 61
20220064 2010
32
Commentary on the WHO classification of tumors of lymphoid tissues (2008): indolent B cell lymphomas. 61
19669189 2009

Variations for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Expression for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Search GEO for disease gene expression data for Splenic Diffuse Red Pulp Small B-Cell Lymphoma.

Pathways for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Pathways related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma according to GeneCards Suite gene sharing:

(show all 33)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.32 TP53 NOTCH1 MAP2K1 CCND3 CCNA2
2
Show member pathways
12.55 TP53 NOTCH1 MAP2K1 CCND3 CCNA2
3
Show member pathways
12.49 TP53 NOTCH1 MAP2K1
4
Show member pathways
12.48 TP53 MAP2K1 CCND3 CCNA2
5
Show member pathways
12.39 TP53 NOTCH1 MAP2K1
6
Show member pathways
12.29 TP53 CCND3 CCNA2
7 12.26 TP53 NOTCH1 MAP2K1
8 12.22 TP53 CCND3 CCNA2
9 12.13 TP53 CCND3 CCNA2
10
Show member pathways
12.1 TP53 MAP2K1 CCND3
11 12.06 TP53 CCND3 CCNA2
12
Show member pathways
12.04 TP53 CCND3 CCNA2
13 11.97 TP53 CCND3 CCNA2
14
Show member pathways
11.94 TP53 MAP2K1 CCND3
15 11.9 TP53 MAP2K1 CCND3 CCNA2
16 11.75 TP53 NOTCH1 MAP2K1 CCND3 CCNA2
17 11.71 TP53 NOTCH1 MAP2K1
18
Show member pathways
11.55 CCND3 CCNA2
19 11.55 TP53 CCND3 CCNA2
20
Show member pathways
11.52 TP53 CCNA2
21 11.52 CCND3 CCNA2
22 11.51 TP53 MAP2K1
23 11.48 TP53 MAP2K1
24 11.47 MAP2K1 CCND3
25
Show member pathways
11.46 CCND3 CCNA2
26 11.46 TP53 MAP2K1 CCND3 CCNA2
27 11.4 TP53 CCNA2
28 11.4 TP53 NOTCH1 BCOR
29 11.32 TP53 CCNA2
30 11.3 TP53 CCND3
31 11.19 TP53 CCND3 CCNA2
32 11.11 NOTCH1 MAP2K1
33 10.84 TP53 CCND3 CCNA2

GO Terms for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

Cellular components related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cyclin-dependent protein kinase holoenzyme complex GO:0000307 8.62 CCND3 CCNA2

Biological processes related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma according to GeneCards Suite gene sharing:

(show all 16)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II GO:0000122 9.76 TP53 NOTCH1 CCND3 BCOR
2 positive regulation of gene expression GO:0010628 9.72 TP53 NOTCH1 MAP2K1
3 negative regulation of cell proliferation GO:0008285 9.71 TP53 NOTCH1 MAP2K1
4 positive regulation of transcription, DNA-templated GO:0045893 9.67 TP53 NOTCH1 MAP2K1 CCNA2
5 negative regulation of gene expression GO:0010629 9.65 TP53 NOTCH1 MAP2K1
6 regulation of cell proliferation GO:0042127 9.58 TP53 NOTCH1 CCND3
7 Ras protein signal transduction GO:0007265 9.56 TP53 CCNA2
8 keratinocyte differentiation GO:0030216 9.52 NOTCH1 MAP2K1
9 regulation of cyclin-dependent protein serine/threonine kinase activity GO:0000079 9.51 CCND3 CCNA2
10 animal organ regeneration GO:0031100 9.49 NOTCH1 CCNA2
11 mitotic cell cycle phase transition GO:0044772 9.43 CCND3 CCNA2
12 cell cycle arrest GO:0007050 9.43 TP53 NOTCH1 MAP2K1
13 cardiac septum morphogenesis GO:0060411 9.32 TP53 NOTCH1
14 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.16 TP53 MAP2K1
15 positive regulation of transcription from RNA polymerase II promoter in response to hypoxia GO:0061419 8.96 TP53 NOTCH1
16 heart development GO:0007507 8.92 TP53 NOTCH1 MAP2K1 BCOR

Molecular functions related to Splenic Diffuse Red Pulp Small B-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein kinase binding GO:0019901 9.13 TP53 CCND3 CCNA2
2 cyclin-dependent protein serine/threonine kinase regulator activity GO:0016538 8.62 CCND3 CCNA2

Sources for Splenic Diffuse Red Pulp Small B-Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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