MCID: INT221
MIFTS: 36

Intravascular Large B-Cell Lymphoma malady

Categories: Blood diseases, Rare diseases, Immune diseases

Aliases & Classifications for Intravascular Large B-Cell Lymphoma

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Sources:
30ICD10 via Orphanet, 53Orphanet, 67UMLS
See all MalaCards sources

Aliases & Descriptions for Intravascular Large B-Cell Lymphoma:

Name: Intravascular Large B-Cell Lymphoma 53
Angioendotheliomatosis Proliferans Systemisata 53
Malignant Angioendotheliomatosis 53
Angiotropic Large Cell Lymphoma 53
 
Intravascular Lymphomatosis 53
Tappeiner-Pfleger Disease 53
Angioendotheliomatosis 67

Characteristics:

Orphanet epidemiological data:

53

Classifications:



External Ids:

Orphanet53 ORPHA98839
ICD10 via Orphanet30 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 hemolytic anemia 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 IL2 signaling events mediated by PI3K and IL-15 Signaling Pathways and their Primary Biological Effects in Different Immune Cell Types. Affiliated tissues include b cells, bone and skin, and related mouse phenotypes are liver/biliary system and integument.

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 91)
idRelated DiseaseScoreTop Affiliating Genes
1hemolytic anemia28.9B2M, BCL6, CD5, MME
2b-cell lymphomas11.0
3lymphoma11.0
4lung cancer susceptibility 410.3BCL2, BCL6
5primary cutaneous unspecified peripheral t-cell lymphoma10.3BCL2, BCL6
6prostatitis10.2
7congenital myasthenic syndrome10.2BCL6, CD5
8solitary osseous plasmacytoma10.2B2M, BCL2
9cerebritis10.2
10monkeypox10.2BCL2, CD5
11inherited metabolic disorder10.2BCL6, MME
12testis refractory cancer10.2BCL2, CD5
13lymphoma, small cleaved-cell, diffuse10.1CD5, KMT2A
14diffuse large b-cell lymphoma10.1
15hemangioma10.1
16trachea squamous cell carcinoma10.1CD5, MME
17teebi kaurah syndrome10.1BCL6, MME
18chronic inflammatory demyelinating polyneuritis10.1BCL6, CD5
19cholecystitis10.1
20senile angioma10.1
21hypopituitarism10.1
22chromosome 13q14 deletion syndrome10.1BCL2, CD5
23polyembryoma of the ovary10.1BCL6, CD5
24multicentric reticulohistiocytosis10.0CD5, MME
25lymphoma, large-cell, immunoblastic10.0BCL2, KMT2A
26gray zone lymphoma10.0CD5, MME
27glomerulonephritis10.0
28hepatitis10.0
29thrombocytopenia10.0
30colitis10.0
31pancytopenia10.0
32dementia10.0
33panniculitis10.0
34membranoproliferative glomerulonephritis10.0
35interstitial lung disease10.0
36lung disease10.0
37meningitis10.0
38uterine body mixed cancer10.0BCL2, MME
39acanthoma10.0CD5, MME
40acral lentiginous melanoma9.9CD5, MME
41non-gestational choriocarcinoma9.9BCL2, MME
42lacrimal passage granuloma9.9CD5, MME
43primary angiitis of the central nervous system9.9
44rheumatoid arthritis9.8
45gastrointestinal stromal tumor9.8
46systemic lupus erythematosus9.8
47breast cancer9.8
48hodgkin lymphoma9.8
49myelodysplastic syndrome9.8
50aspergillosis9.8

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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Interventional clinical trials:

idNameStatusNCT IDPhase
1Study to Evaluate Safety and Tolerability of XmAb13676 in Patients With CD20-expressing Hematologic MalignanciesRecruitingNCT02924402Phase 1

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:

35
B cells, Bone, Skin, Lung, Bone marrow, Prostate, Uterus

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:

40
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.4B2M, BCL6, IL2RB, KMT2A, MME
2MP:00107718.4B2M, BCL2, IL2RB, KMT2A, MME
3MP:00053978.4B2M, BCL2, BCL6, CD5, IL2RB, KMT2A
4MP:00053898.1B2M, BCL2, BCL6, IL2RB, KMT2A
5MP:00053877.5B2M, BCL2, BCL6, CD5, IL2RB, KMT2A

Publications for Intravascular Large B-Cell Lymphoma

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Articles related to Intravascular Large B-Cell Lymphoma:

(show top 50)    (show all 242)
idTitleAuthorsYear
1
Intravascular large b-cell lymphoma diagnosed at autopsy: A case report. (27773147)
2016
2
Glomerular infiltration by intravascular large B-cell lymphoma. (27391281)
2016
3
Characteristics of Intravascular Large B-Cell Lymphoma Limited to the Glomerular Capillaries: A Case Report. (26351627)
2015
4
Intravascular large B-cell lymphoma secondary to lymphoplasmacytic lymphoma: a case report and review of literature with clonality analysis. (26045864)
2015
5
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
6
Synchronous invasive ductal carcinoma and intravascular large B-cell lymphoma of the breast: a case report and review of the literature. (24708809)
2014
7
Intravascular large B-cell lymphoma presenting as cauda equina syndrome and showing aberrant cytokeratin expression: a diagnostic challenge. (24614706)
2014
8
Intravascular Large B-Cell Lymphoma Diagnosed on Prostate Biopsy: A Case Report. (25541658)
2014
9
Intravascular large B-cell lymphoma manifesting as cholecystitis: report of an Asian variant showing gain of chromosome 18 with concurrent deletion of chromosome 6q. (25550870)
2014
10
Picture in clinical hematology no. 77. Intravascular large B-cell lymphoma]. (25501403)
2014
11
Intravascular large B-cell lymphoma confirmed by lung biopsy. (25337283)
2014
12
Intravascular large B-cell lymphoma confirmed by brain biopsy: a case report. (23420723)
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 presenting with fulminant pseudomembranous colitis. (23611409)
2013
16
Pitfalls in the diagnosis of intravascular large B-cell lymphoma. (23795899)
2013
17
Intravascular large B-cell lymphoma with hemophagocytic syndrome (Asian variant) in a Caucasian patient. (22808298)
2012
18
Indication for random skin biopsy for the diagnosis of intravascular large B cell lymphoma. (22414723)
2012
19
Intravascular large B-cell lymphoma. (22372911)
2012
20
A fatal case of hemoperitoneum after ultrasound-guided liver biopsy in a patient with intravascular large B-cell lymphoma. (21377915)
2011
21
An autopsy case of lymphomatosis cerebri showing pathological changes of intravascular large B-cell lymphoma in visceral organs. (21382094)
2011
22
Intravascular large B-cell lymphoma of the uterus: a case with favorable clinical outcome. (19948641)
2011
23
Frequency of intravascular large B-cell lymphoma in Japan: study of the Osaka Lymphoma Study Group. (21481222)
2011
24
Disseminated intravascular large B cell lymphoma with slowly decreasing high-density lipoprotein cholesterol. (21119558)
2011
25
Intravascular large B-cell lymphoma originating in the left epididymis. (20387066)
2011
26
18F-FDG PET/CT in a case of intravascular large B-cell lymphoma. (20607536)
2010
27
Intravascular large B-cell lymphoma, an exclusively small vessel disease? A case report and review of literature. (20542564)
2010
28
Fluorodeoxyglucose-PET/CT for diagnosis of intravascular large B-cell lymphoma. (20675506)
2010
29
Involvement of bone marrow with intravascular large B-cell lymphoma. (20231812)
2010
30
Intravascular large B-cell lymphoma of the skin: typical clinical manifestations and a favourable response to rituximab-containing therapy. (19590171)
2009
31
Intravascular large B cell lymphoma: proposed of the strategy for early diagnosis and treatment of patients with rapid deteriorating condition. (19363707)
2009
32
Gallium scan-prompted skin biopsy revealed intravascular large B-cell lymphoma in a patient who presented with fever of unknown origin. (19387217)
2009
33
Intravascular large B-cell lymphoma presenting in subcutaneous fat tissue and simulating panniculitis clinically. (20415677)
2009
34
The addition of rituximab to anthracycline-based chemotherapy significantly improves outcome in 'Western' patients with intravascular large B-cell lymphoma. (18699850)
2008
35
Sustained remission after rituximab-containing chemotherapy for intravascular large B-cell lymphoma. (18434690)
2008
36
Prolonged survival using anti-CD20 combined chemotherapy in primary prostatic intravascular large B-cell lymphoma. (18493873)
2008
37
Can rituximab change the usually dismal prognosis of patients with intravascular large B-cell lymphoma? (18838697)
2008
38
Asian variant of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy]. (17313078)
2007
39
Renal intravascular large B-cell lymphoma localized only within peritubular capillaries. Report of a case. (17527039)
2007
40
Intravascular large B-cell lymphoma presenting with neurological syndromes: clinicopathologic study. (17702500)
2007
41
Leukemic and meningeal relapse of CD5+ intravascular large B-cell lymphoma with down-modulation of CD20 after rituximab therapy. (16867907)
2006
42
Intravascular large B-cell lymphoma involving hemangiomas: an unusual presentation of a rare neoplasm. (15803190)
2005
43
Detection of t(14;18) translocation in a case of intravascular large B-cell lymphoma: a germinal centre cell origin in a subset of these lymphomas? (15810961)
2005
44
Intravascular large B-Cell lymphoma with bone marrow involvement and superior sagittal sinus thrombosis: report of a case successfully treated with a CHOP/rituximab combination regimen. (15989707)
2005
45
Primary bilateral adrenal intravascular large B-cell lymphoma associated with adrenal failure. (12879956)
2003
46
Intravascular large B-cell lymphoma presenting as cutaneous panniculitis. (14677722)
2003
47
Prevalence of intravascular large B-cell lymphoma with bone marrow involvement at initial presentation. (12627851)
2003
48
Intravascular large B-cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem. (12196378)
2002
49
Myeloperoxidase-positive intravascular large B-cell lymphoma. (11419984)
2001
50
Unusual sites of involvement by hematologic malignancies. Case 1. Intravascular large B-cell lymphoma presenting with CNS symptoms. (11579120)
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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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:00098979.0B2M, CD5, IL2RB

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

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of B cell proliferationGO:003089010.2BCL2, BCL6
2B cell differentiationGO:003018310.1BCL2, BCL6
3cell morphogenesisGO:000090210.0BCL2, BCL6
4homeostasis of number of cells within a tissueGO:00488739.9BCL2, KMT2A
5spleen developmentGO:00485369.9BCL2, KMT2A
6T cell differentiation in thymusGO:00330779.9B2M, BCL2
7post-embryonic developmentGO:00097919.8BCL2, KMT2A
8negative regulation of cell proliferationGO:00082859.1BCL2, BCL6, KMT2A
9negative regulation of apoptotic processGO:00430668.9BCL2, BCL6, IL2RB

Sources for Intravascular Large B-Cell Lymphoma

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
26GTR
27HGMD
28HMDB
29ICD10
30ICD10 via Orphanet
31ICD9CM
32IUPHAR
33KEGG
36MedGen
38MeSH
39MESH via Orphanet
40MGI
43NCI
44NCIt
45NDF-RT
48NINDS
49Novoseek
51OMIM
52OMIM via Orphanet
56PubMed
57QIAGEN
62SNOMED-CT via Orphanet
66Tumor Gene Family of Databases
67UMLS
68UMLS via Orphanet