MCID: INT221
MIFTS: 41

Intravascular Large B-Cell Lymphoma

Categories: Blood diseases, Rare diseases, Immune diseases

Aliases & Classifications for Intravascular Large B-Cell Lymphoma

MalaCards integrated aliases for Intravascular Large B-Cell Lymphoma:

Name: Intravascular Large B-Cell Lymphoma 59
Angioendotheliomatosis Proliferans Systemisata 59
Malignant Angioendotheliomatosis 59
Angiotropic Large Cell Lymphoma 59
Intravascular Lymphomatosis 59
Tappeiner-Pfleger Disease 59
Angioendotheliomatosis 73

Characteristics:

Orphanet epidemiological data:

59
intravascular large b-cell lymphoma
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 59 ORPHA98839
ICD10 via Orphanet 34 C83.3
UMLS 73 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 b-cell lymphomas and diffuse large b-cell lymphoma. An important gene associated with Intravascular Large B-Cell Lymphoma is BCL6 (B Cell CLL/Lymphoma 6), and among its related pathways/superpathways are Cytokine Signaling in Immune system and NF-kappaB Signaling. The drugs nivolumab and Immunoglobulin G have been mentioned in the context of this disorder. Affiliated tissues include b cells, skin and lung, and related phenotypes are hematopoietic system 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 125)
# Related Disease Score Top Affiliating Genes
1 b-cell lymphomas 30.4 BCL2 BCL6 CD5 MME
2 diffuse large b-cell lymphoma 29.8 BCL6 CD5 MME
3 leukemia, chronic lymphocytic 28.9 B2M BCL2 BCL6 CD5
4 lymphoma 11.1
5 breast lymphoma 10.4 BCL6 CD5
6 high grade b-cell lymphoma with myc and/ or bcl2 and/or bcl6 rearrangement 10.4 BCL2 BCL6
7 composite lymphoma 10.4 BCL6 CD5
8 nodal marginal zone b-cell lymphoma 10.4 BCL2 BCL6
9 follicular lymphoma 1 10.4 BCL2 BCL6
10 colon lymphoma 10.4 BCL6 CD5
11 prostatitis 10.3
12 cerebritis 10.3
13 primary mediastinal large b-cell lymphoma 10.3 BCL2 BCL6
14 epidural neoplasm 10.3 BCL6 CD5
15 lymphatic system cancer 10.3 BCL6 CD5
16 primary central nervous system lymphoma 10.3 BCL2 BCL6
17 gallbladder lymphoma 10.2 BCL6 MME
18 ectopic thymus 10.2 BCL2 CD5
19 dendritic cell thymoma 10.2 BCL2 CD5
20 heart lymphoma 10.2 BCL6 MME
21 human herpesvirus 8 10.2 BCL2 BCL6
22 epithelial malignant thymoma 10.2 BCL2 CD5
23 reticulosarcoma 10.2 B2M BCL6
24 lung lymphoma 10.2 BCL6 CD5
25 anterior scleritis 10.2 B2M CD5
26 gastric lymphoma 10.2 BCL2 BCL6
27 testicular lymphoma 10.2 BCL6 MME
28 angioimmunoblastic t-cell lymphoma 10.2 BCL6 MME
29 splenic marginal zone lymphoma 10.2 BCL6 CD5
30 hemangioma 10.2
31 panniculitis 10.2
32 t-cell/histiocyte rich large b cell lymphoma 10.2 BCL6 MME
33 tracheal lymphoma 10.2 CD5 MME
34 cholecystitis 10.1
35 senile angioma 10.1
36 hypopituitarism 10.1
37 thrombosis 10.1
38 dementia 10.1
39 stroke, ischemic 10.1
40 thrombocytopenia 10.1
41 syphilis 10.1 B2M BCL2
42 bladder lymphoma 10.1 CD5 MME
43 lung disease 10.0
44 colitis 10.0
45 membranoproliferative glomerulonephritis 10.0
46 interstitial lung disease 10.0
47 meningitis 10.0
48 arthritis 10.0
49 rheumatoid arthritis 10.0
50 glomerulonephritis 10.0

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

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

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.8 B2M BCL2 BCL6 CD5 IL2RB KMT2A
2 immune system MP:0005387 9.8 B2M BCL2 BCL6 CD5 IL2RB KMT2A
3 integument MP:0010771 9.63 B2M BCL2 CD5 IL2RB KMT2A MME
4 liver/biliary system MP:0005370 9.35 MME B2M BCL6 IL2RB KMT2A
5 reproductive system MP:0005389 9.02 B2M BCL2 BCL6 IL2RB KMT2A

Drugs & Therapeutics for Intravascular Large B-Cell Lymphoma

Drugs for Intravascular Large B-Cell Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
nivolumab Approved Phase 2 946414-94-4
2 Immunoglobulin G Phase 2
3 Antibodies Phase 2
4 Immunoglobulins Phase 2
5 Antibodies, Monoclonal Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas Recruiting NCT03038672 Phase 2 Varlilumab
2 Study to Evaluate Safety and Tolerability of XmAb13676 in Patients With CD20-expressing Hematologic Malignancies Recruiting NCT02924402 Phase 1

Search 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:

41
B Cells, Skin, Lung, Bone, Bone Marrow, Prostate, Uterus

Publications for Intravascular Large B-Cell Lymphoma

Articles related to Intravascular Large B-Cell Lymphoma:

(show top 50) (show all 289)
# Title Authors Year
1
Orthodeoxia as a presentation of intravascular large B cell lymphoma. ( 29796279 )
2018
2
Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis. ( 29854538 )
2018
3
Serial chest CT findings of intravascular large B-cell lymphoma of the lungs. ( 29707378 )
2018
4
Small Cell Variant of Intravascular Large B-Cell Lymphoma: Highlighting a Potentially Fatal and Easily Missed Diagnosis. ( 29850589 )
2018
5
Methotrexate-associated Intravascular Large B-cell Lymphoma in a Patient with Rheumatoid Arthritis: A Very Rare Case. ( 29780139 )
2018
6
Recurrent Cytogenetic Abnormalities in Intravascular Large B-Cell Lymphoma. ( 29767679 )
2018
7
Did you 'miss' me? A subtle case of intravascular large B-cell lymphoma. ( 29756305 )
2018
8
Primary pulmonary intravascular large B-cell lymphoma: A report of three cases and literature review. ( 29467882 )
2018
9
Increased uptake on <sup>18</sup> F-fluorodeoxyglucose positron emission tomography/computed tomography is indicative of occult skin lesions in a patient with intravascular large B-cell lymphoma. ( 29569289 )
2018
10
Intravascular Large B Cell Lymphoma of the Gallbladder. ( 29391332 )
2018
11
Intravascular Large B-Cell Lymphoma Within a Thyroid Nodule: A Diagnostic Pitfall. ( 29569516 )
2018
12
Rare Variant of Intravascular Large B-Cell Lymphoma With Hemophagocytic Syndrome. ( 29356737 )
2018
13
Intravascular Large B-Cell Lymphoma Presenting with Diffuse Gallbladder Wall Thickening: A Case Report and Literature Review. ( 29850354 )
2018
14
&amp;quot;Double-expressor&amp;quot; intravascular large B-cell lymphoma involving the female genital tract. ( 29954821 )
2018
15
Random skin biopsy for diagnosis of intravascular large B-cell lymphoma in a patient with hypoxemia and normal lung imaging. ( 29387769 )
2018
16
Intravascular large B-cell lymphoma presenting as panniculitis clinically: a case report. ( 29568849 )
2018
17
Liquid biopsy for the identification of intravascular large B-cell lymphoma. ( 29472348 )
2018
18
A rare case of suspected intravascular large B-cell lymphoma forming nodular masses in the brain and around the prostate and seminal vesicle. ( 29686966 )
2018
19
High prevalence of <i>MYD88</i> and <i>CD79B</i> mutations in intravascular large B-cell lymphoma. ( 29514783 )
2018
20
Autopsy case report of intravascular large B-cell lymphoma with neoplastic PD-L1 expression. ( 29415979 )
2018
21
Intravascular large B-cell lymphoma presenting clinically as rapidly progressive dementia. ( 28726030 )
2017
22
Intravascular large B-cell lymphoma associated with myelofibrosis: A case report. ( 29075489 )
2017
23
A case of intravascular large B-cell lymphoma of lung presenting with progressive multiple nodules on chest computed tomography. ( 28443236 )
2017
24
Complete remission of primary cutaneous intravascular large B-cell lymphoma with R-CHOP followed by rituximab monotherapy. ( 28508527 )
2017
25
Intravascular large B-cell lymphoma presenting as a pituitary mass with bilateral adrenal enlargement and haemophagocytic lymphohistiocytosis. ( 28439895 )
2017
26
Primary Intra-aortic Epstein-Barr Virus-Positive Large B-Cell Lymphoma Presenting as Aortic Mural Thrombosis: An Entity Distinct From Intravascular Large B-Cell Lymphoma. ( 28697692 )
2017
27
Intravascular large B-cell lymphoma presenting as Richter's syndrome with cerebral involvement in a patient with chronic lymphocytic leukemia. ( 28878900 )
2017
28
Pulmonary Intravascular Large B-cell Lymphoma (IVLBCL) Disguised as an Asthma Exacerbation in a Patient with Asthma. ( 28717087 )
2017
29
An autopsy of intravascular large B-cell lymphoma with hemophagocytic syndrome. ( 28515953 )
2017
30
Asian-variant intravascular large B-cell lymphoma. ( 28405077 )
2017
31
Retrospective study of intravascular large B-cell lymphoma cases diagnosed in Quebec: A retrospective study of 29 case reports: Erratum. ( 29019825 )
2017
32
Intravascular large B-cell lymphoma presenting as panniculitis. ( 29296641 )
2017
33
Intravascular large B-cell lymphoma in the United States (US): a population-based study using Surveillance, Epidemiology, and End Results program and National Cancer Database. ( 28278725 )
2017
34
Intravascular large B-cell lymphoma mimicking cerebral amyloid angiopathy-related inflammation. ( 29126561 )
2017
35
A case of intravascular large B cell lymphoma presenting as nodular goiter. ( 28841887 )
2017
36
Synchronous Ductal Carcinoma In Situ and Intravascular Large B-cell Lymphoma of the Breast. ( 28582274 )
2017
37
Intravascular large B-cell lymphoma with multi-organ failure presenting as a pancreatic mass: a case with atypical presentation and definite diagnosis postmortem. ( 29259930 )
2017
38
A Small Case Series of Intravascular Large B-Cell Lymphoma with Unexpected Findings: Subset of Cases with Concomitant Extravascular Central Nervous System (CNS) Involvement Mimicking Primary CNS Lymphoma. ( 28415157 )
2017
39
Molecular Classification of Tumor Cells in a Patient with Intravascular Large B-Cell Lymphoma. ( 28733977 )
2017
40
Low signal intensity lesions on brain susceptibility-weighted MRI in a patient with intravascular large B-cell lymphoma. ( 28804112 )
2017
41
Intravascular large b-cell lymphoma diagnosed via transjugular liver biopsy in a patient with liver dysfunction and thrombocytopenia: A Case Report. ( 28489811 )
2017
42
Intravascular Large B Cell Lymphoma Presenting as Fever of Unknown Origin and Diagnosed by Random Skin Biopsies: A Case Report and Literature Review. ( 28461685 )
2017
43
Renal Intravascular Large B-cell Lymphoma: A Case Report and Review of the Literature. ( 28381751 )
2017
44
Intravascular large B cell lymphoma diagnosed in random duodenal biopsies. A case report and a literature review. ( 28474092 )
2017
45
Image Gallery: Intravascular large B-cell lymphoma presenting with generalized telangiectasia. ( 29192998 )
2017
46
Intravascular large B-cell lymphoma as a cause of terminal acute respiratory distress syndrome: atypical presentation of a rare disease. ( 28444517 )
2017
47
Primary cutaneous B-cell lymphomas with large cell predominance-primary cutaneous follicle center lymphoma, diffuse large B-cell lymphoma, leg type and intravascular large B-cell lymphoma. ( 28065463 )
2016
48
Intravascular large B-cell lymphoma with a high titer of proteinase-3-anti-neutrophil cytoplasmic antibody mimicking granulomatosis with polyangiitis. ( 27398728 )
2016
49
Intravascular Large B-cell Lymphoma of the Bilateral Ovaries and Uterus in an Asymptomatic Patient with a t(11;22)(q23;q11) Constitutional Translocation. ( 27803414 )
2016
50
Circulating lymphoma cells in intravascular large B-cell lymphoma. ( 27657358 )
2016

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

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 8.8 B2M CD5 IL2RB

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of cell proliferation GO:0008285 9.58 BCL2 BCL6 KMT2A
2 cytokine-mediated signaling pathway GO:0019221 9.5 BCL2 BCL6 IL2RB
3 post-embryonic development GO:0009791 9.43 BCL2 KMT2A
4 B cell differentiation GO:0030183 9.37 BCL2 BCL6
5 positive regulation of B cell proliferation GO:0030890 9.26 BCL2 BCL6
6 spleen development GO:0048536 9.16 BCL2 KMT2A
7 T cell differentiation in thymus GO:0033077 8.96 B2M BCL2
8 homeostasis of number of cells within a tissue GO:0048873 8.62 BCL2 KMT2A

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

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 8.92 B2M BCL2 BCL6 KMT2A

Sources for Intravascular Large 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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