MCID: HPT070
MIFTS: 35

Hepatosplenic T-Cell Lymphoma

Categories: Blood diseases, Cancer diseases, Immune diseases, Liver diseases, Rare diseases

Aliases & Classifications for Hepatosplenic T-Cell Lymphoma

MalaCards integrated aliases for Hepatosplenic T-Cell Lymphoma:

Name: Hepatosplenic T-Cell Lymphoma 58 70 32

Characteristics:

Orphanet epidemiological data:

58
hepatosplenic t-cell lymphoma
Age of onset: Adolescent,Adult;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 32 C86.1
ICD10 via Orphanet 33 C86.1
UMLS via Orphanet 71 C1333984
Orphanet 58 ORPHA86882
UMLS 70 C1333984

Summaries for Hepatosplenic T-Cell Lymphoma

MalaCards based summary : Hepatosplenic T-Cell Lymphoma is related to t-cell adult acute lymphocytic leukemia and acute leukemia. An important gene associated with Hepatosplenic T-Cell Lymphoma is ABCB1 (ATP Binding Cassette Subfamily B Member 1). The drugs Infliximab and Antirheumatic Agents have been mentioned in the context of this disorder. Affiliated tissues include spleen, bone marrow and t cells.

Wikipedia : 73 Hepatosplenic T-cell lymphoma is a rare form of lymphoma that is generally incurable, except in the case... more...

Related Diseases for Hepatosplenic T-Cell Lymphoma

Diseases related to Hepatosplenic T-Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 90)
# Related Disease Score Top Affiliating Genes
1 t-cell adult acute lymphocytic leukemia 30.1 TIA1 CD7
2 acute leukemia 30.0 SETD2 CD7 ABCB1
3 composite lymphoma 30.0 TIA1 GZMB
4 t-cell lymphoma, subcutaneous panniculitis-like 29.9 TIA1 GZMB CD7
5 aggressive nk-cell leukemia 29.9 TIA1 GZMB CD7
6 mature t-cell and nk-cell lymphoma 29.9 TIA1 GZMB CD7
7 angioimmunoblastic t-cell lymphoma 29.6 TIA1 GZMB
8 peripheral t-cell lymphoma 10.7
9 lymphoma 10.7
10 crohn's disease 10.7
11 splenomegaly 10.7
12 inflammatory bowel disease 10.6
13 pancytopenia 10.6
14 lymphoma, hodgkin, classic 10.5
15 lymphoma, non-hodgkin, familial 10.5
16 thrombocytopenia 10.4
17 chromosomal triplication 10.4
18 crohn's colitis 10.4
19 leukemia 10.4
20 myelodysplastic syndrome 10.3
21 lymphoproliferative syndrome 10.3
22 t-cell lymphoblastic leukemia/lymphoma 10.3
23 leukemia, t-cell, chronic 10.3
24 inflammatory bowel disease 6 10.2
25 exanthem 10.2
26 glomerulonephritis 10.2
27 hemolytic anemia 10.2
28 ulcerative colitis 10.2
29 47,xyy 10.2
30 enteropathy-associated t-cell lymphoma 10.2
31 ring chromosome 7 10.2
32 ring chromosome 10.2
33 pfeiffer syndrome 10.1
34 spondyloarthropathy 1 10.1
35 systemic lupus erythematosus 10.1
36 schistosoma mansoni infection, susceptibility/ 10.1
37 anemia, autoimmune hemolytic 10.1
38 immune deficiency disease 10.1
39 myelofibrosis 10.1
40 leukemia, acute myeloid 10.1
41 graft-versus-host disease 10.1
42 neuropathy, congenital hypomyelinating, 2 10.1
43 deficiency anemia 10.1
44 disease by infectious agent 10.1
45 hemophagocytic lymphohistiocytosis 10.1
46 adult t-cell leukemia 10.1
47 anaplastic large cell lymphoma 10.1
48 t-cell large granular lymphocyte leukemia 10.1
49 disseminated intravascular coagulation 10.1
50 inflammatory spondylopathy 10.1

Graphical network of the top 20 diseases related to Hepatosplenic T-Cell Lymphoma:



Diseases related to Hepatosplenic T-Cell Lymphoma

Symptoms & Phenotypes for Hepatosplenic T-Cell Lymphoma

Drugs & Therapeutics for Hepatosplenic T-Cell Lymphoma

Drugs for Hepatosplenic T-Cell Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Infliximab Approved Phase 4 170277-31-3
2 Antirheumatic Agents Phase 4
3 Gastrointestinal Agents Phase 4
4 Dermatologic Agents Phase 4

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Research Study to Bank Samples for Future Evaluation to Identify Biomarkers That Predispose Crohn's Disease and Ulcerative Colitis Patients to Develop Hepatosplenic T-Cell Lymphoma (HSTCL) Completed NCT01804166 Phase 4 Infliximab;Golimumab
2 A Retrospective Review of Reports of Hepatosplenic T-cell Lymphoma in the Dutch National Database of Pathology (Pathologisch-Anatomisch Landelijk Geautomatiseerd Archief [PALGA]), a Database of Pathology Results for The Netherlands Completed NCT00731536
3 A Multicenter Study to Collect Samples for Future Evaluation of Biomarkers and Genetic Mutations in Patients Exposed to Adalimumab With Crohn's Disease (CD) or Ulcerative Colitis (UC) Who Developed Hepatosplenic T-Cell Lymphoma (HSTCL) Withdrawn NCT02087878

Search NIH Clinical Center for Hepatosplenic T-Cell Lymphoma

Genetic Tests for Hepatosplenic T-Cell Lymphoma

Anatomical Context for Hepatosplenic T-Cell Lymphoma

MalaCards organs/tissues related to Hepatosplenic T-Cell Lymphoma:

40
Spleen, Bone Marrow, T Cells, Liver, Myeloid, Thyroid, Pituitary

Publications for Hepatosplenic T-Cell Lymphoma

Articles related to Hepatosplenic T-Cell Lymphoma:

(show top 50) (show all 270)
# Title Authors PMID Year
1
Outcomes of Hepatosplenic T-Cell Lymphoma: The Mayo Clinic Experience. 61
33160933 2021
2
Hypercalcaemia: An Extremely Rare Presentation of Hepatosplenic T-Cell Lymphoma. 61
33768082 2021
3
Hepatic involvement by T-cell neoplasms: a clinicopathologic study of 40 cases. 61
33010300 2020
4
Successful treatment of hepatosplenic T cell lymphoma in an adolescent with Turner syndrome using ifosfamide, carboplatin, and etoposide followed by allogeneic hematopoietic stem cell transplant. 61
32776434 2020
5
Pralatrexate-based therapy induced response in an adolescent with refractory hepatosplenic T-cell lymphoma. 61
32860655 2020
6
NCCN Guidelines Insights: T-Cell Lymphomas, Version 1.2021. 61
33152703 2020
7
Hepatosplenic T-cell lymphoma: a rare but challenging entity. 61
32756940 2020
8
How Does It Look in an Autopsy? Hepatosplenic T-Cell Lymphoma in a Patient with Crohn’s Disease on Azathioprine 61
32270949 2020
9
Hepatosplenic αβ T-Cell Lymphoma as Second Malignancy in Young Adult Patient With Previously Undiagnosed Ataxia-Telangiectasia. 61
31259827 2020
10
Causes of double-negative T-cell lymphocytosis in children and adults. 61
31810993 2020
11
Intensive Induction Therapy Compared With CHOP for Hepatosplenic T-cell Lymphoma. 61
32284297 2020
12
Effect of chidamide on treating hepatosplenic T-cell lymphoma: A case report. 61
32775395 2020
13
Hepatosplenic T-Cell Lymphoma in an Immunocompetent Young Male: A Challenging Diagnosis. 61
32775075 2020
14
[Cell morphological analysis of hepatosplenic T-cell lymphoma gamma-delta type]. 61
32536127 2020
15
A case of hepatosplenic T-cell lymphoma successfully treated by HLA haploidentical stem cell transplantation. 61
32404572 2020
16
A Skin Plaque Preceding Systemic Relapse of Gamma-Delta Hepatosplenic T-Cell Lymphoma. 61
31725483 2020
17
Hepatosplenic T-cell lymphoma (HSTCL) in patient with Crohn's disease treated with azathioprin. 61
32439154 2020
18
Hepatosplenic T-Cell Lymphoma Diagnosed by Endoscopic Ultrasound-Guided Fine-Needle Biopsy. 61
32106363 2020
19
Targeting EZH2 for the treatment of hepatosplenic T-cell lymphoma. 61
32232478 2020
20
Systematic review: hepatosplenic T-cell lymphoma on biologic therapy for inflammatory bowel disease, including data from the Food and Drug Administration Adverse Event Reporting System. 61
31990422 2020
21
Complete Spontaneous Regression of Hepatosplenic T-Cell Lymphoma After Surgical Biopsy. 61
31652159 2020
22
Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas. 61
31709579 2020
23
T-cell lymphoproliferative processes in the spleen. 61
31879048 2020
24
Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-α Inhibitors for Ankylosing Spondylitis. 61
31764502 2019
25
Hepatosplenic T-Cell Lymphoma in an Immunocompetent Male with Central Nervous System Invasion: A Rare Clinical Entity. 61
30500103 2019
26
Tetrasomy 8 and isochromosome 7q in CD5-positive hepatosplenic T-cell lymphoma with leukemic presentation. 61
31342408 2019
27
Refractory α/β-Hepatosplenic T-cell Lymphoma With Secondary CNS Dissemination and Severe Morbidity Related to Intrathecal Chemotherapy: A Case Report and Literature Review. 61
31362868 2019
28
Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn's Disease. 61
31328143 2019
29
Deregulated expression of NKL homeobox genes in T-cell lymphomas. 61
31143370 2019
30
Development of Leiomyosarcoma in a Patient Treated with Azathioprine. 61
31454474 2019
31
DNA methylation profiling of hepatosplenic T-cell lymphoma. 61
30337361 2019
32
Hepatosplenic T cell lymphoma presenting as multiorgan failure. 61
30902844 2019
33
Successful Bridging Chemotherapy with Gemcitabine, Carboplatin, and Dexamethasone before Unrelated Stem Cell Transplantation for Hepatosplenic T-cell Lymphoma. 61
30449784 2019
34
The Impact of Inflammatory Bowel Disease in Canada 2018: Children and Adolescents with IBD. 61
31294385 2019
35
Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorders with a T- or NK-cell phenotype. 61
31257346 2019
36
[Acute HIV infection pathologically mimicking T-cell lymphoma]. 61
31391369 2019
37
An unusual presentation of an uncommon lymphoma, hepatosplenic T-cell lymphoma. 61
30956608 2019
38
Case of a CD3 Negative Hepatosplenic T-Cell Lymphoma: Diagnostic and Therapeutic Challenges. 61
31346479 2019
39
Hepatosplenic T-Cell Lymphomas. 61
30596219 2019
40
Collapsing glomerulonephritis with podocyte markers in hemophagocytic syndrome secondary to hepatosplenic T-cell lymphoma 61
30653858 2018
41
A rare case of hepatosplenic γδ T-cell lymphoma expressing CD19 with ring chromosome 7 and trisomy 8. 61
30553467 2018
42
Clinicopathological features of splenic tumours of lymphoid tissue. 61
30087035 2018
43
Successful second allogeneic stem-cell transplantation from the same sibling donor for a patient with recurrent hepatosplenic gamma-delta (γ/δ) T-cell lymphoma: A case report. 61
30383643 2018
44
Hepatosplenic γδ T Cell Lymphoma Involving the Brain. 61
30030187 2018
45
Hepatosplenic T cell lymphoma: a unifying entity in a patient with hemolytic anemia, massive splenomegaly, and liver dysfunction. 61
29766398 2018
46
Malignancy and mortality in paediatric-onset inflammatory bowel disease: a 3-year prospective, multinational study from the paediatric IBD Porto group of ESPGHAN. 61
29984520 2018
47
Peripheral T-Cell Lymphomas: Incorporating New Developments in Diagnostics, Prognostication, and Treatment Into Clinical Practice-PART 2: ENKTL, EATL, Indolent T-Cell LDP of the GI Tract, ATLL, and Hepatosplenic T-Cell Lymphoma. 61
30153323 2018
48
Therapeutic role of methotrexate in pediatric Crohn's disease. 61
29338679 2018
49
Peripheral T-Cell Lymphomas: Incorporating New Developments in Diagnostics, Prognostication, and Treatment Into Clinical Practice-PART 1: PTCL-NOS, FTCL, AITL, ALCL. 61
30080923 2018
50
[Incidence of Bone Marrow Involvement in Different Pathological Type Lymphoma Patients]. 61
29950217 2018

Variations for Hepatosplenic T-Cell Lymphoma

Expression for Hepatosplenic T-Cell Lymphoma

LifeMap Discovery
Genes differentially expressed in tissues of Hepatosplenic T-Cell Lymphoma patients vs. healthy controls: 35 (show top 50) (show all 399)
# Gene Description Tissue Up/Dn Fold Change (log2) P value
1 TMEM178A transmembrane protein 178A Spleen + 9.71 0.000
2 LPAR3 lysophosphatidic acid receptor 3 Spleen + 8.91 0.000
3 SOX2 SRY-box transcription factor 2 Spleen + 8.60 0.000
4 MLC1 modulator of VRAC current 1 Spleen + 7.68 0.001
5 CLEC3B C-type lectin domain family 3 member B Spleen - 7.16 0.001
6 KIR2DS1 killer cell immunoglobulin like receptor, two Ig domains and short cytoplasmic tail 1 Spleen + 7.16 0.000
7 HLA-DQA1 major histocompatibility complex, class II, DQ alpha 1 Spleen - 7.01 0.023
8 KIR3DL1 killer cell immunoglobulin like receptor, three Ig domains and long cytoplasmic tail 1 Spleen + 6.95 0.000
9 KIR3DS1 killer cell immunoglobulin like receptor, three Ig domains and short cytoplasmic tail 1 Spleen + 6.85 0.001
10 ADAMTS17 ADAM metallopeptidase with thrombospondin type 1 motif 17 Spleen + 6.83 0.000
11 KIR3DL3 killer cell immunoglobulin like receptor, three Ig domains and long cytoplasmic tail 3 Spleen + 6.66 0.000
12 KIR2DL3 killer cell immunoglobulin like receptor, two Ig domains and long cytoplasmic tail 3 Spleen + 6.63 0.001
13 MARCO macrophage receptor with collagenous structure Spleen + 6.61 0.009
14 KIR2DS3 killer cell immunoglobulin like receptor, two Ig domains and short cytoplasmic tail 3 Spleen + 6.34 0.001
15 CXCL13 C-X-C motif chemokine ligand 13 Spleen - 6.26 0.004
16 OLFML2A olfactomedin like 2A Spleen - 6.25 0.015
17 KIR2DL2 killer cell immunoglobulin like receptor, two Ig domains and long cytoplasmic tail 2 Spleen + 6.21 0.002
18 KIR2DS2 killer cell immunoglobulin like receptor, two Ig domains and short cytoplasmic tail 2 Spleen + 6.14 0.000
19 KIR2DS4 killer cell immunoglobulin like receptor, two Ig domains and short cytoplasmic tail 4 Spleen + 6.13 0.001
20 GREB1 growth regulating estrogen receptor binding 1 Spleen + 6.09 0.001
21 KIR2DL1 killer cell immunoglobulin like receptor, two Ig domains and long cytoplasmic tail 1 Spleen + 6.02 0.002
22 LOC100505501 uncharacterized LOC100505501 Spleen + 5.96 0.000
23 CFAP91 cilia and flagella associated protein 91 Spleen - 5.93 0.000
24 TRGV5 T cell receptor gamma variable 5 Spleen + 5.90 0.000
25 CRHBP corticotropin releasing hormone binding protein Spleen - 5.89 0.000
26 KLRC3 killer cell lectin like receptor C3 Spleen + 5.87 0.000
27 SMIM24 small integral membrane protein 24 Spleen + 5.85 0.000
28 CCM2L CCM2 like scaffold protein Spleen - 5.83 0.005
29 RUNDC3B RUN domain containing 3B Spleen + 5.82 0.000
30 HLA-DQB1 major histocompatibility complex, class II, DQ beta 1 Spleen + 5.80 0.000
31 PCDH7 protocadherin 7 Spleen - 5.78 0.010
32 APLNR apelin receptor Spleen - 5.76 0.000
33 KIR2DL5A killer cell immunoglobulin like receptor, two Ig domains and long cytoplasmic tail 5A Spleen + 5.73 0.001
34 KIR2DS5 killer cell immunoglobulin like receptor, two Ig domains and short cytoplasmic tail 5 Spleen + 5.69 0.001
35 GIPC2 GIPC PDZ domain containing family member 2 Spleen - 5.69 0.000
36 GTSF1 gametocyte specific factor 1 Spleen + 5.62 0.001
37 GPM6A glycoprotein M6A Spleen - 5.62 0.000
38 IGFBP3 insulin like growth factor binding protein 3 Spleen - 5.61 0.004
39 BMP7 bone morphogenetic protein 7 Spleen + 5.59 0.000
40 PDE2A phosphodiesterase 2A Spleen - 5.58 0.002
41 KIF14 kinesin family member 14 Spleen + 5.55 0.000
42 HYAL1 hyaluronidase 1 Spleen - 5.51 0.006
43 GPR82 G protein-coupled receptor 82 Spleen + 5.49 0.000
44 DNASE1L3 deoxyribonuclease 1 like 3 Spleen - 5.44 0.003
45 USP44 ubiquitin specific peptidase 44 Spleen + 5.44 0.000
46 CD96 CD96 molecule Spleen + 5.42 0.001
47 AKAP5 A-kinase anchoring protein 5 Spleen + 5.41 0.000
48 RNASE1 ribonuclease A family member 1, pancreatic Spleen - 5.40 0.008
49 PLVAP plasmalemma vesicle associated protein Spleen - 5.38 0.001
50 NEK2 NIMA related kinase 2 Spleen + 5.35 0.000
Search GEO for disease gene expression data for Hepatosplenic T-Cell Lymphoma.

Pathways for Hepatosplenic T-Cell Lymphoma

GO Terms for Hepatosplenic T-Cell Lymphoma

Biological processes related to Hepatosplenic T-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 T cell activation GO:0042110 8.8 PIK3CD DPP4 CD7

Molecular functions related to Hepatosplenic T-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 alpha-tubulin binding GO:0043014 8.62 SETD2 INO80

Sources for Hepatosplenic T-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
39 LOVD
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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