MCID: TCL012
MIFTS: 39

T-Cell/histiocyte Rich Large B Cell Lymphoma

Categories: Blood diseases, Immune diseases, Rare 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 54 60
T-Cell/histiocyte Rich Large B-Cell Lymphoma 74
Thrlbcl 60

Characteristics:

Orphanet epidemiological data:

60
t-cell/histiocyte rich large b cell lymphoma
Inheritance: Multigenic/multifactorial,Not applicable;

Classifications:



External Ids:

ICD10 via Orphanet 35 C83.3
UMLS via Orphanet 75 C1321547
Orphanet 60 ORPHA300857
UMLS 74 C1321547

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

NIH Rare Diseases : 54 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 lymphoma, hodgkin, classic and hemophagocytic lymphohistiocytosis. An important gene associated with T-Cell/histiocyte Rich Large B Cell Lymphoma is BCL6 (BCL6 Transcription Repressor), and among its related pathways/superpathways are B cell receptor signaling pathway (KEGG) and Complement and coagulation cascades. The drugs Doxorubicin and rituximab have been mentioned in the context of this disorder. Affiliated tissues include b cells, t cells and spleen, 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 76)
# Related Disease Score Top Affiliating Genes
1 lymphoma, hodgkin, classic 31.3 BCL6 MME
2 hemophagocytic lymphohistiocytosis 31.1 CR1 CR2 IFNG
3 diffuse large b-cell lymphoma 30.6 BCL6 MME TNFRSF8
4 b-cell lymphomas 30.4 BCL6 MME TNFRSF8
5 lymphoma 11.1
6 nodular lymphocyte predominant hodgkin lymphoma 10.7
7 rapidly involuting congenital hemangioma 10.7
8 duodenal obstruction 10.4
9 parapharyngeal meningioma 10.4 CR1 CR2
10 hodgkin's lymphoma, lymphocytic-histiocytic predominance 10.3 BCL6 CR2
11 interdigitating dendritic cell sarcoma 10.3 CR1 CR2
12 heart lymphoma 10.3 BCL6 MME
13 intravascular large b-cell lymphoma 10.3 BCL6 MME
14 central nervous system leukemia 10.3 CR1 MME
15 histiocytic and dendritic cell cancer 10.3 CR1 CR2
16 testicular lymphoma 10.3 BCL6 MME
17 multicentric castleman disease 10.3 BCL6 IFNG
18 lateral medullary syndrome 10.3 CR1 CR2
19 bladder lymphoma 10.3 CR2 MME
20 ovarian lymphoma 10.3 CR2 MME
21 central nervous system hematologic cancer 10.3 BCL6 MME
22 systemic lupus erythematosus 10.3
23 anemia, autoimmune hemolytic 10.3
24 anaplastic large cell lymphoma 10.3
25 hemolytic anemia 10.3
26 lymphomatoid granulomatosis 10.3
27 pure red-cell aplasia 10.3
28 central nervous system lymphoma 10.3
29 lymphopenia 10.3
30 lupus erythematosus 10.3
31 hypereosinophilic syndrome 10.3
32 primary central nervous system lymphoma 10.3
33 follicular dendritic cell sarcoma 10.2 CR1 CR2
34 smooth muscle tumor 10.2 CR2 MME
35 angioimmunoblastic t-cell lymphoma 10.2 BCL6 MME
36 proliferative glomerulonephritis 10.2 CR1 IFNG
37 spleen cancer 10.2 CD68 CR2
38 colon lymphoma 10.2 BCL6 TNFRSF8
39 epidural neoplasm 10.2 BCL6 TNFRSF8
40 balloon cell malignant melanoma 10.2 CD68 MME
41 marginal zone b-cell lymphoma 10.2 BCL6 MME
42 follicular lymphoma 1 10.2 BCL6 IGH
43 mature b-cell neoplasm 10.2 BCL6 MME
44 vulvitis 10.2 CR2 TNFRSF8
45 malignant leydig cell tumor 10.2 MME TNFRSF8
46 mediastinal malignant lymphoma 10.1 BCL6 TNFRSF8
47 acute salpingitis 10.1 CD68 MME
48 sarcomatoid squamous cell skin carcinoma 10.1 CD68 MME
49 meningeal melanocytoma 10.1 CD68 MME
50 sternum cancer 10.1 CD68 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:

47
# 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 BCL6 CD68 CR2 IFNG MME POU2F2

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 38)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 1, Phase 2 23214-92-8 31703
2
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
3
Prednisone Approved, Vet_approved Phase 1, Phase 2 53-03-2 5865
4
Vincristine Approved, Investigational Phase 1, Phase 2 2068-78-2, 57-22-7 5978
5
Cyclophosphamide Approved, Investigational Phase 1, Phase 2 50-18-0, 6055-19-2 2907
6
nivolumab Approved Phase 1, Phase 2,Phase 2 946414-94-4
7
Doxil Approved June 1999 Phase 1, Phase 2 31703
8
Cortisone Experimental Phase 1, Phase 2 53-06-5 222786
9
Emodepside Investigational, Vet_approved Phase 1, Phase 2 155030-63-0
10 Antineoplastic Agents, Alkylating Phase 1, Phase 2
11 Antimitotic Agents Phase 1, Phase 2
12 Hormone Antagonists Phase 1, Phase 2
13 Topoisomerase Inhibitors Phase 1, Phase 2
14 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 1, Phase 2
15 Antineoplastic Agents, Hormonal Phase 1, Phase 2
16 Antineoplastic Agents, Immunological Phase 1, Phase 2,Phase 2
17 Immunologic Factors Phase 1, Phase 2,Phase 2
18 Antibodies, Monoclonal Phase 1, Phase 2,Phase 2
19 Immunoglobulins Phase 1, Phase 2,Phase 2
20 glucocorticoids Phase 1, Phase 2
21 Anti-Bacterial Agents Phase 1, Phase 2
22 Immunosuppressive Agents Phase 1, Phase 2
23 Anti-Inflammatory Agents Phase 1, Phase 2
24 Antirheumatic Agents Phase 1, Phase 2
25 Alkylating Agents Phase 1, Phase 2
26 Antibodies Phase 1, Phase 2,Phase 2
27 Antibiotics, Antitubercular Phase 1, Phase 2
28 Hormones Phase 1, Phase 2
29 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
30 Immunoglobulin G Phase 2
31
Fludarabine Approved Phase 1 75607-67-9, 21679-14-1 30751
32
Vidarabine Approved, Investigational Phase 1 24356-66-9 21704 32326
33
Tyrosine Approved, Investigational, Nutraceutical Phase 1 60-18-4 6057
34 Antiviral Agents Phase 1
35 Antimetabolites, Antineoplastic Phase 1
36 Vidarabine Phosphate Phase 1
37 Anti-Infective Agents Phase 1
38 Antimetabolites Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Nivolumab and Combination Chemotherapy in Treating Participants With Diffuse Large B-Cell Lymphoma Recruiting NCT03704714 Phase 1, Phase 2 Cyclophosphamide;Doxorubicin Hydrochloride;Prednisone;Vincristine Sulfate
2 Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas Recruiting NCT03038672 Phase 2 Varlilumab
3 Combination Chemotherapy and TAK-659 as Front-Line Treatment in Treating Patients With High-Risk Diffuse Large B Cell Lymphoma Recruiting NCT03742258 Phase 1 Cyclophosphamide;Doxorubicin Hydrochloride;Prednisone;Spleen Tyrosine Kinase Inhibitor TAK-659;Vincristine Sulfate
4 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
5 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:

42
B Cells, T Cells, Spleen, Bone Marrow, Skin, Thyroid, Thymus

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

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

(show top 50) (show all 55)
# Title Authors Year
1
Validation of CD137 Immunohistochemical Stain on Paraffin-Embedded Tissue as a Marker to Facilitate Distinction Between Classic Hodgkin Lymphoma, Nodular Lymphocyte-Predominant Hodgkin Lymphoma, T-Cell/Histiocyte-Rich Large B-Cell Lymphoma, and Anaplastic Large Cell Lymphoma. ( 30816829 )
2019
2
JUNB, DUSP2, SGK1, SOCS1 and CREBBP are frequently mutated in T-cell/histiocyte-rich large B-cell lymphoma. ( 30213827 )
2019
3
T cell/histiocyte-rich large B cell lymphoma: incidence, demographic disparities, and long-term outcomes. ( 29845599 )
2019
4
A Case of Spontaneously Improving Secondary Hemophagocytic Lymphohistiocytosis in an Adult Associated with T-Cell Histiocyte-Rich Large B-Cell Lymphoma. ( 29682376 )
2018
5
Hemophagocytic Lymphohistiocytosis Secondary to T Cell/Histiocyte-rich Large B Cell Lymphoma in an Adolescent Male. ( 29187030 )
2018
6
T-cell/histiocyte-rich large B-cell lymphoma in a child: A case report and review of literature. ( 29988902 )
2018
7
T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: Report of the First Case in the Mandible. ( 30019325 )
2018
8
T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease. ( 28912991 )
2017
9
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
10
T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Presented as T-Lymphoid Hyperplasia Involving the Central Nervous System. ( 28451478 )
2017
11
T-cell/histiocyte-rich large B-cell lymphoma of stomach: a rare variant of gastric B cell lymphoma. ( 29694656 )
2017
12
Reactive T cells by flow cytometry distinguish Hodgkin lymphomas from T cell/histiocyte-rich large B cell lymphoma. ( 26084540 )
2016
13
T Cell/Histiocyte-Rich Large B Cell Lymphoma of the Thymus: A Diagnostic Pitfall. ( 26904321 )
2016
14
T-cell/histiocyte-rich large B-cell lymphoma of stomach. ( 27427148 )
2016
15
Rare thymic malignancy of B-cell origin - T-cell÷histiocyte-rich large B-cell lymphoma. ( 28002527 )
2016
16
T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Presenting as a Primary Central Nervous System Lymphoma. ( 26788280 )
2015
17
T-cell/histiocyte-rich large B-cell lymphoma in pediatric patients: an under-recognized entity? ( 25696014 )
2015
18
From a pathologist's point of view: Histiocytic cells in Hodgkin lymphoma and T cell/histiocyte rich large B cell lymphoma. ( 26596262 )
2015
19
A T-cell/histiocyte-rich large B-cell lymphoma involving only the bone marrow. ( 26770945 )
2015
20
Array comparative genomic hybridization reveals similarities between nodular lymphocyte predominant Hodgkin lymphoma and T cell/histiocyte rich large B cell lymphoma. ( 25644177 )
2015
21
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 )
2014
22
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
23
Hemophagocytic lymphohistiocytosis secondary to T-cell/histiocyte-rich large B-cell lymphoma. ( 24955327 )
2014
24
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
25
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
26
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
27
Diagnosis of primary pulmonary T- cell/histiocyte-rich large B cell lymphoma with tissue eosinophilia via clinicopathological observation and molecular assay. ( 25273521 )
2014
28
Nodular Lymphocyte Predominant Hodgkin Lymphoma versus T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: A Diagnostic Challenge. ( 25110597 )
2014
29
T-cell/histiocyte-rich large B-cell lymphoma--transformation of nodular lymphocyte predominant Hodgkin lymphoma in the bone marrow. ( 24483596 )
2014
30
First case of T-cell/histiocyte-rich-large B-cell lymphoma presenting with duodenal obstruction. ( 24345670 )
2013
31
First case of T-cell/histiocyte-rich-large B-cell lymphoma presenting with duodenal obstruction. ( 28156233 )
2013
32
Primary bone marrow T-cell/histiocyte-rich large B-cell lymphoma: a diagnostic challenge. ( 23562349 )
2013
33
Macrophages in T cell/histiocyte rich large B cell lymphoma strongly express metal-binding proteins and show a bi-activated phenotype. ( 23686423 )
2013
34
T-cell/histiocyte-rich large B-cell lymphoma of the thyroid. ( 23298413 )
2013
35
Multiple cytokine- and chemokine-producing T-cell/histiocyte-rich large B-cell lymphoma. ( 23278285 )
2013
36
Nodular lymphocyte predominant hodgkin lymphoma and T cell/histiocyte rich large B cell lymphoma--endpoints of a spectrum of one disease? ( 24244368 )
2013
37
Does functional imaging distinguish nodular lymphocyte-predominant hodgkin lymphoma from T-cell/histiocyte-rich large B-cell lymphoma? ( 23773450 )
2013
38
Extranodal T-cell/histiocyte-rich large B-cell lymphoma presenting primarily on the skin. ( 22948794 )
2012
39
T cell/histiocyte-rich large B-cell lymphoma: an update on its biology and classification. ( 22081105 )
2011
40
Primary T cell/histiocyte-rich large B-cell lymphoma of the orbit. ( 20562665 )
2011
41
Nodular lymphocyte-predominant Hodgkin lymphoma and T cell histiocyte-rich large B cell lymphoma: diagnosis in two monozygotic twins. ( 20653789 )
2010
42
T-cell/histiocyte-rich large B-cell lymphoma. ( 20207840 )
2010
43
T-cell/histiocyte-rich large B-cell lymphoma shows transcriptional features suggestive of a tolerogenic host immune response. ( 19797726 )
2010
44
Splenic micronodular T-cell/histiocyte-rich large B-cell lymphoma: effect of prior corticosteroid therapy. ( 19763612 )
2009
45
Morphologic, immunphenotypic and clinical discriminators between T-cell/histiocyte-rich large B-cell lymphoma and lymphocyte-predominant Hodgkin lymphoma. ( 20063524 )
2008
46
Splenic micronodular T-cell/histiocyte-rich large B-cell lymphoma. ( 18620998 )
2008
47
Nodular lymphocyte-predominant hodgkin lymphoma or T-cell/histiocyte rich large B-cell lymphoma: the problem in "grey zone" lymphomas. ( 18784812 )
2008
48
T-cell/histiocyte-rich large B-cell lymphoma associated with a near-tetraploid karyotype and complex genetic abnormalities. ( 16856972 )
2006
49
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
50
Pediatric EBV-positive T-cell/histiocyte-rich large B-cell lymphoma with clonal cells in the bone marrow without overt involvement. ( 15621841 )
2005

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 humoral immune response GO:0006959 9.16 IFNG POU2F2
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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
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55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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