MCID: INT221
MIFTS: 42

Intravascular Large B-Cell Lymphoma

Categories: Immune diseases, Blood 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 55
Angioendotheliomatosis Proliferans Systemisata 55
Malignant Angioendotheliomatosis 55
Angiotropic Large Cell Lymphoma 55
Intravascular Lymphomatosis 55
Tappeiner-Pfleger Disease 55
Angioendotheliomatosis 69

Characteristics:

Orphanet epidemiological data:

55
intravascular large b-cell lymphoma
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 55 ORPHA98839
ICD10 via Orphanet 33 C83.3
UMLS 69 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 Antibodies, Monoclonal 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 128)
# Related Disease Score Top Affiliating Genes
1 b-cell lymphomas 30.8 BCL2 BCL6 CD5 MME
2 diffuse large b-cell lymphoma 30.0 BCL6 CD5 MME
3 leukemia, chronic lymphocytic 29.2 B2M BCL2 BCL6 CD5
4 lymphoma 11.1
5 prostatitis 10.3
6 cerebritis 10.3
7 breast lymphoma 10.3 BCL6 CD5
8 high grade b-cell lymphoma with myc and/ or bcl2 and/or bcl6 rearrangement 10.2 BCL2 BCL6
9 nodal marginal zone b-cell lymphoma 10.2 BCL2 BCL6
10 follicular lymphoma 1 10.2 BCL2 BCL6
11 composite lymphoma 10.2 BCL6 CD5
12 colon lymphoma 10.2 BCL6 CD5
13 hemangioma 10.2
14 chops syndrome 10.2
15 chromosomal triplication 10.2 BCL6 KMT2A
16 extramedullary plasmacytoma 10.2 B2M BCL2
17 epidural neoplasm 10.2 BCL6 CD5
18 ectopic thymus 10.2 BCL2 CD5
19 primary central nervous system lymphoma 10.2 BCL2 BCL6
20 dendritic cell thymoma 10.1 BCL2 CD5
21 gallbladder lymphoma 10.1 BCL6 MME
22 heart lymphoma 10.1 BCL6 MME
23 epithelial malignant thymoma 10.1 BCL2 CD5
24 human herpesvirus 8 10.1 BCL2 BCL6
25 lymphatic system cancer 10.1 BCL6 CD5
26 anterior scleritis 10.1 B2M CD5
27 dementia 10.1
28 glass syndrome 10.1
29 panniculitis 10.1
30 ring chromosome y syndrome 10.1
31 thrombocytopenia 10.1
32 cholecystitis 10.1
33 senile angioma 10.1
34 hypopituitarism 10.1
35 complement hyperactivation, angiopathic thrombosis, and protein-losing enteropathy 10.1
36 thrombosis 10.1
37 stroke, ischemic 10.1
38 gastric lymphoma 10.1 BCL2 BCL6
39 testicular lymphoma 10.1 BCL6 MME
40 lymphoid leukemia 10.1 CD5 KMT2A
41 angioimmunoblastic t-cell lymphoma 10.1 BCL6 MME
42 lung lymphoma 10.1 BCL6 CD5
43 tracheal lymphoma 10.1 CD5 MME
44 lymphosarcoma 10.1 BCL2 BCL6
45 t-cell/histiocyte rich large b cell lymphoma 10.1 BCL6 MME
46 reticulosarcoma 10.1 B2M BCL6
47 splenic marginal zone lymphoma 10.1 BCL6 CD5
48 pancytopenia 10.0
49 hepatitis 10.0
50 lung disease 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:

43
# 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 B2M BCL6 IL2RB KMT2A MME
5 reproductive system MP:0005389 9.02 BCL2 BCL6 IL2RB KMT2A B2M

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 Antibodies, Monoclonal Phase 2
3 Immunoglobulins Phase 2
4 Antibodies 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:

38
B Cells, Skin, Lung, Bone, Bone Marrow, Prostate, Liver

Publications for Intravascular Large B-Cell Lymphoma

Articles related to Intravascular Large B-Cell Lymphoma:

(show top 50) (show all 272)
# Title Authors Year
1
Random skin biopsy for diagnosis of intravascular large B-cell lymphoma in a patient with hypoxemia and normal lung imaging. ( 29387769 )
2018
2
Autopsy case report of intravascular large B-cell lymphoma with neoplastic PD-L1 expression. ( 29415979 )
2018
3
Intravascular Large B Cell Lymphoma of the Gallbladder. ( 29391332 )
2018
4
Rare Variant of Intravascular Large B-Cell Lymphoma With Hemophagocytic Syndrome. ( 29356737 )
2018
5
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
6
Intravascular large B-cell lymphoma presenting clinically as rapidly progressive dementia. ( 28726030 )
2017
7
Intravascular large B-cell lymphoma presenting as a pituitary mass with bilateral adrenal enlargement and haemophagocytic lymphohistiocytosis. ( 28439895 )
2017
8
Complete remission of primary cutaneous intravascular large B-cell lymphoma with R-CHOP followed by rituximab monotherapy. ( 28508527 )
2017
9
Intravascular large B cell lymphoma diagnosed in random duodenal biopsies. A case report and a literature review. ( 28474092 )
2017
10
An autopsy of intravascular large B-cell lymphoma with hemophagocytic syndrome. ( 28515953 )
2017
11
Molecular Classification of Tumor Cells in a Patient with Intravascular Large B-Cell Lymphoma. ( 28733977 )
2017
12
Image Gallery: Intravascular large B-cell lymphoma presenting with generalized telangiectasia. ( 29192998 )
2017
13
Intravascular large B-cell lymphoma associated with myelofibrosis: A case report. ( 29075489 )
2017
14
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
15
Intravascular large B-cell lymphoma as a cause of terminal acute respiratory distress syndrome: atypical presentation of a rare disease. ( 28444517 )
2017
16
Intravascular large B-cell lymphoma presenting as Richter's syndrome with cerebral involvement in a patient with chronic lymphocytic leukemia. ( 28878900 )
2017
17
Low signal intensity lesions on brain susceptibility-weighted MRI in a patient with intravascular large B-cell lymphoma. ( 28804112 )
2017
18
Intravascular large b-cell lymphoma diagnosed via transjugular liver biopsy in a patient with liver dysfunction and thrombocytopenia: A Case Report. ( 28489811 )
2017
19
Retrospective study of intravascular large B-cell lymphoma cases diagnosed in Quebec: A retrospective study of 29 case reports: Erratum. ( 29019825 )
2017
20
Pulmonary Intravascular Large B-cell Lymphoma (IVLBCL) Disguised as an Asthma Exacerbation in a Patient with Asthma. ( 28717087 )
2017
21
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
22
Intravascular large B-cell lymphoma mimicking cerebral amyloid angiopathy-related inflammation. ( 29126561 )
2017
23
A case of intravascular large B cell lymphoma presenting as nodular goiter. ( 28841887 )
2017
24
Asian-variant intravascular large B-cell lymphoma. ( 28405077 )
2017
25
Synchronous Ductal Carcinoma In Situ and Intravascular Large B-cell Lymphoma of the Breast. ( 28582274 )
2017
26
A case of intravascular large B-cell lymphoma of lung presenting with progressive multiple nodules on chest computed tomography. ( 28443236 )
2017
27
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
28
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
29
Renal Intravascular Large B-cell Lymphoma: A Case Report and Review of the Literature. ( 28381751 )
2017
30
Intravascular large B-cell lymphoma presenting as panniculitis. ( 29296641 )
2017
31
Reversible Hypopituitarism Associated with Intravascular Large B-Cell Lymphoma: Case Report of Successful Immunochemotherapy. ( 26936510 )
2016
32
Intravascular large b-cell lymphoma diagnosed at autopsy: A case report. ( 27773147 )
2016
33
Intravascular Large B-Cell Lymphoma Resembling Myelodysplastic Syndrome in the Bone Marrow. ( 27913782 )
2016
34
A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure. ( 27777803 )
2016
35
Methotrexate-associated Intravascular Large B-cell Lymphoma in a Patient with Rheumatoid Arthritis. ( 27301524 )
2016
36
An autopsy case of intravascular large B-cell lymphoma with subcortical U-fiber sparing and unique lymphocyte markers. ( 27653906 )
2016
37
Intravascular large B-cell lymphoma associated with silicone breast implant, HLA-DRB1*11:01, and HLA-DQB1*03:01 manifesting as macrophage activation syndrome and with severe neurological symptoms: a case report. ( 27634631 )
2016
38
Intravascular large B-cell lymphoma with a high titer of proteinase-3-anti-neutrophil cytoplasmic antibody mimicking granulomatosis with polyangiitis. ( 27398728 )
2016
39
Intense focal pituitary FDG uptake due to intravascular large B-cell lymphoma in pyrexia of unknown origin. ( 27387341 )
2016
40
The Value of 18F-FDG PET/CT in Diagnostic Procedure of Intravascular Large B-Cell Lymphoma Presenting Fever of Unknown Origin and Pulmonary Hypertension as an Initial Manifestation. ( 26914573 )
2016
41
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
42
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
43
Circulating lymphoma cells in intravascular large B-cell lymphoma. ( 27657358 )
2016
44
Cutaneous variant of intravascular large B-cell lymphoma in a Japanese patient: An occult lesion localized in a solitary angiolipoma. ( 27422850 )
2016
45
High Frequency of Bone Marrow Involvement in Intravascular Large B-Cell Lymphoma: A Clinicopathological Study of 13 Cases in China. ( 27553679 )
2016
46
A case of intravascular large B cell lymphoma: New clinical and immunohistochemical findings. ( 27090763 )
2016
47
A case of intravascular large B-cell lymphoma in the left adrenal and another tumor in the right adrenal detected by (18)F-FDG PET/CT. ( 26929945 )
2016
48
Dominant neurologic symptomatology in intravascular large B-cell lymphoma. ( 27546361 )
2016
49
Development and analysis of patient-derived xenograft mouse models in intravascular large B-cell lymphoma. ( 27001523 )
2016
50
Autologous hematopoietic stem cell transplantation for intravascular large B-cell lymphoma: the European Society for Blood and Marrow Transplantation experience. ( 27991887 )
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 post-embryonic development GO:0009791 9.43 BCL2 KMT2A
3 B cell differentiation GO:0030183 9.37 BCL2 BCL6
4 cell morphogenesis GO:0000902 9.32 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 homeostasis of number of cells within a tissue GO:0048873 8.96 BCL2 KMT2A
8 T cell differentiation in thymus GO:0033077 8.62 B2M BCL2

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
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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