MCID: INT221
MIFTS: 38

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 56
Angioendotheliomatosis Proliferans Systemisata 56
Malignant Angioendotheliomatosis 56
Angiotropic Large Cell Lymphoma 56
Intravascular Lymphomatosis 56
Tappeiner-Pfleger Disease 56
Angioendotheliomatosis 69

Characteristics:

Orphanet epidemiological data:

56
intravascular large b-cell lymphoma
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 56 ORPHA98839
ICD10 via Orphanet 34 C83.3

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 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 Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include b cells, bone and skin, 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 111)
id Related Disease Score Top Affiliating Genes
1 b-cell lymphomas 11.0
2 lymphoma 11.0
3 cervical endometrioid adenocarcinoma 10.4 BCL6 CD5
4 thrombocytopenia, anemia, and myelofibrosis 10.4 BCL2 BCL6
5 pseudotyphus of california 10.4 BCL2 BCL6
6 pediatric lymphoma 10.4 BCL6 CD5
7 penile disease 10.3 BCL6 CD5
8 systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease 10.3 BCL6 KMT2A
9 childhood spinal cord tumor 10.3 BCL6 CD5
10 solitary plasmacytoma of chest wall 10.3 B2M BCL2
11 gastric small cell carcinoma 10.3 BCL6 MME
12 vaccinia 10.3 BCL2 CD5
13 primary malignant melanoma of the cervix 10.3 BCL2 BCL6
14 glandular-alveolar pattern testicular yolk sac tumor 10.2 BCL2 CD5
15 prostatitis 10.2
16 lymphoma, small cleaved-cell, diffuse 10.2 CD5 KMT2A
17 heterotaxy, visceral, 4, autosomal 10.2 BCL2 BCL6
18 inclusion conjunctivitis 10.2 B2M CD5
19 angiomatosis, diffuse corticomeningeal, of divry and van bogaert 10.2 BCL6 MME
20 cerebritis 10.2
21 estrogen-receptor positive breast cancer 10.2 BCL6 CD5
22 pylorus cancer 10.2 BCL2 BCL6
23 trigeminal nerve neoplasm 10.2 CD5 MME
24 villoglandular endometrial endometrioid adenocarcinoma 10.2 BCL6 CD5
25 tang hsi ryu syndrome 10.2 BCL6 MME
26 paraneoplastic polyneuropathy 10.2 BCL2 BCL6
27 syngnathia cleft palate 10.1 BCL6 CD5
28 diffuse large b-cell lymphoma 10.1
29 hemangioma 10.1
30 degenerative myopia 10.1 CD5 MME
31 hodgkin's lymphoma, lymphocytic-histiocytic predominance 10.1 B2M BCL6
32 acral lentiginous melanoma 10.1 B2M BCL6
33 thrombocytopenia 10.1
34 thrombosis 10.1
35 dementia 10.1
36 cholecystitis 10.1
37 senile angioma 10.1
38 hypopituitarism 10.1
39 ovarian mesodermal adenosarcoma 10.1 BCL2 MME
40 hepatic osteogenic sarcoma 10.0 CD5 MME
41 glomerulonephritis 10.0
42 hepatitis 10.0
43 colitis 10.0
44 pancytopenia 10.0
45 panniculitis 10.0
46 membranoproliferative glomerulonephritis 10.0
47 interstitial lung disease 10.0
48 lung disease 10.0
49 meningitis 10.0
50 hemeralopia, congenital essential 9.9 BCL2 IL2RB

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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.8 CD5 IL2RB KMT2A B2M BCL2 BCL6
2 immune system MP:0005387 9.8 CD5 IL2RB KMT2A MME B2M BCL2
3 integument MP:0010771 9.63 CD5 IL2RB KMT2A MME B2M BCL2
4 liver/biliary system MP:0005370 9.35 IL2RB KMT2A MME B2M BCL6
5 reproductive system MP:0005389 9.02 BCL6 IL2RB KMT2A B2M BCL2

Drugs & Therapeutics for Intravascular Large B-Cell Lymphoma

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


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

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas Not yet 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:

39
B Cells, Bone, Skin, Lung, Bone Marrow, Prostate, Uterus

Publications for Intravascular Large B-Cell Lymphoma

Articles related to Intravascular Large B-Cell Lymphoma:

(show top 50) (show all 262)
id Title Authors Year
1
An autopsy of intravascular large B-cell lymphoma with hemophagocytic syndrome. ( 28515953 )
2017
2
Pulmonary Intravascular Large B-cell Lymphoma (IVLBCL) Disguised as an Asthma Exacerbation in a Patient with Asthma. ( 28717087 )
2017
3
A case of intravascular large B cell lymphoma presenting as nodular goiter. ( 28841887 )
2017
4
Synchronous Ductal Carcinoma In Situ and Intravascular Large B-cell Lymphoma of the Breast. ( 28582274 )
2017
5
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
6
Intravascular large B cell lymphoma diagnosed in random duodenal biopsies. A case report and a literature review. ( 28474092 )
2017
7
Intravascular large B-cell lymphoma presenting as a pituitary mass with bilateral adrenal enlargement and haemophagocytic lymphohistiocytosis. ( 28439895 )
2017
8
Intravascular large B-cell lymphoma presenting as Richter's syndrome with cerebral involvement in a patient with chronic lymphocytic leukemia. ( 28878900 )
2017
9
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
10
Intravascular large B-cell lymphoma as a cause of terminal acute respiratory distress syndrome: atypical presentation of a rare disease. ( 28444517 )
2017
11
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
12
Intravascular large B-cell lymphoma presenting clinically as rapidly progressive dementia. ( 28726030 )
2017
13
Renal Intravascular Large B-cell Lymphoma: A Case Report and Review of the Literature. ( 28381751 )
2017
14
A case of intravascular large B-cell lymphoma of lung presenting with progressive multiple nodules on chest computed tomography. ( 28443236 )
2017
15
Molecular Classification of Tumor Cells in a Patient with Intravascular Large B-Cell Lymphoma. ( 28733977 )
2017
16
Asian-variant intravascular large B-cell lymphoma. ( 28405077 )
2017
17
Complete remission of primary cutaneous intravascular large B-cell lymphoma with R-CHOP followed by rituximab monotherapy. ( 28508527 )
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
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
20
Low signal intensity lesions on brain susceptibility-weighted MRI in a patient with intravascular large B-cell lymphoma. ( 28804112 )
2017
21
Cutaneous variant of intravascular large B-cell lymphoma in a Japanese patient: An occult lesion localized in a solitary angiolipoma. ( 27422850 )
2016
22
Dominant neurologic symptomatology in intravascular large B-cell lymphoma. ( 27546361 )
2016
23
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
24
Intravascular large B-cell lymphoma: masquerade as membranoproliferative glomerulonephritis. ( 27312458 )
2016
25
Intravascular large B cell lymphoma presenting as transient erythema nodosum-like lesions and fever. ( 27687069 )
2016
26
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
27
A case of intravascular large B cell lymphoma: New clinical and immunohistochemical findings. ( 27090763 )
2016
28
Intravascular large B-cell lymphoma with a high titer of proteinase-3-anti-neutrophil cytoplasmic antibody mimicking granulomatosis with polyangiitis. ( 27398728 )
2016
29
A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure. ( 27777803 )
2016
30
Development and analysis of patient-derived xenograft mouse models in intravascular large B-cell lymphoma. ( 27001523 )
2016
31
Early diagnosis of intravascular large B-cell lymphoma. ( 27879065 )
2016
32
Intravascular large b-cell lymphoma diagnosed at autopsy: A case report. ( 27773147 )
2016
33
Three Cases of Intravascular Large B-Cell Lymphoma Detected in a Biopsy of Skin Lesions. ( 26871422 )
2016
34
High Frequency of Bone Marrow Involvement in Intravascular Large B-Cell Lymphoma: A Clinicopathological Study of 13 Cases in China. ( 27553679 )
2016
35
Circulating lymphoma cells in intravascular large B-cell lymphoma. ( 27657358 )
2016
36
Intravascular Large B-Cell Lymphoma Coexisting with an Ovarian Carcinoma. ( 27334860 )
2016
37
Autologous hematopoietic stem cell transplantation for intravascular large B-cell lymphoma: the European Society for Blood and Marrow Transplantation experience. ( 27991887 )
2016
38
Rituximab monotherapy to treat intravascular large B-cell lymphoma manifesting as pruritic calf patch in an elderly female. ( 27098895 )
2016
39
Neurologic manifestations of intravascular large B-cell lymphoma. ( 26918203 )
2016
40
Seemed Like Cellulitis: Intravascular Large B-Cell Lymphoma. ( 27038674 )
2016
41
Intestinal Intravascular Large B-cell Lymphoma Mimicking Ulcerative Colitis with Secondary Membranoproliferative Glomerulonephritis. ( 27580553 )
2016
42
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
43
Methotrexate-associated Intravascular Large B-cell Lymphoma in a Patient with Rheumatoid Arthritis. ( 27301524 )
2016
44
Glomerular infiltration by intravascular large B-cell lymphoma. ( 27391281 )
2016
45
Intravascular large B-cell lymphoma in a kidney biopsy. ( 28092895 )
2016
46
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
47
Hemophagocytosis-Related (Asian Variant) Intravascular Large B-Cell Lymphoma in a Hispanic Patient: a88A Case Report Highlighting a Micronodular Pattern in the Spleen. ( 27247375 )
2016
48
An autopsy case of intravascular large B-cell lymphoma with subcortical U-fiber sparing and unique lymphocyte markers. ( 27653906 )
2016
49
Intravascular Large B-Cell Lymphoma Resembling Myelodysplastic Syndrome in the Bone Marrow. ( 27913782 )
2016
50
Intravascular Large B Cell Lymphoma as a Rare Cause of Reversed Halo Sign: A Case Report. ( 27015192 )
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:

id 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:

id 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:

id 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
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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 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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