MCID: INT221
MIFTS: 38

Intravascular Large B-Cell Lymphoma malady

Categories: Blood diseases, Rare diseases, Immune diseases

Aliases & Classifications for Intravascular Large B-Cell Lymphoma

Aliases & Descriptions 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 lymphoma and b-cell lymphomas. 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, 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 91)
id Related Disease Score Top Affiliating Genes
1 lymphoma 11.0
2 b-cell lymphomas 11.0
3 prostatitis 10.2
4 cerebritis 10.2
5 rubella panencephalitis 10.2 BCL2 BCL6
6 penile disease 10.1 BCL6 CD5
7 ethmoid sinus inverted papilloma 10.1 BCL6 MME
8 ichthyosis, congenital, autosomal recessive 10 10.1 BCL2 BCL6
9 trigeminal nerve neoplasm 10.1 CD5 MME
10 spinal cord intramedullary teratoma 10.1 BCL6 CD5
11 diffuse large b-cell lymphoma 10.1
12 hemangioma 10.1
13 teratocarcinoma 10.1 BCL2 CD5
14 schwannian stroma-rich and stroma-poor composite ganglioneuroblastoma 10.1 BCL2 CD5
15 teebi naguib al awadi syndrome 10.1 BCL6 MME
16 degenerative myopia 10.1 CD5 MME
17 basal ganglia disease 10.1 BCL6 CD5
18 pyloric antrum cancer 10.1 BCL2 BCL6
19 acute allergic sanguinous otitis media 10.1 B2M BCL2
20 inclusion conjunctivitis 10.1 B2M CD5
21 childhood brain stem glioma 10.1 CD5 MME
22 lymphomatoid granulomatosis 10.1 CD5 KMT2A
23 cholecystitis 10.1
24 senile angioma 10.1
25 hypopituitarism 10.1
26 vaginal adenosarcoma 10.1 BCL2 MME
27 anterior foramen magnum meningioma 10.0 B2M BCL6
28 henoch-schoenlein purpura 10.0 B2M MME
29 theileriasis 10.0 B2M CD5
30 lip cancer 10.0 B2M BCL2 BCL6
31 tall cell variant papillary carcinoma 10.0 B2M BCL6 MME
32 hepatitis 10.0
33 meningitis 10.0
34 thrombocytopenia 10.0
35 membranoproliferative glomerulonephritis 10.0
36 interstitial lung disease 10.0
37 colitis 10.0
38 thrombosis 10.0
39 pancytopenia 10.0
40 dementia 10.0
41 glomerulonephritis 10.0
42 lung disease 10.0
43 panniculitis 10.0
44 gingival recession 9.9 BCL2 BCL6 MME
45 lymphoma, small cleaved-cell, diffuse 9.9 KMT2A MME
46 primary angiitis of the central nervous system 9.9
47 hyperaldosteronism, familial, type ii 9.8 B2M CD5 IL2RB
48 hereditary ataxia 9.8 B2M CD5 IL2RB
49 splenic infarction 9.8
50 rheumatoid arthritis 9.8

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 IL2RB KMT2A MME B2M BCL2 BCL6
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 IL2RB KMT2A B2M BCL2 BCL6

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 Immunoglobulin G Phase 2
3 Immunoglobulins Phase 2
4 Antibodies, Monoclonal Phase 2
5 Antibodies Phase 2

Interventional clinical trials:


id Name Status NCT ID Phase
1 Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas Not yet recruiting NCT03038672 Phase 2
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 241)
id Title Authors Year
1
A case of intravascular large B cell lymphoma: New clinical and immunohistochemical findings. ( 27090763 )
2016
2
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
3
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
4
Intravascular large B-cell lymphoma: masquerade as membranoproliferative glomerulonephritis. ( 27312458 )
2016
5
Seemed Like Cellulitis: Intravascular Large B-Cell Lymphoma. ( 27038674 )
2016
6
Glomerular infiltration by intravascular large B-cell lymphoma. ( 27391281 )
2016
7
Reversible Hypopituitarism Associated with Intravascular Large B-Cell Lymphoma: Case Report of Successful Immunochemotherapy. ( 26936510 )
2016
8
Three Cases of Intravascular Large B-Cell Lymphoma Detected in a Biopsy of Skin Lesions. ( 26871422 )
2016
9
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
10
Methotrexate-associated Intravascular Large B-cell Lymphoma in a Patient with Rheumatoid Arthritis. ( 27301524 )
2016
11
Intestinal Intravascular Large B-cell Lymphoma Mimicking Ulcerative Colitis with Secondary Membranoproliferative Glomerulonephritis. ( 27580553 )
2016
12
Cutaneous variant of intravascular large B-cell lymphoma in a Japanese patient: An occult lesion localized in a solitary angiolipoma. ( 27422850 )
2016
13
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
14
Intravascular Large B Cell Lymphoma as a Rare Cause of Reversed Halo Sign: A Case Report. ( 27015192 )
2016
15
Intravascular Large B-Cell Lymphoma Resembling Myelodysplastic Syndrome in the Bone Marrow. ( 27913782 )
2016
16
Intravascular large B-cell lymphoma with a high titer of proteinase-3-anti-neutrophil cytoplasmic antibody mimicking granulomatosis with polyangiitis. ( 27398728 )
2016
17
Rituximab monotherapy to treat intravascular large B-cell lymphoma manifesting as pruritic calf patch in an elderly female. ( 27098895 )
2016
18
Isolated Intravascular Large B-cell Lymphoma Presenting with Diffuse Ground Glass Pulmonary Infiltrates. ( 27408440 )
2016
19
Intravascular large B cell lymphoma presenting as transient erythema nodosum-like lesions and fever. ( 27687069 )
2016
20
Intravascular large B-cell lymphoma in a kidney biopsy. ( 28092895 )
2016
21
Intravascular Large B-Cell Lymphoma Coexisting with an Ovarian Carcinoma. ( 27334860 )
2016
22
Development and analysis of patient-derived xenograft mouse models in intravascular large B-cell lymphoma. ( 27001523 )
2016
23
Autologous hematopoietic stem cell transplantation for intravascular large B-cell lymphoma: the European Society for Blood and Marrow Transplantation experience. ( 27991887 )
2016
24
Neurologic manifestations of intravascular large B-cell lymphoma. ( 26918203 )
2016
25
Intravascular large b-cell lymphoma diagnosed at autopsy: A case report. ( 27773147 )
2016
26
Intense focal pituitary FDG uptake due to intravascular large B-cell lymphoma in pyrexia of unknown origin. ( 27387341 )
2016
27
Early diagnosis of intravascular large B-cell lymphoma. ( 27879065 )
2016
28
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
29
High Frequency of Bone Marrow Involvement in Intravascular Large B-Cell Lymphoma: A Clinicopathological Study of 13 Cases in China. ( 27553679 )
2016
30
Dominant neurologic symptomatology in intravascular large B-cell lymphoma. ( 27546361 )
2016
31
A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure. ( 27777803 )
2016
32
An autopsy case of intravascular large B-cell lymphoma with subcortical U-fiber sparing and unique lymphocyte markers. ( 27653906 )
2016
33
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
34
Circulating lymphoma cells in intravascular large B-cell lymphoma. ( 27657358 )
2016
35
Spontaneous Regression of Intravascular Large B-Cell Lymphoma and Apoptosis of Lymphoma Cells: A Case Report. ( 26763363 )
2015
36
Imaging findings for intravascular large B-cell lymphoma of the liver. ( 26523272 )
2015
37
Intravascular large B cell lymphoma presenting in the lung: the diagnostic value of transbronchial cryobiopsy. ( 25591148 )
2015
38
Heparin-responsive angiopathy in the central nervous system caused by intravascular large B-cell lymphoma. ( 25829082 )
2015
39
Useful Strategy of Pulmonary Microvascular Cytology in the Early Diagnosis of Intravascular Large B-cell Lymphoma in a Patient with Hypoxemia: A Case Report and Literature Review. ( 26027996 )
2015
40
Subacute encephalopathy after high-dose methotrexate as prophylaxis for central nervous system relapse in a patient with intravascular large B-cell lymphoma. ( 25876788 )
2015
41
Characteristics of Intravascular Large B-Cell Lymphoma Limited to the Glomerular Capillaries: A Case Report. ( 26351627 )
2015
42
Intravascular large B cell lymphoma with haemophagocytic syndrome: a double lethal masquerade. ( 26648194 )
2015
43
Random skin biopsy of patients with intravascular large B-cell lymphoma associated with thrombocytopenia and coagulation abnormalities: proposal of a modified biopsy method. ( 25557352 )
2015
44
Intravascular large B-cell lymphoma involving gastrointestinal stromal tumor: a case report and literature review. ( 26670158 )
2015
45
Intravascular large B-cell lymphoma secondary to lymphoplasmacytic lymphoma: a case report and review of literature with clonality analysis. ( 26045864 )
2015
46
Intravascular Large B-Cell Lymphoma Complicated by Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis that was Successfully Treated with Rituximab-Containing Chemotherapy. ( 26106006 )
2015
47
Ground-glass opacities and a solitary nodule on chest in intravascular large B-cell lymphoma. ( 26392859 )
2015
48
One Patient, Two Uncommon B-Cell Neoplasms: Solitary Plasmacytoma following Complete Remission from Intravascular Large B-Cell Lymphoma Involving Central Nervous System. ( 24715915 )
2014
49
Early diagnosis of intravascular large B-cell lymphoma: clues from routine blood smear morphologic findings. ( 25051078 )
2014
50
Intravascular large B cell lymphoma. ( 24596677 )
2014

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 Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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