MCID: INT221
MIFTS: 37

Intravascular Large B-Cell Lymphoma

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases

Aliases & Classifications for Intravascular Large B-Cell Lymphoma

MalaCards integrated aliases for Intravascular Large B-Cell Lymphoma:

Name: Intravascular Large B-Cell Lymphoma 58
Angioendotheliomatosis Proliferans Systemisata 58
Malignant Angioendotheliomatosis 58
Angiotropic Large Cell Lymphoma 58
Intravascular Lymphomatosis 58
Tappeiner-Pfleger Disease 58
Angioendotheliomatosis 70

Characteristics:

Orphanet epidemiological data:

58
intravascular large b-cell lymphoma
Inheritance: Not applicable;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 via Orphanet 33 C83.3
Orphanet 58 ORPHA98839
UMLS 70 C0334660

Summaries for Intravascular Large B-Cell Lymphoma

MalaCards based summary : Intravascular Large B-Cell Lymphoma, also known as angioendotheliomatosis proliferans systemisata, is related to lymphosarcoma and primary central nervous system lymphoma. An important gene associated with Intravascular Large B-Cell Lymphoma is BCL6 (BCL6 Transcription Repressor), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. Affiliated tissues include bone marrow, pituitary and prostate, and related phenotypes are Reduced mammosphere formation and immune system

Related Diseases for Intravascular Large B-Cell Lymphoma

Diseases related to Intravascular Large B-Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 317)
# Related Disease Score Top Affiliating Genes
1 lymphosarcoma 30.4 BCL6 BCL2
2 primary central nervous system lymphoma 30.3 MYD88 BCL6 BCL2
3 lymphoma 30.3 MYD88 MYC CD5 BCL6 BCL2 B2M
4 b-cell lymphoma 30.3 MYD88 MYC MME CD79B CD5 BCL6
5 lymphoma aids related 30.1 MYC BCL6
6 prostate lymphoma 30.0 MME CD5 BCL6
7 lung lymphoma 30.0 MME CD5 BCL6
8 gallbladder lymphoma 30.0 MME CD5 BCL6
9 central nervous system lymphoma 29.9 MYD88 MYC BCL6
10 primary cutaneous diffuse large b-cell lymphoma, leg type 29.9 MYD88 MYC
11 richter's syndrome 29.8 MYC CD5
12 cll/sll 29.7 MME CD79B CD5 BCL6
13 peripheral t-cell lymphoma 29.6 MME IL2RB CD5 BCL6
14 testicular lymphoma 29.6 MYD88 MME CD79B BCL6
15 acquired immunodeficiency syndrome 29.5 MYC IL2RB BCL6 B2M
16 plasmacytoma 29.5 MYC CD79B CD5 B2M
17 reticulosarcoma 29.4 MYC BCL6 BCL2 B2M
18 adult lymphoma 29.4 MYC MME CD5 BCL6
19 mature t-cell and nk-cell lymphoma 29.4 MYC MME CD5 BCL6
20 diffuse large b-cell lymphoma 29.3 MYD88 MYC MME CD79B CD5 BCL6
21 burkitt lymphoma 29.3 MYC MME BCL6 BCL2
22 myeloma, multiple 29.1 MYD88 MYC CD5 BCL6 B2M
23 follicular lymphoma 29.1 MYC MME CD5 BCL6 BCL2
24 mature b-cell neoplasm 29.1 MYC MME CD5 BCL6 B2M
25 mantle cell lymphoma 29.0 MYC MME CD5 BCL6 BCL2 B2M
26 lymphoplasmacytic lymphoma 29.0 MYD88 MME CD79B CD5 BCL6 B2M
27 systemic lupus erythematosus 29.0 MYD88 IL2RB CD5 BCL6 B2M
28 lymphoblastic lymphoma 28.9 MYC MME KMT2A CD5 BCL6
29 lymphoma, non-hodgkin, familial 28.2 MYD88 MYC MME KMT2A IL2RB CD79B
30 leukemia, chronic lymphocytic 27.9 MYD88 MYC MME KMT2A CD79B CD5
31 lymphoma, mucosa-associated lymphoid type 27.9 MYD88 MYC MME CD79B CD5 BCL6
32 lymphoma, hodgkin, classic 10.7
33 pancytopenia 10.6
34 proteinuria, chronic benign 10.6
35 splenomegaly 10.6
36 hemangioma 10.6
37 thrombocytopenia 10.6
38 cauda equina syndrome 10.5
39 respiratory failure 10.5
40 vasculitis 10.5
41 panniculitis 10.5
42 hemophagocytic lymphohistiocytosis 10.4
43 lung disease 10.4
44 rheumatoid arthritis 10.4
45 lymphoproliferative syndrome 10.4
46 telangiectasis 10.4
47 cholecystitis 10.4
48 interstitial lung disease 10.4
49 arthritis 10.4
50 hypopituitarism 10.4

Graphical network of the top 20 diseases related to Intravascular Large B-Cell Lymphoma:



Diseases related to Intravascular Large B-Cell Lymphoma

Symptoms & Phenotypes for Intravascular Large B-Cell Lymphoma

GenomeRNAi Phenotypes related to Intravascular Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.02 BCL6 CD5 KMT2A MYC MYD88

MGI Mouse Phenotypes related to Intravascular Large B-Cell Lymphoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 10.07 B2M BCL2 BCL6 CD5 CD79B IL2RB
2 hematopoietic system MP:0005397 10.06 B2M BCL2 BCL6 CD5 CD79B IL2RB
3 growth/size/body region MP:0005378 10.03 B2M BCL2 BCL6 CD5 IL2RB KMT2A
4 cardiovascular system MP:0005385 10 B2M BCL2 BCL6 KMT2A MME MYC
5 integument MP:0010771 9.92 B2M BCL2 BCL6 CD5 IL2RB KMT2A
6 liver/biliary system MP:0005370 9.8 B2M BCL6 IL2RB KMT2A MME MYC
7 neoplasm MP:0002006 9.55 B2M BCL2 KMT2A MYC MYD88
8 no phenotypic analysis MP:0003012 9.43 BCL6 CD5 CD79B KMT2A MYC MYD88
9 reproductive system MP:0005389 9.17 B2M BCL2 BCL6 IL2RB KMT2A MYC

Drugs & Therapeutics for Intravascular Large B-Cell Lymphoma

Search Clinical Trials , NIH Clinical Center for Intravascular Large B-Cell Lymphoma

Genetic Tests for Intravascular Large B-Cell Lymphoma

Anatomical Context for Intravascular Large B-Cell Lymphoma

MalaCards organs/tissues related to Intravascular Large B-Cell Lymphoma:

40
Bone Marrow, Pituitary, Prostate, Lung, Myeloid, Bone, Spleen

Publications for Intravascular Large B-Cell Lymphoma

Articles related to Intravascular Large B-Cell Lymphoma:

(show top 50) (show all 535)
# Title Authors PMID Year
1
Cytogenetic complexity and heterogeneity in intravascular lymphoma. 61
32763919 2021
2
CD40L from platelet, endothelial cell, and smooth muscle cell may contribute to the proliferation of tumor cell in intravascular large B cell lymphoma. 61
33560569 2021
3
Intravascular large B-cell lymphoma diagnosed by incisional random skin biopsy after failure of diagnosis using punch biopsy: A case report. 61
32940371 2021
4
Intravascular large B-cell lymphoma mimicking erythema nodosum: A case report and review of published works. 61
33554355 2021
5
Frequent genetic alterations in immune checkpoint-related genes in intravascular large B-cell lymphoma. 61
33512416 2021
6
Impact of event-free survival status after stem cell transplantation on subsequent survival of patients with lymphoma. 61
33666656 2021
7
[Clinicopathological features and MYD88 L265P mutation status of intravascular large B cell lymphoma]. 61
33677885 2021
8
Clinicopathological features of Chinese patients with B-cell lymphoproliferative disorders and kidney infiltration. 61
33729651 2021
9
Intravascular large B-cell lymphoma presenting with reticular telangiectasia on the trunk and panhypopituitarism: an autopsy case. 61
33731406 2021
10
[The 485th case: fever of undetermined origin and hypoxemia]. 61
33663183 2021
11
A case of intravascular large B-cell lymphoma with renal involvement presenting with elevated serum ANCA titers. 61
32776181 2021
12
Occult Diffuse Neoplasm in the Lungs: Intravascular Large B-Cell Lymphoma. 61
33640342 2021
13
Renal intravascular large B-cell lymphoma predominantly localized in peritubular capillaries. 61
33537904 2021
14
Intravascular large B-cell lymphoma presenting with extensive pulmonary embolism. 61
33336796 2021
15
Intravascular Large B-Cell Lymphoma Within the Appendix Presenting as Acute Abdomen: A Challenging Diagnosis for Hematologists 61
32755127 2021
16
Encephalomyeloradiculoneuropathy Revealing a Rare Case of Intravascular Large B-Cell Lymphoma. 61
33614346 2021
17
A case report and literature review of cutaneous intravascular large B-cell lymphoma presenting clinically as panniculitis: a difficult diagnosis, but a good prognosis. 61
33261907 2021
18
The first case of a collision tumor of gastrointestinal stromal tumor and intravascular large B cell lymphoma. 61
33459824 2021
19
A Case of Intravascular Diffuse Large B-Cell Lymphoma Initially Suspected as Interstitial Pneumonia Associated With Systemic Scleroderma. 61
33736495 2021
20
Primary Pulmonary B-Cell Lymphoma: A Review and Update. 61
33499258 2021
21
Intravascular Large B-Cell Lymphoma and Renal Clear Cell Carcinoma as Collision Tumor: A Case Report and Review of the Literature. 61
33345662 2020
22
Interleukin-6-producing Intravascular Large B-cell Lymphoma with Lymphadenopathy Mimicking the Histology of Multicentric Castleman Disease. 61
32759587 2020
23
A poisoned cherry: Migratory cutaneous intravascular large B-cell lymphoma with subsequent systemic nodal lymphoma. 61
33299917 2020
24
Appropriate indication and procedure for random skin biopsy in the diagnosis of intravascular large B-cell lymphoma. 61
33338260 2020
25
Intravascular large B-cell lymphoma presenting as a nondescript macular eruption. 61
32297309 2020
26
Going Skin Deep: Excavating a Diagnosis of Intravascular Large B Cell Lymphoma. 61
32815059 2020
27
[Intravascular large B cell lymphoma pathological findings led by positron emission tomography findings: About one case]. 61
32753278 2020
28
Incidental intravascular large B-cell lymphoma in a renal cell carcinoma. 61
32863023 2020
29
Successful myeloablative unrelated bone marrow transplantation for relapsed intravascular large B cell lymphoma after autologous peripheral blood stem cell transplantation. 61
33090263 2020
30
Analysis of clinicopathological features and prognostic factors of non-Hodgkin's intravascular large B-cell lymphoma. 61
32802165 2020
31
Intravascular large B-cell lymphoma in Hispanics: a case series and literature review. 61
33194130 2020
32
Aggressive B-cell lymphomas with a primary bone marrow presentation. 61
32324290 2020
33
Renal intravascular large B cell lymphoma: the first case report in Korea and a review of the literature. 61
32781826 2020
34
CNS lymphoma, the Irish experience: A retrospective review of neuropathologically confirmed cases over 10 years. 61
32301694 2020
35
Intravascular Large B-Cell Lymphoma Involving Multinodular Goiter and Mimicking Carcinoma. 61
31566036 2020
36
Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge. 61
32750245 2020
37
PD-L1 (SP142) expression in neoplastic cells predicts a poor prognosis for patients with intravascular large B-cell lymphoma treated with rituximab-based multi-agent chemotherapy. 61
32367674 2020
38
Concurrent mycosis fungoides and intravascular large B-cell lymphoma in a single patient. 61
32087036 2020
39
Intravascular large B-cell lymphoma presenting as an isolated cauda equina-conus medullaris syndrome - A case report. 61
30346252 2020
40
Skin biopsies using dermoscopy for earlier diagnosis of intravascular large B-cell lymphoma. 61
32275085 2020
41
Asian variant of intravascular large B-cell lymphoma: a comparison of clinical features based on involvement of the central nervous system. 61
31752477 2020
42
Intravascular large B cell lymphoma presenting as an isolated cauda equina-conus medullaris syndrome-a case report and review of the literature of the neuroimaging features at onset. 61
31950461 2020
43
Serum interleukin-10 as a valuable biomarker for early diagnosis and therapeutic monitoring in intravascular large B-cell lymphoma. 61
32634257 2020
44
Post-mortem diagnosis of intravascular large B-cell lymphoma. 61
32485117 2020
45
Hemophagocytic syndrome-associated intravascular large B-cell lymphoma. 61
32585028 2020
46
Multiple Cerebral Hemorrhages With Microbleeds in Intravascular Large B-Cell Lymphoma. 61
32249205 2020
47
EBV-positive intravascular large B-cell lymphoma of the liver: a case report and literature review. 61
32513269 2020
48
Intravascular large B-cell lymphoma: a forgotten stroke "mimic". 61
30848425 2020
49
Intravascular large B-cell lymphoma presenting with acute encephalopathy. 61
32437565 2020
50
Large B-cell lymphoma of the uvea: Histopathologic variants and clinicopathologic correlation. 61
31676312 2020

Variations for Intravascular Large B-Cell Lymphoma

Expression for Intravascular Large B-Cell Lymphoma

Search GEO for disease gene expression data for Intravascular Large B-Cell Lymphoma.

Pathways for Intravascular Large B-Cell Lymphoma

Pathways related to Intravascular Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

(show all 21)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.6 MYD88 MYC MME IL2RB CD79B BCL6
2
Show member pathways
12.91 MYD88 MYC IL2RB BCL6 BCL2 B2M
3
Show member pathways
12.65 MYD88 MYC BCL2 B2M
4
Show member pathways
12.51 MYD88 MYC IL2RB CD79B
5
Show member pathways
12.26 MYD88 MYC IL2RB BCL2 B2M
6 12.18 MYD88 IL2RB BCL6 B2M
7
Show member pathways
12.16 MYC IL2RB BCL2
8
Show member pathways
12.14 MYC CD79B BCL6
9 12.07 MYC IL2RB B2M
10
Show member pathways
11.78 MYC IL2RB BCL2
11 11.69 MYC BCL6 BCL2
12 11.67 MYC BCL6 BCL2
13 11.54 MYC KMT2A IL2RB BCL6
14
Show member pathways
11.26 MYC IL2RB BCL2
15 11.24 MYC BCL2
16
Show member pathways
11.23 MYC BCL2
17
Show member pathways
11.12 IL2RB BCL2
18 10.99 MME IL2RB CD79B CD5
19 10.98 MYC BCL2
20
Show member pathways
10.85 MYC BCL2

GO Terms for Intravascular Large B-Cell Lymphoma

Cellular components related to Intravascular Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 8.92 IL2RB CD79B CD5 B2M

Biological processes related to Intravascular Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of B cell proliferation GO:0030890 9.4 BCL6 BCL2
2 branching involved in ureteric bud morphogenesis GO:0001658 9.37 MYC BCL2
3 negative regulation of apoptotic process GO:0043066 9.35 MYD88 MYC IL2RB BCL6 BCL2
4 B cell differentiation GO:0030183 9.33 CD79B BCL6 BCL2
5 T cell differentiation in thymus GO:0033077 9.32 BCL2 B2M
6 response to gamma radiation GO:0010332 9.26 MYC BCL2
7 cytokine-mediated signaling pathway GO:0019221 9.02 MYD88 MYC IL2RB BCL6 BCL2

Molecular functions related to Intravascular Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein homodimerization activity GO:0042803 9.26 MME KMT2A BCL2 B2M
2 repressing transcription factor binding GO:0070491 9.16 MYC BCL2
3 identical protein binding GO:0042802 9.1 MYD88 KMT2A CD79B BCL6 BCL2 B2M

Sources for Intravascular Large 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 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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