MCID: TCL012
MIFTS: 38

T-Cell/histiocyte Rich Large B Cell Lymphoma

Categories: Rare diseases, Blood diseases, Immune diseases

Aliases & Classifications for T-Cell/histiocyte Rich Large B Cell Lymphoma

MalaCards integrated aliases for T-Cell/histiocyte Rich Large B Cell Lymphoma:

Name: T-Cell/histiocyte Rich Large B Cell Lymphoma 53 59
T-Cell/histiocyte Rich Large B-Cell Lymphoma 73
Thrlbcl 59

Characteristics:

Orphanet epidemiological data:

59
t-cell/histiocyte rich large b cell lymphoma
Inheritance: Multigenic/multifactorial,Not applicable;

Classifications:



External Ids:

Orphanet 59 ORPHA300857
UMLS via Orphanet 74 C1321547
ICD10 via Orphanet 34 C83.3
UMLS 73 C1321547

Summaries for T-Cell/histiocyte Rich Large B Cell Lymphoma

NIH Rare Diseases : 53 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 300857Disease definitionT-cell/histiocyte rich large B cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL; see this term), mainly affecting middle-aged men and often not being discovered until an advanced disease stage, with involvement of the spleen, liver and bone marrow occurring at a greater frequency than in DLBCL. It is often difficult to diagnose due to its similarity with other lymphoid diseases such as classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma (see these terms) and has an aggressive clinical course.Visit the Orphanet disease page for more resources.

MalaCards based summary : T-Cell/histiocyte Rich Large B Cell Lymphoma, also known as t-cell/histiocyte rich large b-cell lymphoma, is related to b-cell lymphomas and hemophagocytic lymphohistiocytosis. An important gene associated with T-Cell/histiocyte Rich Large B Cell Lymphoma is BCL6 (B Cell CLL/Lymphoma 6), and among its related pathways/superpathways are B cell receptor signaling pathway (KEGG) and Complement and coagulation cascades. The drugs nivolumab and Immunoglobulin G have been mentioned in the context of this disorder. Affiliated tissues include b cells, t cells and bone, and related phenotypes are hematopoietic system and immune system

Related Diseases for T-Cell/histiocyte Rich Large B Cell Lymphoma

Diseases related to T-Cell/histiocyte Rich Large B Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 70)
# Related Disease Score Top Affiliating Genes
1 b-cell lymphomas 31.3 BCL6 MME TNFRSF8
2 hemophagocytic lymphohistiocytosis 30.5 CR1 CR2 IFNG
3 diffuse large b-cell lymphoma 30.4 BCL6 MME TNFRSF8
4 lymphoma 11.0
5 primary mediastinal b-cell lymphoma 10.8 BCL6 SLC22A2
6 gallbladder lymphoma 10.7 BCL6 MME
7 heart lymphoma 10.7 BCL6 MME
8 intravascular large b-cell lymphoma 10.7 BCL6 MME
9 testicular lymphoma 10.7 BCL6 MME
10 angioimmunoblastic t-cell lymphoma 10.7 BCL6 MME
11 lymphoma, hodgkin, classic 10.6
12 central nervous system leukemia 10.6 CR1 MME
13 nodular lymphocyte predominant hodgkin lymphoma 10.6
14 balloon cell malignant melanoma 10.4 CD68 MME
15 bladder lymphoma 10.4 CR2 MME
16 ovarian lymphoma 10.4 CR2 MME
17 parapharyngeal meningioma 10.4 CR1 CR2
18 interdigitating dendritic cell sarcoma 10.4 CR1 CR2
19 marginal zone b-cell lymphoma 10.4 BCL6 MME
20 histiocytic and dendritic cell cancer 10.4 CR1 CR2
21 castleman disease 10.3 CR1 CR2
22 mature b-cell neoplasm 10.3 BCL6 MME
23 meningeal melanocytoma 10.3 CD68 MME
24 malignant leydig cell tumor 10.3 MME TNFRSF8
25 colon lymphoma 10.3 BCL6 TNFRSF8
26 proliferative glomerulonephritis 10.2 CR1 IFNG
27 spleen cancer 10.2 CD68 CR2
28 lateral medullary syndrome 10.2 CR1 CR2
29 epidural neoplasm 10.2 BCL6 TNFRSF8
30 lymphatic system cancer 10.1 BCL6 TNFRSF8
31 follicular lymphoma 1 10.1 BCL6 IGH
32 pure red-cell aplasia 10.1
33 central nervous system lymphoma 10.1
34 duodenal obstruction 10.1
35 lymphopenia 10.1
36 thyroiditis 10.1
37 duodenitis 10.1
38 lupus erythematosus 10.1
39 systemic lupus erythematosus 10.1
40 primary central nervous system lymphoma 10.1
41 anemia, autoimmune hemolytic 10.1
42 hemolytic anemia 10.1
43 lymphomatoid granulomatosis 10.1
44 mediastinal malignant lymphoma 10.1 BCL6 TNFRSF8
45 follicular dendritic cell sarcoma 10.1 CR1 CR2
46 sternum cancer 10.1 CD68 TNFRSF8
47 burkitt lymphoma 10.0 BCL6 CR2 MME
48 langerhans cell sarcoma 10.0 CR1 CR2 VSIG4
49 hodgkin's lymphoma, lymphocytic depletion 10.0 CD68 TNFRSF8
50 pediatric lymphoma 10.0 MME TNFRSF8

Graphical network of the top 20 diseases related to T-Cell/histiocyte Rich Large B Cell Lymphoma:



Diseases related to T-Cell/histiocyte Rich Large B Cell Lymphoma

Symptoms & Phenotypes for T-Cell/histiocyte Rich Large B Cell Lymphoma

MGI Mouse Phenotypes related to T-Cell/histiocyte Rich Large B Cell Lymphoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.5 BCL6 CD68 CR2 IFNG POU2F2 TNFRSF8
2 immune system MP:0005387 9.23 CD68 CR2 IFNG MME POU2F2 TNFRSF8

Drugs & Therapeutics for T-Cell/histiocyte Rich Large B Cell Lymphoma

Drugs for T-Cell/histiocyte Rich Large B Cell Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 17)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
nivolumab Approved Phase 2 946414-94-4
2 Immunoglobulin G Phase 2
3 Antibodies Phase 2
4 Immunoglobulins Phase 2
5 Antibodies, Monoclonal Phase 2
6
Vidarabine Approved, Investigational Phase 1 24356-66-9 32326 21704
7
Fludarabine Approved Phase 1 21679-14-1, 75607-67-9 30751
8
Cyclophosphamide Approved, Investigational Phase 1 50-18-0, 6055-19-2 2907
9 Alkylating Agents Phase 1
10 Vidarabine Phosphate Phase 1
11 Immunosuppressive Agents Phase 1
12 Anti-Infective Agents Phase 1
13 Antirheumatic Agents Phase 1
14 Antimetabolites Phase 1
15 Antiviral Agents Phase 1
16 Antimetabolites, Antineoplastic Phase 1
17 Antineoplastic Agents, Alkylating Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas Recruiting NCT03038672 Phase 2 Varlilumab
2 CD19/CD22 Chimeric Antigen Receptor T Cells and Chemotherapy in Treating Patients With Recurrent or Refractory CD19 Positive Diffuse Large B-Cell Lymphoma or B Acute Lymphoblastic Leukemia Recruiting NCT03233854 Phase 1 Cyclophosphamide;Fludarabine Phosphate
3 Study to Evaluate Safety and Tolerability of XmAb13676 in Patients With CD20-expressing Hematologic Malignancies Recruiting NCT02924402 Phase 1

Search NIH Clinical Center for T-Cell/histiocyte Rich Large B Cell Lymphoma

Genetic Tests for T-Cell/histiocyte Rich Large B Cell Lymphoma

Anatomical Context for T-Cell/histiocyte Rich Large B Cell Lymphoma

MalaCards organs/tissues related to T-Cell/histiocyte Rich Large B Cell Lymphoma:

41
B Cells, T Cells, Bone, Bone Marrow, Spleen, Liver, Thymus

Publications for T-Cell/histiocyte Rich Large B Cell Lymphoma

Articles related to T-Cell/histiocyte Rich Large B Cell Lymphoma:

(show all 48)
# Title Authors Year
1
T cell/histiocyte-rich large B cell lymphoma: incidence, demographic disparities, and long-term outcomes. ( 29845599 )
2018
2
Hemophagocytic Lymphohistiocytosis Secondary to T Cell/Histiocyte-rich Large B Cell Lymphoma in an Adolescent Male. ( 29187030 )
2018
3
T-cell/histiocyte-rich large B-cell lymphoma of stomach: a rare variant of gastric B cell lymphoma. ( 29694656 )
2017
4
T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease. ( 28912991 )
2017
5
Predominance of CD4+ T Cells in T-Cell/Histiocyte-Rich Large B-Cell Lymphoma and Identification of a Subset of Patients With Peripheral B-Cell Lymphopenia. ( 28575178 )
2017
6
T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Presented as T-Lymphoid Hyperplasia Involving the Central Nervous System. ( 28451478 )
2017
7
T-cell/histiocyte-rich large B-cell lymphoma of stomach. ( 27427148 )
2016
8
T Cell/Histiocyte-Rich Large B Cell Lymphoma of the Thymus: A Diagnostic Pitfall. ( 26904321 )
2016
9
From a pathologist's point of view: Histiocytic cells in Hodgkin lymphoma and T cell/histiocyte rich large B cell lymphoma. ( 26596262 )
2015
10
A T-cell/histiocyte-rich large B-cell lymphoma involving only the bone marrow. ( 26770945 )
2015
11
Array comparative genomic hybridization reveals similarities between nodular lymphocyte predominant Hodgkin lymphoma and T cell/histiocyte rich large B cell lymphoma. ( 25644177 )
2015
12
Reactive T cells by flow cytometry distinguish Hodgkin lymphomas from T cell/histiocyte-rich large B cell lymphoma. ( 26084540 )
2015
13
T-cell/histiocyte-rich large B-cell lymphoma in pediatric patients: an under-recognized entity? ( 25696014 )
2015
14
T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Presenting as a Primary Central Nervous System Lymphoma. ( 26788280 )
2015
15
Hemophagocytic lymphohistiocytosis secondary to T-cell/histiocyte-rich large B-cell lymphoma. ( 24955327 )
2014
16
T-cell/histiocyte-rich large B-cell lymphoma with zonal expansion of fibroblastic reticulum cells and infiltration by a subpopulation of myeloperoxidase positive histiocytes. ( 25042387 )
2014
17
Diagnosis of primary pulmonary T- cell/histiocyte-rich large B cell lymphoma with tissue eosinophilia via clinicopathological observation and molecular assay. ( 25273521 )
2014
18
Anti-erythropoietin receptor antibody-associated pure red cell aplasia accompanied by Coombs-negative autoimmune hemolytic anemia in a patient with T cell/histiocyte-rich large B cell lymphoma. ( 25096221 )
2014
19
Fatal necrotizing angiotropic Epstein-Barr virus-negative large B-cell lymphoma: a case report with unusual clinicopathological features in-between lymphomatoid granulomatosis and T-cell/histiocyte-rich large B-cell lymphoma. ( 25546693 )
2014
20
Upregulation of innate immune responses in a T cell/histiocyte-rich large B cell lymphoma patient with significant autoimmune disorders mimicking systemic lupus erythematosus. ( 23743539 )
2014
21
Nodular Lymphocyte Predominant Hodgkin Lymphoma versus T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: A Diagnostic Challenge. ( 25110597 )
2014
22
T-cell/histiocyte-rich large B-cell lymphoma--transformation of nodular lymphocyte predominant Hodgkin lymphoma in the bone marrow. ( 24483596 )
2014
23
Matched-Pair Analysis Comparing the Outcomes of T Cell/Histiocyte-Rich Large B Cell Lymphoma and Diffuse Large B Cell Lymphoma in Patients Treated with Rituximab-CHOP. ( 24192382 )
2013
24
First case of T-cell/histiocyte-rich-large B-cell lymphoma presenting with duodenal obstruction. ( 24345670 )
2013
25
T-cell/histiocyte-rich large B-cell lymphoma of the thyroid. ( 23298413 )
2013
26
Primary bone marrow T-cell/histiocyte-rich large B-cell lymphoma: a diagnostic challenge. ( 23562349 )
2013
27
Does functional imaging distinguish nodular lymphocyte-predominant hodgkin lymphoma from T-cell/histiocyte-rich large B-cell lymphoma? ( 23773450 )
2013
28
Nodular lymphocyte predominant hodgkin lymphoma and T cell/histiocyte rich large B cell lymphoma - endpoints of a spectrum of one disease? ( 24244368 )
2013
29
Macrophages in T cell/histiocyte rich large B cell lymphoma strongly express metal-binding proteins and show a bi-activated phenotype. ( 23686423 )
2013
30
Multiple cytokine- and chemokine-producing T-cell/histiocyte-rich large B-cell lymphoma. ( 23278285 )
2013
31
Extranodal T-cell/histiocyte-rich large B-cell lymphoma presenting primarily on the skin. ( 22948794 )
2012
32
T cell/histiocyte-rich large B-cell lymphoma: an update on its biology and classification. ( 22081105 )
2011
33
Primary T cell/histiocyte-rich large B-cell lymphoma of the orbit. ( 20562665 )
2011
34
T-cell/histiocyte-rich large B-cell lymphoma. ( 20207840 )
2010
35
T-cell/histiocyte-rich large B-cell lymphoma shows transcriptional features suggestive of a tolerogenic host immune response. ( 19797726 )
2010
36
Nodular lymphocyte-predominant Hodgkin lymphoma and T cell histiocyte-rich large B cell lymphoma: diagnosis in two monozygotic twins. ( 20653789 )
2010
37
Splenic micronodular T-cell/histiocyte-rich large B-cell lymphoma: effect of prior corticosteroid therapy. ( 19763612 )
2009
38
Nodular lymphocyte-predominant hodgkin lymphoma or T-cell/histiocyte rich large B-cell lymphoma: the problem in "grey zone" lymphomas. ( 18784812 )
2008
39
Morphologic, immunphenotypic and clinical discriminators between T-cell/histiocyte-rich large B-cell lymphoma and lymphocyte-predominant Hodgkin lymphoma. ( 20063524 )
2008
40
Splenic micronodular T-cell/histiocyte-rich large B-cell lymphoma. ( 18620998 )
2008
41
T-cell/histiocyte-rich large B-cell lymphoma associated with a near-tetraploid karyotype and complex genetic abnormalities. ( 16856972 )
2006
42
T-cell/histiocyte-rich large B-cell lymphoma displays a heterogeneity similar to diffuse large B-cell lymphoma: a clinicopathologic, immunohistochemical, and molecular study of 30 cases. ( 15894921 )
2005
43
Pediatric EBV-positive T-cell/histiocyte-rich large B-cell lymphoma with clonal cells in the bone marrow without overt involvement. ( 15621841 )
2005
44
Micronodular T-cell/histiocyte-rich large B-cell lymphoma of the spleen: histology, immunophenotype, and differential diagnosis. ( 12826882 )
2003
45
T-cell/histiocyte-rich large B-cell lymphoma: a heterogeneous entity with derivation from germinal center B cells. ( 12409722 )
2002
46
T-cell/histiocyte-rich large B-cell lymphoma: a distinct clinicopathologic entity. ( 11870169 )
2002
47
CD5+ T-cell/histiocyte-rich large B-cell lymphoma. ( 12379751 )
2002
48
T-cell/histiocyte-rich large B-cell lymphoma is a disseminated aggressive neoplasm: differential diagnosis from Hodgkin's lymphoma. ( 12207783 )
2002

Variations for T-Cell/histiocyte Rich Large B Cell Lymphoma

Expression for T-Cell/histiocyte Rich Large B Cell Lymphoma

Search GEO for disease gene expression data for T-Cell/histiocyte Rich Large B Cell Lymphoma.

Pathways for T-Cell/histiocyte Rich Large B Cell Lymphoma

GO Terms for T-Cell/histiocyte Rich Large B Cell Lymphoma

Biological processes related to T-Cell/histiocyte Rich Large B Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein import into nucleus, translocation GO:0000060 9.16 BCL6 IFNG
2 regulation of regulatory T cell differentiation GO:0045589 8.96 CR1 IFNG
3 complement receptor mediated signaling pathway GO:0002430 8.62 CR1 CR2

Sources for T-Cell/histiocyte Rich Large B Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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