MCID: INT221
MIFTS: 35

Intravascular Large B-Cell Lymphoma

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Intravascular Large B-Cell Lymphoma

MalaCards integrated aliases for Intravascular Large B-Cell Lymphoma:

Name: Intravascular Large B-Cell Lymphoma 58
Angioendotheliomatosis Proliferans Systemisata 58
Malignant Angioendotheliomatosis 58
Angiotropic Large Cell Lymphoma 58
Intravascular Lymphomatosis 58
Tappeiner-Pfleger Disease 58
Angioendotheliomatosis 71

Characteristics:

Orphanet epidemiological data:

58
intravascular large b-cell lymphoma
Inheritance: Not applicable;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 via Orphanet 33 C83.3
Orphanet 58 ORPHA98839
UMLS 71 C0334660

Summaries for Intravascular Large B-Cell Lymphoma

MalaCards based summary : Intravascular Large B-Cell Lymphoma, also known as angioendotheliomatosis proliferans systemisata, is related to central nervous system lymphoma and lymphosarcoma. An important gene associated with Intravascular Large B-Cell Lymphoma is BCL6 (BCL6 Transcription Repressor), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. Affiliated tissues include b cells, skin and lung, 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 308)
# Related Disease Score Top Affiliating Genes
1 central nervous system lymphoma 30.2 MYD88 BCL6
2 lymphosarcoma 30.2 BCL6 BCL2
3 plasmacytoma 30.0 CD79B CD5 B2M
4 acquired immunodeficiency syndrome 29.9 IL2RB BCL6 B2M
5 reticulosarcoma 29.8 BCL6 BCL2 B2M
6 b-cell lymphoma 29.8 MYD88 MME CD79B CD5 BCL6 BCL2
7 prostate lymphoma 29.8 MME CD5 BCL6
8 lung lymphoma 29.8 MME CD5 BCL6
9 adult lymphoma 29.8 MME CD5 BCL6
10 primary central nervous system lymphoma 29.6 MYD88 BCL6 BCL2
11 lymphoplasmacytic lymphoma 29.6 MYD88 CD5
12 immune deficiency disease 29.6 IL2RB CD5 BCL6 B2M
13 lymphoma 29.5 MYD88 MME CD5 BCL6 BCL2 B2M
14 mature b-cell neoplasm 29.4 MME CD5 BCL6 B2M
15 cll/sll 29.4 MME CD79B CD5 BCL6
16 diffuse large b-cell lymphoma 29.3 MYD88 MME CD79B CD5 BCL6 B2M
17 peripheral t-cell lymphoma 29.3 MME IL2RB CD5 BCL6
18 lymphoblastic lymphoma 29.2 MME KMT2A CD5 BCL6
19 follicular lymphoma 29.2 MME CD5 BCL6 BCL2
20 mantle cell lymphoma 29.1 MME CD5 BCL6 BCL2 B2M
21 testicular lymphoma 28.9 MYD88 MME CD79B BCL6
22 burkitt lymphoma 28.8 MME BCL6 BCL2
23 systemic lupus erythematosus 28.4 MYD88 IL2RB CD5 BCL6 B2M
24 lymphoma, mucosa-associated lymphoid type 27.4 MYD88 MME CD79B CD5 BCL6 BCL2
25 lymphoma, non-hodgkin, familial 27.3 MYD88 MME KMT2A IL2RB CD79B CD5
26 leukemia, chronic lymphocytic 27.1 MYD88 MME KMT2A CD79B CD5 BCL6
27 lymphoma, hodgkin, classic 10.7
28 pancytopenia 10.6
29 splenomegaly 10.6
30 thrombocytopenia 10.6
31 hemangioma 10.5
32 cauda equina syndrome 10.5
33 vasculitis 10.4
34 respiratory failure 10.4
35 neutropenia 10.4
36 panniculitis 10.4
37 lung disease 10.4
38 lymphoproliferative syndrome 10.3
39 cholecystitis 10.3
40 interstitial lung disease 10.3
41 hypopituitarism 10.3
42 hemophagocytic lymphohistiocytosis 10.3
43 telangiectasis 10.3
44 dementia 10.3
45 senile angioma 10.3
46 hemolytic anemia 10.3
47 marek disease 10.3
48 paresthesia 10.3
49 rare tumor 10.3
50 eye lymphoma 10.2 CD5 BCL6

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.97 B2M BCL2 BCL6 CD5 CD79B IL2RB
2 immune system MP:0005387 9.97 B2M BCL2 BCL6 CD5 CD79B IL2RB
3 integument MP:0010771 9.8 B2M BCL2 BCL6 CD5 IL2RB KMT2A
4 liver/biliary system MP:0005370 9.63 B2M BCL6 IL2RB KMT2A MME MYD88
5 no phenotypic analysis MP:0003012 9.35 BCL6 CD5 CD79B KMT2A MYD88
6 reproductive system MP:0005389 9.1 B2M BCL2 BCL6 IL2RB KMT2A MYD88

Drugs & Therapeutics for Intravascular Large B-Cell Lymphoma

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase 1 Multidose Study to Evaluate the 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:

40
B Cells, Skin, Lung, Bone, Bone Marrow, Brain, Prostate

Publications for Intravascular Large B-Cell Lymphoma

Articles related to Intravascular Large B-Cell Lymphoma:

(show top 50) (show all 496)
# Title Authors PMID Year
1
Concurrent mycosis fungoides and intravascular large B-cell lymphoma in a single patient. 61
32087036 2020
2
Intravascular large B-cell lymphoma: a forgotten stroke "mimic". 61
30848425 2020
3
EBV-positive intravascular large B-cell lymphoma of the liver: a case report and literature review. 61
32513269 2020
4
Post-mortem diagnosis of intravascular large B-cell lymphoma. 61
32485117 2020
5
Multiple Cerebral Hemorrhages With Microbleeds in Intravascular Large B-Cell Lymphoma. 61
32249205 2020
6
Intravascular large B-cell lymphoma presenting with acute encephalopathy. 61
32437565 2020
7
PD-L1 (SP142) expression in neoplastic cells predicts a poor prognosis for patients with intravascular large B-cell lymphoma treated with rituximab-based multi-agent chemotherapy. 61
32367674 2020
8
The broad and challenging landscape of extranodal lymphoproliferations. 61
31758317 2020
9
Large B-cell lymphoma of the uvea: Histopathologic variants and clinicopathologic correlation. 61
31676312 2020
10
CNS lymphoma, the Irish experience: A retrospective review of neuropathologically confirmed cases over 10 years. 61
32301694 2020
11
Intravascular Large B-cell lymphoma: A case series and review of literatures. 61
32344119 2020
12
Aggressive B-cell lymphomas with primary bone marrow presentation. 61
32324290 2020
13
"Hot Lung" Sign in Pulmonary Intravascular Large B-Cell Lymphoma on 18F-FDG PET/CT. 61
31977490 2020
14
Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone combined with high-dose methotrexate plus intrathecal chemotherapy for newly diagnosed intravascular large B-cell lymphoma (PRIMEUR-IVL): a multicentre, single-arm, phase 2 trial. 61
32171071 2020
15
Skin biopsies using dermoscopy for earlier diagnosis of intravascular large B-cell lymphoma. 61
32275085 2020
16
Intravascular large B-cell lymphoma presenting as a nondescript macular eruption. 61
32297309 2020
17
Usefulness of dermoscopy-aided skin biopsy for clinically recognizable intravascular large B-cell lymphoma: prediction of histopathological localization of occlusive vessels based on dermoscopic findings. 61
32301717 2020
18
[Intravascular large B-cell lymphoma with multiple intracranial hemorrhages diagnosed by brain biopsy]. 61
32101844 2020
19
Successful Intensive Care Treatment of Severe Lactic Acidosis and Tumor Lysis Syndrome Related to Intravascular Lymphoma. 61
31308316 2020
20
Dermoscopy of primary cutaneous intravascular large B-cell lymphoma. 61
31419326 2020
21
Intravascular Large B-Cell Lymphoma: Clinical and Histopathologic Findings. 61
32226333 2020
22
Pulmonary Intravascular Large B-cell Lymphoma in a Patient Administered Methotrexate for Rheumatoid Arthritis. 61
31619597 2020
23
Demyelinating Neuropathy Due to Intravascular Large B-cell Lymphoma. 61
31588080 2020
24
Prognostic Value of Concurrent Expression of C-MYC and BCL2 in Intravascular Large B-Cell Lymphoma: A 10-Year Retrospective Study. 61
32566654 2020
25
Primary Cutaneous B-Cell Lymphomas: An Update. 61
32528871 2020
26
Unusual and Fatal Case of an Undiagnosed Intravascular Large B-cell Lymphoma: The Oncologist's Great Imitator†. 61
31361917 2020
27
Primary lung intravascular large B-Cell lymphoma clinically mimicking sarcoidosis: A rare case report and review of literature. 61
31921597 2020
28
Non-mycosis fungoides cutaneous lymphomas in a referral center in Taiwan: A retrospective case series and literature review. 61
31978091 2020
29
Intravascular Lymphoma with Progressive CNS Hemorrhage and Multiple Dissections. 61
32547798 2020
30
Intravascular Large B-cell Lymphoma Associated with Systemic and Central Nervous System Hemophagocytic Lymphohistiocytosis: A Case Report. 61
31852057 2020
31
Diffuse large B-cell lymphoma variants: an update. 61
31735345 2020
32
Intravascular large B cell lymphoma presenting as an isolated cauda equina-conus medullaris syndrome-a case report and review of the literature of the neuroimaging features at onset. 61
31950461 2020
33
A case report of primary prostate intravascular large B cell lymphoma presenting as prostatic hyperplasia. 61
31852153 2019
34
Intravascular large B-cell lymphoma presenting with altered mental status: A case report. 61
31890649 2019
35
Asian variant of intravascular large B-cell lymphoma: a comparison of clinical features based on involvement of the central nervous system. 61
31752477 2019
36
Diagnosis of intravascular large B cell lymphoma: novel insights into clinicopathological features from 42 patients at a single institution over 20 years. 61
31267524 2019
37
Synchronous Ductal Carcinoma In Situ and Intravascular Large B-cell Lymphoma of the Breast. 61
28582274 2019
38
Pulmonary intravascular large B-cell lymphoma accompanying synchronous primary pulmonary adenocarcinoma and benign interstitial lesions. 61
31391407 2019
39
Li-Fraumeni syndrome presenting as precursor B lymphoblastic leukemia mimicking intravascular large B-cell lymphoma. 61
31537539 2019
40
Intravascular Large B-Cell Lymphoma Involving Multinodular Goiter and Mimicking Carcinoma. 61
31566036 2019
41
Intravascular Large B-Cell Lymphoma with Initial Presentation in Skin: A Rare Mimicker of Cellulitis. 61
30998920 2019
42
Outcome of patients receiving consolidative autologous peripheral blood stem cell transplantation in the frontline treatment of intravascular large B-cell lymphoma: Adult Lymphoma Working Group of the Japan Society for Hematopoietic Cell Transplantation. 61
30809035 2019
43
Vasculitis on brain angiography is not always vasculitis: intravascular large B-cell lymphoma mimicking central nervous system vasculitis. 61
31451471 2019
44
A study of PD-L1 expression in intravascular large B cell lymphoma: correlation with clinical and pathological features. 61
30938862 2019
45
Intravascular Large B-Cell Lymphoma Presenting with Hypoxemia without any Abnormalities on Standard Imaging Studies. 61
31409762 2019
46
Multicentre retrospective study of intravascular large B-cell lymphoma treated at academic institutions within the United States. 61
31044423 2019
47
Incisional random skin biopsy, not punch biopsy, is an appropriate method for diagnosis of intravascular large B-cell lymphoma: a clinicopathological study of 25 patients. 61
30609011 2019
48
Timely Diagnosis and Successful Treatment of Intravascular Large B-Cell Lymphoma in an Elderly Patient with Poor Performance Status. 61
31155607 2019
49
Nephrotic syndrome without kidney injury revealing intravascular large B cell lymphoma. 61
31196971 2019
50
CD30 expression in a case of intravascular large B-cell lymphoma, cutaneous variant. 61
30779218 2019

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:

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 8.92 IL2RB CD79B CD5 B2M

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

# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.67 MYD88 CD79B BCL6 B2M
2 negative regulation of apoptotic process GO:0043066 9.46 MYD88 IL2RB BCL6 BCL2
3 positive regulation of B cell proliferation GO:0030890 9.32 BCL6 BCL2
4 T cell differentiation in thymus GO:0033077 9.26 BCL2 B2M
5 B cell differentiation GO:0030183 9.13 CD79B BCL6 BCL2
6 cytokine-mediated signaling pathway GO:0019221 8.92 MYD88 IL2RB BCL6 BCL2

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

# Name GO ID Score Top Affiliating Genes
1 protein homodimerization activity GO:0042803 9.26 MME KMT2A BCL2 B2M
2 identical protein binding GO:0042802 9.1 MYD88 KMT2A CD79B BCL6 BCL2 B2M

Sources for Intravascular Large B-Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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