MCID: TCL012
MIFTS: 31

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 20 58
T-Cell/histiocyte Rich Large B-Cell Lymphoma 70
Thrlbcl 58

Characteristics:

Orphanet epidemiological data:

58
t-cell/histiocyte rich large b cell lymphoma
Inheritance: Multigenic/multifactorial,Not applicable;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 via Orphanet 33 C83.3
UMLS via Orphanet 71 C1321547
Orphanet 58 ORPHA300857
UMLS 70 C1321547

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

GARD : 20 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 300857 Definition T- 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.

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 diffuse large b-cell lymphoma. 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 Immune response Lectin induced complement pathway. Affiliated tissues include bone marrow, spleen and bone, and related phenotype is 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 151)
# Related Disease Score Top Affiliating Genes
1 lymphoma, hodgkin, classic 31.3 TNFRSF8 CD68 BCL6
2 diffuse large b-cell lymphoma 30.9 TNFRSF8 MME BCL6
3 b-cell lymphoma 30.5 TNFRSF8 POU2F2 MME IGH CR2 BCL6
4 follicular lymphoma 30.3 MME IGH CR2 CR1 BCL6
5 lupus erythematosus 30.2 IFNG CR2 CR1
6 lung lymphoma 30.2 TNFRSF8 MME BCL6
7 peripheral t-cell lymphoma 30.1 TNFRSF8 MME BCL6
8 lymphoma, non-hodgkin, familial 29.5 TNFRSF8 MME IGH CR2 BCL6
9 rapidly involuting congenital hemangioma 11.2
10 lymphoma 11.1
11 nodular lymphocyte predominant hodgkin lymphoma 11.0
12 hemophagocytic lymphohistiocytosis 10.6
13 lymphoproliferative syndrome 10.5
14 duodenal obstruction 10.5
15 lymphomatoid granulomatosis 10.5
16 splenomegaly 10.5
17 systemic lupus erythematosus 10.3
18 sarcoidosis 1 10.3
19 vitiligo-associated multiple autoimmune disease susceptibility 6 10.3
20 anemia, autoimmune hemolytic 10.3
21 ocular motor apraxia 10.3
22 thymoma, familial 10.3
23 vitiligo-associated multiple autoimmune disease susceptibility 1 10.3
24 deficiency anemia 10.3
25 anaplastic large cell lymphoma 10.3
26 cardiac arrest 10.3
27 tonsillitis 10.3
28 pancytopenia 10.3
29 pure red-cell aplasia 10.3
30 lymphadenitis 10.3
31 pharyngitis 10.3
32 hidradenitis suppurativa 10.3
33 hidradenitis 10.3
34 agammaglobulinemia 10.3
35 central nervous system lymphoma 10.3
36 thymoma 10.3
37 pustulosis of palm and sole 10.3
38 hemolytic anemia 10.3
39 lymphopenia 10.3
40 thyroiditis 10.3
41 psoriasis 10.3
42 exophthalmos 10.3
43 hypereosinophilic syndrome 10.3
44 cytokine deficiency 10.3
45 indolent b cell lymphoma 10.3
46 primary central nervous system lymphoma 10.3
47 primary pulmonary lymphoma 10.3
48 secondary hemophagocytic lymphohistiocytosis 10.3
49 thyroid tumor 10.3
50 spinal cord lymphoma 10.3 CR1 BCL6

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 immune system MP:0005387 9.23 BCL6 CD68 CR2 IFNG MME POU2F2

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

Search Clinical Trials , 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:

40
Bone Marrow, Spleen, Bone, Breast, T Cells, B Cells, 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 108)
# Title Authors PMID Year
1
Spatial signatures identify immune escape via PD-1 as a defining feature of T-cell/histiocyte-rich large B-cell lymphoma. 61
32871584 2021
2
Markers of Follicular Helper T Cells Are Occasionally Expressed in T-Cell or Histiocyte-Rich Large B-Cell Lymphoma, Classic Hodgkin Lymphoma, and Atypical Paracortical HyperplasiaA Diagnostic Pitfall For T-Cell Lymphomas of T Follicular Helper Origin. 61
33624021 2021
3
T-cell/histiocyte-rich large B-cell lymphoma in a 27-year-old with hidradenitis suppurativa, psoriasis, and vitiligo: Implications for screening. 61
33294556 2020
4
PD-1 and PD-L1 Immunohistochemistry as a Diagnostic Tool for Classic Hodgkin Lymphoma in Small-volume Biopsies. 61
32649320 2020
5
Role of FNA with core biopsy or cell block in patients with nodular lymphocyte-predominant Hodgkin lymphoma. 61
32343479 2020
6
Metabolic tumor volume, cancer cell fraction, and prognosis - the case of T-cell/histiocyte-rich large B-cell lymphoma. 61
32022621 2020
7
Characteristics of diffuse large B-cell lymphoma in patients with primary Sjögren's syndrome. 61
32100426 2020
8
Immunophenotypic Characterization and Purification of Neoplastic Cells from Lymph Nodes Involved by T-Cell/Histiocyte-rich Large B-cell Lymphoma by Flow Cytometry and Flow Cytometric Cell Sorting. 61
31254446 2020
9
Diffuse large B-cell lymphoma variants: an update. 61
31735345 2020
10
Nodular lymphocyte predominant Hodgkin lymphoma: pathology, clinical course and relation to T-cell/histiocyte rich large B-cell lymphoma. 61
31785822 2020
11
Meningeal Relapse of Nodular Lymphocyte Predominant Hodgkin Lymphoma Transformed to T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: A Case Report. 61
33042818 2020
12
Hypercalcemia in T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: An Unusual Presentation of a Rare Disease and Literature Review. 61
31921379 2019
13
Anaplastic variant of diffuse large B-cell lymphoma: Reappraisal as a nodal disease with sinusoidal involvement. 61
31872533 2019
14
Clinical features and survival of patients with T-cell/histiocyte-rich large B-cell lymphoma: analysis of the National Cancer Data Base. 61
31287335 2019
15
T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: Report of the First Case in the Mandible. 61
30019325 2019
16
T-cell/Histiocyte-rich Large B-cell Lymphoma of the Larynx in a Juvenile Japanese Macaque (Macaca fuscata). 61
31159945 2019
17
T cell/histiocyte-rich large B cell lymphoma: incidence, demographic disparities, and long-term outcomes. 61
29845599 2019
18
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. 61
30816829 2019
19
Aggressive B cell lymphomas in the 2017 revised WHO classification of tumors of hematopoietic and lymphoid tissues. 61
30380402 2019
20
JUNB, DUSP2, SGK1, SOCS1 and CREBBP are frequently mutated in T-cell/histiocyte-rich large B-cell lymphoma. 61
30213827 2019
21
Identification of a novel monocytic phenotype in Classic Hodgkin Lymphoma tumor microenvironment. 61
31714922 2019
22
Utility of Combined EZH2, p-ERK1/2, p-STAT, and MYC Expression in the Differential Diagnosis of EZH2-positive Hodgkin Lymphomas and Related Large B-Cell Lymphomas. 61
30371509 2019
23
T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: A Challenging Diagnosis. 61
31508381 2019
24
T-cell/histiocyte-rich large B-cell lymphoma in a child: A case report and review of literature. 61
29988902 2018
25
Bone Marrow Involvement in Patients With Nodular Lymphocyte Predominant Hodgkin Lymphoma. 61
29309302 2018
26
A Case of Spontaneously Improving Secondary Hemophagocytic Lymphohistiocytosis in an Adult Associated with T-Cell Histiocyte-Rich Large B-Cell Lymphoma. 61
29682376 2018
27
A Case of Chemotherapy-Refractory "THRLBCL like Transformation of NLPHL" Successfully Treated with Lenalidomide. 61
29552367 2018
28
Hemophagocytic Lymphohistiocytosis Secondary to T Cell/Histiocyte-rich Large B Cell Lymphoma in an Adolescent Male. 61
29187030 2018
29
J chain and myocyte enhancer factor 2B are useful in differentiating classical Hodgkin lymphoma from nodular lymphocyte predominant Hodgkin lymphoma and primary mediastinal large B-cell lymphoma. 61
28851661 2017
30
Thioredoxin-1, chemokine (C-X-C motif) ligand-9 and interferon-γ expression in the neoplastic cells and macrophages of Hodgkin lymphoma: clinicopathologic correlations and potential prognostic implications. 61
28571489 2017
31
T-cell/histiocyte-rich large B-cell lymphoma of stomach: a rare variant of gastric B cell lymphoma. 61
29694656 2017
32
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. 61
28575178 2017
33
Pancreatic T/histiocyte-rich large B-cell lymphoma: A case report and review of literature. 61
28706431 2017
34
Cytological findings of langerhans cell sarcoma in a case of quintuple cancer. 61
28233936 2017
35
T cell-rich lymphoid infiltrates with large B cells: a review of key entities and diagnostic approach. 61
27895166 2017
36
T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Presented as T-Lymphoid Hyperplasia Involving the Central Nervous System. 61
28451478 2017
37
[Update on nodular lymphocyte predominant Hodgkin's lymphoma and related lesions]. 61
27999937 2017
38
Cyclin D1 expression and polysomy in lymphocyte-predominant cells of nodular lymphocyte-predominant Hodgkin lymphoma. 61
28038705 2017
39
Atypical variants of nodular lymphocyte-predominant Hodgkin lymphoma show low microvessel density and vessels of distention type. 61
27816718 2017
40
T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease. 61
28912991 2017
41
Acute Liver Failure Caused by Primary Non-Hodgkin's Lymphoma of the Liver. 61
27932130 2016
42
Reactive T cells by flow cytometry distinguish Hodgkin lymphomas from T cell/histiocyte-rich large B cell lymphoma. 61
26084540 2016
43
T-cell/histiocyte-rich large B-cell lymphoma of stomach. 61
27427148 2016
44
Single-agent MOR208 salvage and maintenance therapy in a patient with refractory/relapsing diffuse large B-cell lymphoma: a case report. 61
27178351 2016
45
Rare thymic malignancy of B-cell origin - T-cell÷histiocyte-rich large B-cell lymphoma. 61
28002527 2016
46
T Cell/Histiocyte-Rich Large B Cell Lymphoma of the Thymus: A Diagnostic Pitfall. 61
26904321 2016
47
Diagnostic Utility of Flow Cytometry Analysis of Reactive T Cells in Nodular Lymphocyte-Predominant Hodgkin Lymphoma. 61
26712878 2016
48
From a pathologist's point of view: Histiocytic cells in Hodgkin lymphoma and T cell/histiocyte rich large B cell lymphoma. 61
26596262 2015
49
T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Presenting as a Primary Central Nervous System Lymphoma. 61
26788280 2015
50
A T-cell/histiocyte-rich large B-cell lymphoma involving only the bone marrow. 61
26770945 2015

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

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.94 POU2F2 CR2 BCL6
2
Show member pathways
11.7 IGH CR2 CR1
3 11.59 VSIG4 CR2 CR1
4 11.4 MME IFNG CD68
5 11.13 MME CR2 CR1
6 10.62 CR2 CR1 BCL6

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 negative regulation of T cell proliferation GO:0042130 9.43 VSIG4 CR1
2 positive regulation of neurogenesis GO:0050769 9.4 MME IFNG
3 negative regulation of interleukin-2 production GO:0032703 9.37 VSIG4 CR1
4 regulation of regulatory T cell differentiation GO:0045589 9.32 IFNG CR1
5 positive regulation of regulatory T cell differentiation GO:0045591 9.26 CR1 BCL6
6 complement receptor mediated signaling pathway GO:0002430 9.16 CR2 CR1
7 negative regulation of complement activation, alternative pathway GO:0045957 8.96 VSIG4 CR1
8 complement activation, alternative pathway GO:0006957 8.8 VSIG4 CR2 CR1

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

# Name GO ID Score Top Affiliating Genes
1 complement component C3b binding GO:0001851 8.62 VSIG4 CR1

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

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
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41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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