MCID: INT221
MIFTS: 46

Intravascular Large B-Cell Lymphoma malady

Blood diseases, Cancer diseases, Rare diseases, Immune diseases categories

Aliases & Classifications for Intravascular Large B-Cell Lymphoma

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Sources:
47Orphanet, 60UMLS, 26ICD10 via Orphanet
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Intravascular Large B-Cell Lymphoma, Aliases & Descriptions:

Name: Intravascular Large B-Cell Lymphoma 47
Angioendotheliomatosis Proliferans Systemisata 47
Malignant Angioendotheliomatosis 47
Angiotropic Large Cell Lymphoma 47
 
Intravascular Lymphomatosis 47
Tappeiner-Pfleger Disease 47
Angioendotheliomatosis 60


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases, Rare diseases
Anatomical: Blood diseases, Immune diseases


External Ids:

Orphanet47 98839
ICD10 via Orphanet26 C83.3

Summaries for Intravascular Large B-Cell Lymphoma

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MalaCards based summary: Intravascular Large B-Cell Lymphoma, also known as angioendotheliomatosis proliferans systemisata, is related to pancytopenia 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 are Inflammatory Response Pathway and B Cell Receptor Signaling Pathway. The compounds lentinan and mycophenolate have been mentioned in the context of this disorder. Affiliated tissues include b cells, bone and lung, and related mouse phenotypes are tumorigenesis and liver/biliary system.

Related Diseases for Intravascular Large B-Cell Lymphoma

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

(show top 50)    (show all 144)
idRelated DiseaseScoreTop Affiliating Genes
1pancytopenia31.2IL2RA, KMT2A
2b-cell lymphomas30.7CCND1, ACPP, BCL6, BCL2, CD5, CXCR3
3breast cancer30.7CCND1, BCL2
4systemic lupus erythematosus30.6MPO, CXCR3, IL2RA, IL2RB
5plasmacytoma30.4B2M, TNFRSF8, BCL6
6anaplastic large cell lymphoma30.1TNFRSF8, CXCR3, IL2RA, IL2RB, BCL6
7hodgkin lymphoma29.8TNFRSF8, KMT2A, IL2RA, IL2RB, BCL6, BCL2
8prostatitis10.8
9cerebritis10.7
10hemangioma10.6
11diffuse large b-cell lymphoma10.6
12senile angioma10.6
13cholecystitis10.6
14hepatitis10.5
15thrombocytopenia10.5
16dementia10.5
17hypopituitarism10.5
18interstitial lung disease10.5
19meningitis10.5
20panniculitis10.5
21proteinuria10.5
22malaria10.4IL2RB
23oral candidiasis10.4B2M
24uveitis10.4IL2RA, CXCR3
25intermediate uveitis10.4IL2RA, CXCR3
26posterior uveitis10.4IL2RB, IL2RA
27lymphomatoid papulosis10.4CXCR3, TNFRSF8
28benign breast phyllodes tumor10.4MME, ACPP
29malignant histiocytosis10.3
30histiocytosis10.3
31pfeiffer syndrome10.3TNFRSF8, IL2RB
32aspergillosis10.3
33hemolytic anemia10.3
34myoma10.3
35splenic infarction10.3
36hypercalcemia10.3
37cauda equina syndrome10.3
38choroiditis10.3
39sagittal sinus thrombosis10.3
40colitis10.3
41ischemia10.3
42lupus erythematosus10.3
43mononeuropathy10.3
44myopathy10.3
45thyroiditis10.3
46ring chromosome 1110.3
47syndrome of inappropriate antidiuretic hormone10.3
48aneurysm10.3
49blindness10.3
50alk-positive anaplastic large cell lymphoma10.3

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



Diseases related to intravascular large b-cell lymphoma

Symptoms for Intravascular Large B-Cell Lymphoma

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Drugs & Therapeutics for Intravascular Large B-Cell Lymphoma

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Drug clinical trials:

Search ClinicalTrials for Intravascular Large B-Cell Lymphoma

Search NIH Clinical Center for Intravascular Large B-Cell Lymphoma

Genetic Tests for Intravascular Large B-Cell Lymphoma

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Anatomical Context for Intravascular Large B-Cell Lymphoma

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

31
B cells, Bone, Lung, Skin, Bone marrow, Prostate, Uterus, Liver, Breast, Brain, Colon, Kidney, Spleen, Thyroid

Animal Models for Intravascular Large B-Cell Lymphoma or affiliated genes

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

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020067.9B2M, KMT2A, CXCR3, CCND1, BCL2
2MP:00053707.8B2M, KMT2A, MME, IL2RB, BCL6
3MP:00053897.5B2M, KMT2A, CCND1, IL2RB, BCL6, BCL2
4MP:00107717.4B2M, KMT2A, MME, CCND1, ACPP, IL2RB
5MP:00053857.3B2M, MPO, KMT2A, CXCR3, CCND1, BCL6
6MP:00053787.3BCL2, BCL6, IL2RB, IL2RA, CCND1, KMT2A
7MP:00053766.9B2M, MPO, KMT2A, MME, CXCR3, CCND1
8MP:00107686.6B2M, MPO, KMT2A, CXCR3, CCND1, IL2RA
9MP:00053975.9KMT2A, MPO, TNFRSF8, B2M, CXCR3, CCND1
10MP:00053875.5CD5, B2M, TNFRSF8, MPO, KMT2A, MME

Publications for Intravascular Large B-Cell Lymphoma

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Articles related to Intravascular Large B-Cell Lymphoma:

(show top 50)    (show all 196)
idTitleAuthorsYear
1
Antemortem diagnosis with multiple random skin biopsies and transbronchial lung biopsy in a patient with intravascular large B-cell lymphoma, the so-called Asian variant lymphoma. (24632902)
2014
2
Synchronous invasive ductal carcinoma and intravascular large B-cell lymphoma of the breast: a case report and review of the literature. (24708809)
2014
3
Tandem triplication of the BCL2 gene in CD5-positive intravascular large B cell lymphoma with bone marrow involvement. (24577512)
2014
4
Simultaneous thrombosis of the mesenteric artery and vein as a novel clinical manifestation of intravascular large B-cell lymphoma. (24525901)
2014
5
Benefits of skin biopsy of senile hemangioma in intravascular large B-cell lymphoma: A case report and review of the literature. (24932279)
2014
6
Intravascular large B-cell lymphoma presenting as cauda equina syndrome and showing aberrant cytokeratin expression: a diagnostic challenge. (24614706)
2014
7
Intravascular large B-cell lymphoma confirmed by brain biopsy: a case report. (23420723)
2013
8
Intravascular large B-cell lymphoma presenting with anasarca-type edema and acute renal failure. (23885711)
2013
9
Liver dysfunction and thrombocytopenia diagnosed as intravascular large B-cell lymphoma using a timely and accurate transjugular liver biopsy. (23994980)
2013
10
Intravascular large B-cell lymphoma presenting as acute hemorrhagic cerebral infarct with delirium. (23884226)
2013
11
Mild proteinuria in a patient with glomerularlimited intravascular large B-cell lymphoma. (22541680)
2013
12
Generalized telangiectasia revealing intravascular large B-cell lymphoma. (23406023)
2013
13
Intravascular large B cell lymphoma presenting as cholecystitis: diagnostic challenges persist. (24288109)
2013
14
SIADH and partial hypopituitarism in a patient with intravascular large B-cell lymphoma: a rare cause of a common presentation. (23362070)
2013
15
Intravascular large B-cell lymphoma with hemophagocytic syndrome (Asian variant) in a Caucasian patient. (22808298)
2012
16
Indication for random skin biopsy for the diagnosis of intravascular large B cell lymphoma. (22414723)
2012
17
A fatal case of hemoperitoneum after ultrasound-guided liver biopsy in a patient with intravascular large B-cell lymphoma. (21377915)
2011
18
Progressive multifocal cerebral infarction from intravascular large B cell lymphoma presenting in a man: a case report. (21251318)
2011
19
Haemophagocytosis-related intravascular large B-cell lymphoma associated with skin eruption. (21279301)
2011
20
Intravascular large B-cell lymphoma of the uterus: a case with favorable clinical outcome. (19948641)
2011
21
Frequency of intravascular large B-cell lymphoma in Japan: study of the Osaka Lymphoma Study Group. (21481222)
2011
22
18F-FDG PET/CT in a case of intravascular large B-cell lymphoma. (20607536)
2010
23
Intravascular large B-cell lymphoma, an exclusively small vessel disease? A case report and review of literature. (20542564)
2010
24
Central nervous system involvement in intravascular large B-cell lymphoma: a retrospective analysis of 109 patients. (20412122)
2010
25
Neurolymphomatosis involving the trigeminal nerve and deep peroneal nerve in a patient with relapsed intravascular large B-cell lymphoma. (20528901)
2010
26
Fluorodeoxyglucose-PET/CT for diagnosis of intravascular large B-cell lymphoma. (20675506)
2010
27
Intravascular large B cell lymphoma: an elusive cause of pyrexia of unknown origin diagnosed postmortem. (20868278)
2010
28
Intravascular large B-cell lymphoma within a subcutaneous cavernous haemangioma. (20735401)
2010
29
Intravascular large B-cell lymphoma of the skin: typical clinical manifestations and a favourable response to rituximab-containing therapy. (19590171)
2009
30
Intravascular large B cell lymphoma: proposed of the strategy for early diagnosis and treatment of patients with rapid deteriorating condition. (19363707)
2009
31
Gallium scan-prompted skin biopsy revealed intravascular large B-cell lymphoma in a patient who presented with fever of unknown origin. (19387217)
2009
32
Intravascular large B cell lymphoma: when lymphoma is suspected but routine diagnostic work-up is negative. (19883320)
2009
33
Intravascular large B-cell lymphoma: remission after rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy. (19632742)
2009
34
Intravascular large B-cell lymphoma presenting in subcutaneous fat tissue and simulating panniculitis clinically. (20415677)
2009
35
Analysis of CXCL9 and CXCR3 expression in a case of intravascular large B-cell lymphoma. (19766346)
2009
36
The addition of rituximab to anthracycline-based chemotherapy significantly improves outcome in 'Western' patients with intravascular large B-cell lymphoma. (18699850)
2008
37
Sustained remission after rituximab-containing chemotherapy for intravascular large B-cell lymphoma. (18434690)
2008
38
Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. (17577023)
2007
39
Intravascular large B-cell lymphoma presenting as cerebellar and cerebral infarction. (17502477)
2007
40
Asian variant of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy]. (17313078)
2007
41
Intravascular large B-cell lymphoma of the uterus presenting as fever of unknown origin (FUO) and revealed by FDG-PET. (17672184)
2007
42
Intravascular large B-cell lymphoma with multisystem involvement. (16966288)
2006
43
Intravascular large B-cell lymphoma associated with a near-tetraploid karyotype, rearrangement of BCL6, and a t(11;14)(q13;q32). (17116487)
2006
44
Primary bilateral adrenal intravascular large B-cell lymphoma associated with adrenal failure. (12879956)
2003
45
A case of intravascular large B-cell lymphoma with multiple organ involvement. (14739519)
2003
46
Intravascular large B-cell lymphoma presenting as cutaneous panniculitis. (14677722)
2003
47
Intravascular large B-cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem. (12196378)
2002
48
Myeloperoxidase-positive intravascular large B-cell lymphoma. (11419984)
2001
49
Unusual sites of involvement by hematologic malignancies. Case 1. Intravascular large B-cell lymphoma presenting with CNS symptoms. (11579120)
2001
50
Primary cutaneous large B-cell lymphoma of the leg relapsing as cutaneous intravascular large B-cell lymphoma. (11735727)
2001

Variations for Intravascular Large B-Cell Lymphoma

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Expression for genes affiliated with Intravascular Large B-Cell Lymphoma

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

Pathways for genes affiliated with Intravascular Large B-Cell Lymphoma

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

(show all 25)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8IL2RB, IL2RA
29.7BCL6, BCL2, CD5
3
Show member pathways
Immune response IL 22 signaling pathway58
G protein signaling N RAS regulation pathway58
9.5BCL2, IL2RB, IL2RA
4
Show member pathways
9.5BCL2, IL2RB, IL2RA
5
Show member pathways
IL-2 Signaling pathway36
9.5BCL2, IL2RB, IL2RA
6
Show member pathways
IL2 signaling events mediated by STAT536
9.5IL2RA, IL2RB, BCL2
79.4MME, IL2RA, CD5
89.3BCL2, BCL6, CCND1
9
Show member pathways
DNA damage response (only ATM dependent)36
Wnt Signaling Pathway and Pluripotency36
9.3CCND1, BCL6, BCL2
10
Show member pathways
Immune response IL 12 induced IFN gamma production58
9.2IL2RB, IL2RA, B2M
11
Show member pathways
Ras signaling in the CD4+ TCR pathway36
9.2IL2RB, IL2RA, B2M
12
Show member pathways
9.2B2M, TNFRSF8, BCL2
139.1IL2RB, IL2RA, CCND1
14
Show member pathways
Type III interferon signaling36
9.1CCND1, IL2RA, IL2RB
15
Show member pathways
IL-9 Signaling Pathway36
Development Thrombopoietin regulated cell processes58
IL-7 Signaling Pathway36
Immune response IL 9 signaling pathway58
9.1CCND1, IL2RA, IL2RB
169.1MPO, CCND1, BCL2
17
Show member pathways
9.0B2M, CCND1, IL2RA
188.9CCND1, IL2RA, IL2RB, BCL2
19
Show member pathways
8.9BCL2, IL2RB, IL2RA, CCND1
20
Show member pathways
8.6TNFRSF8, CXCR3, IL2RA, IL2RB, BCL2
21
Show member pathways
8.6TNFRSF8, CXCR3, IL2RA, IL2RB, BCL2
228.6MPO, KMT2A, IL2RB, BCL6
238.1BCL6, IL2RB, IL2RA, MME, MPO, B2M
24
Show member pathways
8.0TNFRSF8, CXCR3, CCND1, IL2RA, IL2RB, BCL2
25
Show member pathways
8.0TNFRSF8, CXCR3, CCND1, IL2RA, IL2RB, BCL2

Compounds for genes affiliated with Intravascular Large B-Cell Lymphoma

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

(show top 50)    (show all 121)
idCompoundScoreTop Affiliating Genes
1lentinan4310.1IL2RA, IL2RB
2mycophenolate4310.0IL2RB, IL2RA
3ricin439.7IL2RB, CD5, TNFRSF8
4il 10439.6IL2RA, IL2RB, CXCR3
52-chlorodeoxyadenosine439.6IL2RA, BCL2, B2M
6cuprophan439.6IL2RB, B2M, IL2RA
7ginsenoside439.6MME, CCND1, BCL2
8polysulfone439.6IL2RB, IL2RA, B2M
9infliximab43 49 1211.5IL2RB, CXCR3, IL2RA
102,5-oligoadenylate439.5B2M, IL2RA, IL2RB
11rituximab43 49 1211.5IL2RA, BCL2, B2M, BCL6
12ifn-alpha439.4CXCR3, IL2RB, IL2RA
13methacholine43 49 1211.4MME, IL2RB, MPO
14methimazole43 2 1211.4IL2RA, B2M, MPO
15neopterin439.3ACPP, B2M, IL2RA, IL2RB
16erythromycin43 2 49 1212.3IL2RA, MPO, IL2RB
17pentoxifylline43 1210.2MPO, IL2RB, IL2RA
18tacrolimus43 49 1211.1CCND1, IL2RB, IL2RA, CXCR3
19adriamycin439.0CCND1, B2M, MME, BCL2
20chloride438.9MME, B2M, IL2RB, MPO
21histamine43 28 2410.9CXCR3, MME, MPO, IL2RA, IL2RB
22prednisolone43 28 1210.8MPO, IL2RB, IL2RA, KMT2A
23formaldehyde43 249.8B2M, MPO, CCND1, IL2RA
24n acetylcysteine438.8IL2RB, BCL2, CCND1, MPO, IL2RA
25cyclophosphamide43 49 1210.7IL2RA, IL2RB, B2M, BCL2, BCL6, MPO
26cyclosporin a43 28 5910.6MME, IL2RA, B2M, IL2RB, TNFRSF8
27progesterone43 28 59 24 1212.6MME, CCND1, BCL2, IL2RB, ACPP, CXCR3
28lactate438.6IL2RB, MME, ACPP, MPO, B2M, BCL6
29phosphatidylinositol438.5MME, CXCR3, CCND1, IL2RA, IL2RB, BCL2
30pge2438.5IL2RB, IL2RA, MME, MPO, B2M, CXCR3
31cycloheximide438.5IL2RB, B2M, BCL6, MME, CCND1, BCL2
32indomethacin43 28 59 1211.4B2M, IL2RA, MPO, MME
33aspartate438.4KMT2A, IL2RA, IL2RB, ACPP, B2M
34testosterone43 59 24 1211.3MME, CCND1, ACPP, B2M, IL2RA
35arginine438.3CXCR3, ACPP, IL2RA, IL2RB, MME, KMT2A
36creatinine438.2B2M, MME, CXCR3, ACPP, IL2RB, MPO
37vincristine43 49 1210.1KMT2A, IL2RA, BCL6, B2M, BCL2, CCND1
38cisplatin43 49 59 1211.0IL2RB, IL2RA, ACPP, MPO, BCL2, CCND1
39steroid438.0CXCR3, CCND1, ACPP, IL2RA, IL2RB, MME
40genistein43 28 59 2 24 1212.9IL2RB, B2M, BCL2, KMT2A, CCND1, IL2RA
41zinc43 248.9MME, TNFRSF8, BCL6, KMT2A, IL2RB, IL2RA
42calcium43 49 24 1210.7CXCR3, IL2RB, CD5, MME, ACPP, B2M
43doxorubicin43 49 129.6B2M, KMT2A, MME, CCND1, IL2RA, BCL2
44thymidine43 248.5B2M, MME, TNFRSF8, IL2RB, IL2RA, ACPP
45vegf437.3ACPP, BCL2, B2M, MPO, MME, CXCR3
46retinoic acid43 248.2BCL6, B2M, MPO, KMT2A, MME, BCL2
47estrogen437.0B2M, BCL2, IL2RA, BCL6, ACPP, CCND1
48paraffin436.9BCL6, ACPP, CD5, BCL2, IL2RA, B2M
49tyrosine436.7MME, KMT2A, CD5, CXCR3, BCL6, ACPP
50serine436.1MPO, KMT2A, CXCR3, CCND1, ACPP, IL2RB

GO Terms for genes affiliated with Intravascular Large B-Cell Lymphoma

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

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:00098978.5CD5, IL2RB, IL2RA, CXCR3, B2M
2extracellular vesicular exosomeGO:00700628.3ACPP, MME, MPO, TNFRSF8, B2M
3plasma membraneGO:00058867.1B2M, TNFRSF8, MME, CXCR3, ACPP, IL2RA

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

idNameGO IDScoreTop Affiliating Genes
1positive regulation of B cell proliferationGO:003089010.0BCL2, BCL6
2response to iron ionGO:00100399.7BCL2, CCND1
3T cell differentiation in thymusGO:00330779.4BCL2, B2M
4cellular response to DNA damage stimulusGO:00069749.3BCL2, BCL6, CCND1
5apoptotic processGO:00069158.6KMT2A, CXCR3, IL2RA, BCL2

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

idNameGO IDScoreTop Affiliating Genes
1interleukin-2 receptor activityGO:00049119.7IL2RB, IL2RA
2interleukin-2 bindingGO:00199769.5IL2RB, IL2RA
3identical protein bindingGO:00428028.6BCL2, ACPP, KMT2A, B2M

Products for genes affiliated with Intravascular Large B-Cell Lymphoma

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Sources for Intravascular Large B-Cell Lymphoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet