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

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Sources:
31ICD10 via Orphanet, 54Orphanet, 68UMLS
See all MalaCards sources

Aliases & Descriptions for Intravascular Large B-Cell Lymphoma:

Name: Intravascular Large B-Cell Lymphoma 54
Angioendotheliomatosis Proliferans Systemisata 54
Malignant Angioendotheliomatosis 54
Angiotropic Large Cell Lymphoma 54
 
Intravascular Lymphomatosis 54
Tappeiner-Pfleger Disease 54
Angioendotheliomatosis 68

Characteristics:

Orphanet epidemiological data:

54

Classifications:



External Ids:

Orphanet54 ORPHA98839
ICD10 via Orphanet31 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 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 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 hematopoietic 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 91)
idRelated DiseaseScoreTop Affiliating Genes
1b-cell lymphomas11.0
2lymphoma11.0
3prostatitis10.2
4cerebritis10.2
5rubella panencephalitis10.2BCL2, BCL6
6penile disease10.1BCL6, CD5
7ethmoid sinus inverted papilloma10.1BCL6, MME
8ichthyosis, congenital, autosomal recessive 1010.1BCL2, BCL6
9trigeminal nerve neoplasm10.1CD5, MME
10spinal cord intramedullary teratoma10.1BCL6, CD5
11diffuse large b-cell lymphoma10.1
12hemangioma10.1
13teratocarcinoma10.1BCL2, CD5
14schwannian stroma-rich and stroma-poor composite ganglioneuroblastoma10.1BCL2, CD5
15teebi naguib al awadi syndrome10.1BCL6, MME
16degenerative myopia10.1CD5, MME
17basal ganglia disease10.1BCL6, CD5
18pyloric antrum cancer10.1BCL2, BCL6
19acute allergic sanguinous otitis media10.1B2M, BCL2
20inclusion conjunctivitis10.1B2M, CD5
21childhood brain stem glioma10.1CD5, MME
22lymphomatoid granulomatosis10.1CD5, KMT2A
23cholecystitis10.1
24senile angioma10.1
25hypopituitarism10.1
26vaginal adenosarcoma10.1BCL2, MME
27anterior foramen magnum meningioma10.0B2M, BCL6
28henoch-schoenlein purpura10.0B2M, MME
29theileriasis10.0B2M, CD5
30lip cancer10.0B2M, BCL2, BCL6
31tall cell variant papillary carcinoma10.0B2M, BCL6, MME
32glomerulonephritis10.0
33hepatitis10.0
34thrombocytopenia10.0
35colitis10.0
36thrombosis10.0
37pancytopenia10.0
38dementia10.0
39panniculitis10.0
40membranoproliferative glomerulonephritis10.0
41interstitial lung disease10.0
42lung disease10.0
43meningitis10.0
44gingival recession9.9BCL2, BCL6, MME
45lymphoma, small cleaved-cell, diffuse9.9KMT2A, MME
46primary angiitis of the central nervous system9.9
47hyperaldosteronism, familial, type ii9.8B2M, CD5, IL2RB
48hereditary ataxia9.8B2M, CD5, IL2RB
49rheumatoid arthritis9.8
50gastrointestinal stromal tumor9.8

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

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MGI Mouse Phenotypes related to Intravascular Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.4B2M, BCL6, IL2RB, KMT2A, MME
2MP:00053978.2B2M, BCL2, BCL6, CD5, IL2RB, KMT2A
3MP:00107717.9B2M, BCL2, CD5, IL2RB, KMT2A, MME
4MP:00053897.8B2M, BCL2, BCL6, IL2RB, KMT2A
5MP:00053877.5B2M, BCL2, BCL6, CD5, IL2RB, KMT2A

Drugs & Therapeutics for Intravascular Large B-Cell Lymphoma

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

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
nivolumabapprovedPhase 2424946414-94-4
Synonyms:
BMS-936558
 
MDX-1106
ONO-4538
nivolumab
2ImmunoglobulinsPhase 26394
3Immunoglobulin GPhase 2275
4Antibodies, MonoclonalPhase 24039
5AntibodiesPhase 26394

Interventional clinical trials:

idNameStatusNCT IDPhase
1Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell LymphomasNot yet recruitingNCT03038672Phase 2
2Study 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:

36
B cells, Bone, Skin, Lung, Bone marrow, Prostate, Uterus

Publications for Intravascular Large B-Cell Lymphoma

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Articles related to Intravascular Large B-Cell Lymphoma:

(show top 50)    (show all 241)
idTitleAuthorsYear
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

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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 8)
idNameGO IDScoreTop Affiliating Genes
1B cell differentiationGO:003018310.4BCL2, BCL6
2cell morphogenesisGO:000090210.4BCL2, BCL6
3positive regulation of B cell proliferationGO:003089010.3BCL2, BCL6
4homeostasis of number of cells within a tissueGO:00488739.9BCL2, KMT2A
5post-embryonic developmentGO:00097919.7BCL2, KMT2A
6spleen developmentGO:00485369.6BCL2, KMT2A
7T cell differentiation in thymusGO:00330779.5B2M, BCL2
8negative regulation of cell proliferationGO:00082859.4BCL2, BCL6, KMT2A

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

idNameGO IDScoreTop Affiliating Genes
1identical protein bindingGO:00428028.3B2M, BCL2, BCL6, KMT2A

Sources for Intravascular Large B-Cell Lymphoma

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet