MCID: HPT070
MIFTS: 50

Hepatosplenic T-Cell Lymphoma

Categories: Blood diseases, Cancer diseases, Immune diseases, Liver diseases, Rare diseases
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Aliases & Classifications for Hepatosplenic T-Cell Lymphoma

MalaCards integrated aliases for Hepatosplenic T-Cell Lymphoma:

Name: Hepatosplenic T-Cell Lymphoma 11 58 71 31 33
Doid:0081049 14

Characteristics:


Prevelance:

<1/1000000 (United States) 58

Age Of Onset:

Adolescent,Adult 58

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 11 DOID:0081049
NCIt 49 C8459
ICD10 31 C86.1
ICD10 via Orphanet 32 C86.1
UMLS via Orphanet 72 C1333984
Orphanet 58 ORPHA86882
ICD11 33 1301206942
UMLS 71 C1333984

Summaries for Hepatosplenic T-Cell Lymphoma

Orphanet: 58 A rare T-cell non-Hodgkin lymphoma characterized by a proliferation of cytotoxic T-cells, usually gamma delta T-cells, with involvement of the liver and spleen, but without involvement of lymph nodes. The bone marrow is consistently affected. Patients typically present during adolescence or young adulthood with hepatosplenomegaly, pancytopenia, and systemic symptoms. Peripheral blood involvement may develop later in the disease course. There is a clear male preponderance. The disease often occurs in the context of long-term immunosuppression. The course is aggressive with poor therapy response.

MalaCards based summary: Hepatosplenic T-Cell Lymphoma, also known as doid:0081049, is related to lymphoma and lymphoma, hodgkin, classic. An important gene associated with Hepatosplenic T-Cell Lymphoma is TIA1 (TIA1 Cytotoxic Granule Associated RNA Binding Protein), and among its related pathways/superpathways are NF-kappaB Signaling and IL12-mediated signaling events. The drugs Infliximab and Golimumab have been mentioned in the context of this disorder. Affiliated tissues include spleen, t cells and bone marrow, and related phenotypes are no effect and no effect

Disease Ontology: 11 A mature T-cell and NK-cell lymphoma that is characterized by the presence of medium-size neoplastic lymphocytes infiltrating the hepatic sinusoids and that originates from cytotoxic T-cells, usually of gamma/delta T-cell type.

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 472)
# Related Disease Score Top Affiliating Genes
1 lymphoma 31.3 TNFRSF8 TIA1 GZMB CD5 ALK
2 lymphoma, hodgkin, classic 30.9 TNFRSF8 TIA1 GZMB ALK
3 lymphoproliferative syndrome 30.8 TNFRSF8 TIA1 CD5 ALK
4 aplastic anemia 30.6 TNF GZMB CD8A CD4
5 composite lymphoma 30.5 TNFRSF8 CD5
6 anaplastic large cell lymphoma 30.5 TNFRSF8 GZMB ALK
7 adult lymphoma 30.5 TNFRSF8 CD5 ALK
8 anemia, autoimmune hemolytic 30.4 CD8A CD5 CD4
9 exanthem 30.3 TNF CD8A CD4 ALK
10 progressive multifocal leukoencephalopathy 30.3 TNF CD8A CD4
11 t-cell acute lymphoblastic leukemia 30.3 TNFRSF8 CD8A CD7 CD5 CD4 CD2
12 spondyloarthropathy 1 30.2 TNF CD8A CD4
13 disease by infectious agent 30.0 TNFRSF8 TNF CD8A CD4
14 lymphoma, non-hodgkin, familial 30.0 TNFRSF8 NCAM1 GZMB CD8A CD7 CD5
15 hemophagocytic lymphohistiocytosis 30.0 TNF NCAM1 GZMB CD8A CD4
16 acute leukemia 30.0 TPMT SETD2 NCAM1 CD8A CD7
17 pancytopenia 30.0 TPMT NCAM1 CD8A CD7 CD5 CD4
18 nasal type extranodal nk/t-cell lymphoma 30.0 TNFRSF8 TIA1 NCAM1 GZMB CD2
19 pfeiffer syndrome 29.9 TNFRSF8 TNF NCAM1 CD8A CD4
20 peripheral t-cell lymphoma 29.9 TNFRSF8 TIA1 NCAM1 GZMB CD8A CD7
21 adult t-cell leukemia/lymphoma 29.8 TNFRSF8 CD8A CD7 CD5 CD4 CD2
22 immune deficiency disease 29.8 TNFRSF8 TNF STAT5B CD8A CD5 CD4
23 myelodysplastic syndrome 29.7 TNF STAT5B NCAM1 GZMB CD8A CD7
24 deficiency anemia 29.5 TPMT TNF CD8A CD7 CD5 CD4
25 primary cutaneous gamma-delta t-cell lymphoma 29.5 TNFRSF8 TIA1 NCAM1 GZMB CD8A CD7
26 t-cell adult acute lymphocytic leukemia 29.5 TNFRSF8 TIA1 CD8A CD7 CD5 CD4
27 t-cell large granular lymphocyte leukemia 29.4 STAT5B NCAM1 GZMB CD8A CD7 CD5
28 angioimmunoblastic t-cell lymphoma 29.0 TNFRSF8 TIA1 DUSP22 CD8A CD7 CD5
29 aggressive nk-cell leukemia 29.0 TNFRSF8 TIA1 NCAM1 GZMB CD8A CD7
30 leukemia, acute lymphoblastic 28.9 TPMT STAT5B SETD2 CD8A CD7 CD5
31 leukemia, chronic lymphocytic 28.5 TNFRSF8 TNF STAT5B NCAM1 GZMB CD8A
32 mature t-cell and nk-cell lymphoma 28.1 TNFRSF8 TIA1 STAT5B NCAM1 GZMB DUSP22
33 leukemia, acute myeloid 28.1 TNFRSF8 TNF STAT5B SETD2 NCAM1 GZMB
34 burkitt lymphoma 10.8
35 splenomegaly 10.7
36 crohn's disease 10.7
37 inflammatory bowel disease 10.5
38 chromosomal triplication 10.5
39 inflammatory bowel disease 1 10.4
40 thrombocytopenia 10.4
41 orbit lymphoma 10.4 TIA1 NCAM1
42 sternum lymphoma 10.4 NCAM1 CD5
43 sternum cancer 10.4 NCAM1 CD5
44 papillary thymic adenocarcinoma 10.4 NCAM1 CD5
45 tracheal lymphoma 10.3 TNFRSF8 CD5
46 retroperitoneal lymphoma 10.3 TNFRSF8 CD5
47 mixed phenotype acute leukemia, t/myeloid 10.3 CD7 CD2
48 small intestine lymphoma 10.3 TNFRSF8 CD5
49 rete testis adenoma 10.3 TNFRSF8 NCAM1
50 inflammatory bowel disease 16 10.3 CD7 CD2

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

GenomeRNAi Phenotypes related to Hepatosplenic T-Cell Lymphoma according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 10.1 ALK B3GAT1 CD2 CD4 CD5 CD7
2 no effect GR00402-S-2 10.1 ALK CD2 CD4 CD5 CD7 DERL2
3 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-1 9.65 CD2 DERL2 DUSP22 GZMB TNF
4 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 9.65 CD2 DERL2 DUSP22 GZMB TNF

MGI Mouse Phenotypes related to Hepatosplenic T-Cell Lymphoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.73 CD4 CD5 CD7 CD8A DERL2 DUSP22
2 hematopoietic system MP:0005397 9.4 CD4 CD5 CD7 CD8A DUSP22 GZMB

Drugs & Therapeutics for Hepatosplenic T-Cell Lymphoma

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

(show top 50) (show all 121)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Infliximab Approved Phase 4 170277-31-3
2
Golimumab Approved Phase 4 476181-74-5
3
Pirarubicin Investigational Phase 4 72496-41-4 4844
4 Gastrointestinal Agents Phase 4
5 Tumor Necrosis Factor Inhibitors Phase 4
6
Etoposide Approved Phase 2 33419-42-0 36462
7
Ifosfamide Approved Phase 2 3778-73-2 3690
8
Cisplatin Approved Phase 2 15663-27-1 2767 5702198 441203
9
Levoleucovorin Approved, Experimental, Investigational Phase 2 68538-85-2, 58-05-9, 73951-54-9 149436 6006
10
Carboplatin Approved Phase 2 41575-94-4 10339178 38904
11
Panobinostat Approved, Investigational Phase 2 404950-80-7 6918837
12
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 4112 126941
13
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
14
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
15
Busulfan Approved, Investigational Phase 2 55-98-1 2478
16
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
17
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
18
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
19
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
20
Prednisolone Approved, Vet_approved Phase 2 50-24-8 4894 5755
21
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5 1875
22
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 4159 6741
23
Everolimus Approved Phase 1, Phase 2 159351-69-6 70789204 6442177
24
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 5284616 6436030
25
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239
26
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3 3680
27
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 3003 5743
28
Gemcitabine Approved Phase 2 95058-81-4, 122111-03-9 60750
29
Alemtuzumab Approved, Investigational Phase 2 216503-57-0
30
Iron Approved Phase 2 7439-89-6 29936
31
Deferasirox Approved, Investigational Phase 2 201530-41-8 214348 5493381
32
Pembrolizumab Approved Phase 1, Phase 2 1374853-91-4 254741536
33
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
34
Vidarabine Approved, Investigational Phase 2 24356-66-9 21704
35
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
36
Picropodophyllin Approved, Investigational Phase 1, Phase 2 518-28-5, 477-47-4 10607 72435
37
Prednisone Approved, Vet_approved Phase 1, Phase 2 53-03-2 5865
38
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
39
Daunorubicin Approved Phase 2 20830-81-3 30323
40
Brentuximab vedotin Approved, Investigational Phase 1, Phase 2 914088-09-8
41
Nivolumab Approved Phase 2 946414-94-4
42
Rituximab Approved Phase 2 174722-31-7
43
Lenalidomide Approved Phase 2 191732-72-6 216326
44
Ipilimumab Approved Phase 1, Phase 2 477202-00-9
45
Palifosfamide Investigational Phase 2 31645-39-3 100427
46
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7 4897
47
Veliparib Investigational Phase 1, Phase 2 912444-00-9 11960529
48
Cortisone Experimental Phase 1, Phase 2 53-06-5 222786
49
Etoposide phosphate Phase 2 16760419
50 Folic Acid Antagonists Phase 2

Interventional clinical trials:

(show top 50) (show all 67)
# Name Status NCT ID Phase Drugs
1 An Open-label,Multicenter Randomised Study of CTOP/ITE/MTX Compared With CHOP as the First-line Therapy for the New Diagnosed Young Patients With T Cell Non-hodgkin Lymphoma Unknown status NCT01746992 Phase 4 Cyclophosphamide 750mg/m2;Vincristine 1.4mg/m2;Doxorubicin 50mg/m2;prednisone 60mg/m2;ifosfamide 2000mg/m2;pirarubicin 50mg/m2;pirarubicin 25mg/m2;Etoposide phosphate 100mg/m2;methotrexate 1500mg/m2
2 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
3 Chimeric Antigen Receptor-Modified pNK Cells for CD7 Positive Relapsed or Refractory Leukemia and Lymphoma Unknown status NCT02742727 Phase 1, Phase 2
4 CEOP/IVE/GDP Compared With CEOP as the First-line Therapy for Newly Diagnosed Adult Patients With Peripheral T-cell Lymphoma (PTCL) Unknown status NCT02533700 Phase 2 CEOP/IVE/GDP chemotherapy regimen;CEOP chemotherapy regimen for 6 cycles
5 A Phase II Study of the Histone Deacetylase (HDAC) Inhibitor LBH589 (Panobinostat) in Patients With Relapsed or Refractory Non-Hodgkin Lymphoma Completed NCT01261247 Phase 2 panobinostat
6 Phase II Study of MK-2206 in Patients With Relapsed Lymphoma Completed NCT01258998 Phase 2 Akt inhibitor MK2206
7 A Phase II Study to Evaluate the Efficacy of Posttransplant Cyclophosphamide for Prevention of Chronic Graft-versus-Host Disease After Allogeneic Peripheral Blood Stem Cell Transplantation Completed NCT01427881 Phase 2 cyclophosphamide;cyclosporine;fludarabine phosphate;busulfan
8 High-dose Busulfan, High-dose Cyclophosphamide, and Allogeneic Bone Marrow Transplantation for Leukemia, Myelodysplastic Syndromes, Multiple Myeloma and Lymphoma Completed NCT01177371 Phase 2 busulfan;cyclophosphamide;methylprednisolone;methotrexate;cyclosporine
9 A Phase II Trial of Reduced Intensity Allogeneic Stem Cell Transplantation With Fludarabine, Melphalan and Low Dose Total Body Irradiation Completed NCT01529827 Phase 2 fludarabine phosphate;melphalan;tacrolimus;mycophenolate mofetil;methotrexate
10 A Phase I/II Clinical Trial of the mTor Inhibitor RAD001 (Everolimus) in Combination With Lenalidomide (Revlimid) for Patients With Relapsed or Refractory Lymphoid Malignancy Completed NCT01075321 Phase 1, Phase 2 everolimus;lenalidomide
11 A Phase II Study of Sorafenib (BAY 43-9006) in Recurrent Aggressive Non-Hodgkin's Lymphoma Completed NCT00131937 Phase 2 sorafenib tosylate
12 Prolonged Mycophenolate Mofetil and Truncated Cyclosporine Postgrafting Immunosuppression to Reduce Life-Threatening GVHD After Unrelated Donor Peripheral Blood Cell Transplantation Using Nonmyeloablative Conditioning for Patients With Hematologic Malignancies and Renal Cell Carcinoma - A Multi-Center Trial Completed NCT00078858 Phase 1, Phase 2 fludarabine phosphate;cyclosporine;mycophenolate mofetil
13 A Phase II Study Evaluating the Efficacy of Gemcitabine, Carboplatin, and Dexamethasone and Rituximab for Previously Treated Lymphoid Malignancies Completed NCT00072514 Phase 2 gemcitabine hydrochloride;carboplatin;dexamethasone
14 An Open-label Phase 2 Study of Intravenous Bortezomib and Oral Panobinostat (LBH589) in Adult Patients With Relapsed/Refractory Peripheral T-cell Lymphoma or NK/T-cell Lymphoma After Failure of Conventional Chemotherapy Completed NCT00901147 Phase 2 panobinostat and bortezomib
15 Campath® [Alemtuzumab] Dose Escalation, Low-Dose TBI and Fludarabine Followed by HLA Class I Mismatched Donor Stem Cell Transplantation for Patients With Hematologic Malignancies - A Multi-Center Trial Completed NCT00040846 Phase 2 fludarabine phosphate;mycophenolate mofetil;cyclosporine
16 A Phase I/II Study of the Histone Deacetylase (HDAC) Inhibitor LBH589 (Panobinostat) in Combination With mTOR Inhibitor RAD001 (Everolimus) in Patients With Relapsed Multiple Myeloma or Lymphoma Completed NCT00918333 Phase 1, Phase 2 panobinostat;everolimus
17 Nonmyeloablative Hematopoietic Stem Cell Transplantation for Patients With High-Risk Hematologic Malignancies Using Related, HLA-Haploidentical Donors: A Phase II Trial of Combined Immunosuppression Before and After Transplantation Completed NCT00049504 Phase 2 cyclophosphamide;fludarabine phosphate;tacrolimus;mycophenolate mofetil
18 Induction of Mixed Hematopoietic Chimerism in Patients Using Fludarabine, Low Dose TBI, PBSC Infusion and Post-Transplant Immunosuppression With Cyclosporine and Mycophenolate Mofetil Completed NCT00006251 Phase 1, Phase 2 fludarabine phosphate;cyclosporine;mycophenolate mofetil
19 Phase II Trial of the Aurora Kinase A Inhibitor MLN8237, in Relapsed or Refractory Peripheral T-Cell Non-Hodgkin Lymphoma Completed NCT01466881 Phase 2 Alisertib
20 Pilot Study of Prophylactic Dose-Escalation Donor Lymphocyte Infusion After T Cell Depleted Allogeneic Stem Cell Transplant in High Risk Patients With Hematologic Malignancies Completed NCT01839916 Phase 2
21 Impact of Intervention With Deferasirox on the Immune Function of Patients With Hematologic Diseases and Transfusion-Related Iron Overload Completed NCT01273766 Phase 2 deferasirox
22 A Phase II Study of Cyclophosphamide, Etoposide, Vincristine and Prednisone (CEOP) Alternating With Pralatrexate (P) as Front Line Therapy for Patients With Stage II, III and IV Peripheral T-Cell Non-Hodgkin Lymphoma Completed NCT01336933 Phase 2 prednisone;cyclophosphamide;etoposide;Vincristine;pralatrexate
23 A Phase 1b/2a Study of ABT-888 in Combination With Bendamustine +/- Rituximab in Lymphoma, Multiple Myeloma and Solid Tumors Completed NCT01326702 Phase 1, Phase 2 Bendamustine Hydrochloride;Veliparib
24 Campath (Alemtuzumab) Dose Escalation, Low-Dose TBI and Fludarabine Followed by HLA Class II Mismatched Donor Stem Cell Transplantation for Patients With Hematologic Malignancies: A Multicenter Trial Completed NCT00118352 Phase 2 fludarabine phosphate;cyclosporine;mycophenolate mofetil
25 A Multi-Center Study of Nonmyeloablative Conditioning With TBI or Fludarabine/TBI for HLA-matched Related Hematopoietic Cell Transplantation for Treatment of Hematologic Malignancies With Post Grafting Immunosuppression With Tacrolimus and Mycophenolate Mofetil Completed NCT00089011 Phase 2 fludarabine phosphate;mycophenolate mofetil;tacrolimus
26 A Phase 1/2 Study of Pembrolizumab Plus Pralatrexate for Treatment of Relapsed or Refractory Peripheral T-Cell Lymphomas Recruiting NCT03598998 Phase 1, Phase 2 Pralatrexate
27 Phase II Study of Brentuximab Vedotin in Combination With Pembrolizumab in Patients With Recurrent Systemic Peripheral T-Cell Lymphoma (PTCL) Recruiting NCT04795869 Phase 2 Brentuximab Vedotin
28 A Multicenter, Open-Label, First-In-Human, Multiple Expansion Cohort, Phase 1/2 Study to Evaluate the Safety and Efficacy of DR-01 in Adult Subjects With Large Granular Lymphocytic Leukemia or Cytotoxic Lymphomas Recruiting NCT05475925 Phase 1, Phase 2 DR-01
29 A Two Step Approach to Reduced Intensity Allogeneic Hematopoietic Stem Cell Transplantation for High Risk Hematologic Malignancies Recruiting NCT01760655 Phase 2 Fludarabine phosphate;Thiotepa;Cyclophosphamide;Tacrolimus;Mycophenolate mofetil
30 A Phase I/II Trial of CHOEP Chemotherapy Plus Lenalidomide as Front Line Therapy for Patients With Stage II, III and IV Peripheral T-Cell Non-Hodgkin's Lymphoma Active, not recruiting NCT02561273 Phase 1, Phase 2 Cyclophosphamide;Doxorubicin Hydrochloride;Etoposide;Lenalidomide;Prednisone;Vincristine Sulfate
31 Phase II Study of Romidepsin Plus Lenalidomide for Patients With Previously Untreated PTCL Active, not recruiting NCT02232516 Phase 2 romidepsin;lenalidomide
32 A Phase 2 Study of Brentuximab Vedotin Plus Cyclophosphamide, Doxorubicin, Etoposide, and Prednisone (CHEP-BV) Followed by BV Consolidation in Patients With CD30-Positive Peripheral T-Cell Lymphomas Active, not recruiting NCT03113500 Phase 2 Brentuximab Vedotin;Cyclophosphamide;Doxorubicin;Doxorubicin Hydrochloride;Etoposide;Etoposide Phosphate;Prednisone
33 Phase 2 Single-Arm, Open-Label Study of Nivolumab in Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma (PTCL) Terminated NCT03075553 Phase 2
34 A Phase II Study Investigating Treatment of Post-Allogeneic Transplant Progression or Relapse of CLL/SLL/PLL or NHL With Lenalidomide Alone or With Rituximab Terminated NCT01419795 Phase 2 lenalidomide
35 A Phase I-II Trial of Brentuximab Vedotin Plus Rituximab as Frontline Therapy for Patients With CD30+ and/or EBV+ Lymphomas Terminated NCT01805037 Phase 1, Phase 2 brentuximab vedotin
36 A PHASE I/II STUDY OF INTRATUMORAL INJECTION OF IPILIMUMAB IN COMBINATION WITH LOCAL RADIATION IN MELANOMA, NON-HODGKIN LYMPHOMA AND COLORECTAL CARCINOMA Terminated NCT01769222 Phase 1, Phase 2
37 Hematopoietic Stem Cell Transplantation Using Alternate Donor Umbilical Cord Blood Options Withdrawn NCT01652014 Phase 2 cyclophosphamide;fludarabine phosphate;mycophenolate mofetil;tacrolimus
38 Phase I Study of Romidepsin (ISTODAX®) Plus ICE for Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma Completed NCT01590732 Phase 1 Carboplatin;Etoposide;Ifosfamide;Romidepsin
39 Phase I Study of the Aurora Kinase a Inhibitor MLN8237 in Combination With the Histone Deacetylase Inhibitor Vorinostat in Lymphoid Malignancies Completed NCT01567709 Phase 1 Alisertib;Vorinostat
40 A Phase I Study of CD8 Memory T-Cell Donor Lymphocyte Infusion for Relapse of Hematolymphoid Malignancies Following Matched Related Donor Allogeneic Hematopoietic Cell Transplantation Completed NCT01523223 Phase 1
41 Phase I Study of AR-42 in Relapsed Myeloma, Chronic Lymphocytic Leukemia, and Lymphoma Completed NCT01129193 Phase 1 AR-42
42 Phase 1 Trial of Siplizumab and Dose-Adjusted EPOCH-Rituximab (DA-EPOCH-R) in T and NK-Cell Lymphomas Completed NCT01445535 Phase 1 Etoposide;Prednisone;Vincristine;Cyclophosphamide;Doxorubicin
43 Study of the TEM-1 Antibody, MORAb-004 (IND# 103821), in Children With Recurrent or Refractory Solid Tumors Completed NCT01748721 Phase 1
44 Phase I Dose-Escalation Study of Azacitidine (Vidaza) and Bortezomib (Velcade) in T-Cell Lymphoma Completed NCT01129180 Phase 1 azacitidine;bortezomib
45 A Phase 1 Study of Lenalidomide Maintenance Following Allogeneic Hematopoietic Cell Transplantation in Patients With Select High Risk Hematological Malignancies Completed NCT01254578 Phase 1 Lenalidomide
46 Phase I Clinical Trial Evaluating the Toxicity, Pharmacokinetics and Biological Effect of Intravenous Bevacizumab (Avastin TM) in Combination With Escalating Doses of Oral AZD2171 for Patients With Advanced Malignancies Completed NCT00458731 Phase 1 cediranib maleate
47 Phase I Pharmacokinetic Study of Dasatinib (BMS-354825) (NSC-732517) in Patients With Advanced Malignancies and Varying Levels of Liver Dysfunction Completed NCT00608361 Phase 1 Dasatinib
48 A Phase 1/Pharmacokinetic Study of Sunitinib in Patients With Cancer Who Also Have HIV and Are on HAART Therapy Completed NCT00890747 Phase 1 sunitinib malate
49 Phase I Trial of Fludarabine and Methoxyamine (TRC102) for Relapsed or Refractory Hematologic Malignancies Completed NCT01658319 Phase 1 fludarabine phosphate;methoxyamine
50 Phase I Dose-Escalation Study of CPI-613, in Combination With Bendamustine, in Patients With Relapsed or Refractory T-Cell Non-Hodgkin Lymphoma or Classic Hodgkin Lymphoma Active, not recruiting NCT02168140 Phase 1 6,8-bis(benzylthio)octanoic acid;bendamustine hydrochloride

Search NIH Clinical Center for Hepatosplenic T-Cell Lymphoma

Genetic Tests for Hepatosplenic T-Cell Lymphoma

Anatomical Context for Hepatosplenic T-Cell Lymphoma

Organs/tissues related to Hepatosplenic T-Cell Lymphoma:

MalaCards : Spleen, T Cells, Bone Marrow, Liver, Bone, Thyroid, Myeloid

Publications for Hepatosplenic T-Cell Lymphoma

Articles related to Hepatosplenic T-Cell Lymphoma:

(show top 50) (show all 293)
# Title Authors PMID Year
1
Hepatosplenic lymphoma and visceral mast cell tumor in the liver of a dog with synchronous and multiple primary tumors. 62
35909229 2022
2
Homozygous ATM mutation due to germline uniparental isodisomy in patient with T acute lymphoblastic leukemia and hepatosplenic T-cell lymphoma. 62
35644065 2022
3
B-cell lymphoma-2 downregulation is a useful feature supporting a neoplastic phenotype in mature T-cell lymphomas. 62
35452696 2022
4
Hepatosplenic T-cell lymphoma diagnosed using flow cytometry. A single-center study of 12 cases from North India. 62
36149166 2022
5
Expression pattern and diagnostic utility of BCL11B in mature T- and NK-cell neoplasms. 62
35864006 2022
6
Risk of Cancer in Paediatric onset Inflammatory Bowel Diseases: A Nation-wide Study From the epi-IIRN. 62
34791097 2022
7
Hepatosplenic T-cell non-Hodgkin lymphoma cured with tandem autologous and allogeneic stem cell transplantation. 62
35537403 2022
8
Gamma/Delta (γδ) T Cells: The Role of the T-Cell Receptor in Diagnosis and Prognosis of Hematologic Malignancies. 62
35287137 2022
9
Capillaritis as a Skin Manifestation of Hepatosplenic Gamma-Delta T-Cell Lymphoma. 62
35319511 2022
10
Hepatosplenic T Cell Lymphoma: Diagnostic Conundrum. 62
35975114 2022
11
Neuropsychiatric Manifestations of Lymphoma-Associated Cerebral Glucose Hypometabolism Can Be Reversed by Intensive Glucose Supplementation. 62
35356784 2022
12
Successful treatment of hepatosplenic T-cell lymphoma with fludarabine, high-dose cytarabine and subsequent unrelated umbilical cord blood transplantation. 62
34591292 2022
13
Molecular features of non-anaplastic peripheral T-cell lymphoma in children and adolescents. 62
34390161 2021
14
Aberrant Loss of Surface CD3 and TCRγδ Expression in Relapsed Hepatosplenic T-cell Lymphoma. 62
34108288 2021
15
A Cautionary Tale: Florid Splenic γδ T-cell Proliferation and False-Positive T-cell Clonality by PCR Leads to a Grave Misdiagnosis. 62
34158266 2021
16
Hepatosplenic T-cell lymphoma: treatment challenges. 62
34409955 2021
17
CD94 expression patterns in reactive and neoplastic T-cell and NK-cell proliferations. 62
33990003 2021
18
γδ TCR T lymphoblastic leukemia in a child presenting with marked hyperleukocytosis. 62
34306690 2021
19
Hepatosplenic T-cell lymphoma (HSTCL) in patient with Crohn's disease treated with azathioprin. 62
32439154 2021
20
Case of hepatosplenic T-cell lymphoma in a patient with dermatomyositis treated with prednisolone and azathioprine. 62
33866589 2021
21
Identification of Key Genes and Pathways Associated with Hepatosplenic T-Cell Lymphoma (HSTCL) by Bioinformatics Analysis. 62
33978371 2021
22
Outcomes of Hepatosplenic T-Cell Lymphoma: The Mayo Clinic Experience. 62
33160933 2021
23
Survival Analysis of Hepatosplenic T Cell Lymphoma: A Population-Based Study Using SEER. 62
34819748 2021
24
Hepatosplenic T-cell lymphoma with hepatocytotropism in a cat. 62
33959376 2021
25
Hypercalcaemia: An Extremely Rare Presentation of Hepatosplenic T-Cell Lymphoma. 62
33768082 2021
26
The non-leukemic T cell large granular lymphocytic leukemia variant with marked splenomegaly and neutropenia in the setting of rheumatoid arthritis - Felty syndrome and hepatosplenic T cell lymphoma mask. 62
34322285 2021
27
Hepatic involvement by T-cell neoplasms: a clinicopathologic study of 40 cases. 62
33010300 2020
28
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. 62
32776434 2020
29
NCCN Guidelines Insights: T-Cell Lymphomas, Version 1.2021. 62
33152703 2020
30
Pralatrexate-based therapy induced response in an adolescent with refractory hepatosplenic T-cell lymphoma. 62
32860655 2020
31
Hepatosplenic T-cell lymphoma: a rare but challenging entity. 62
32756940 2020
32
Hepatosplenic αβ T-Cell Lymphoma as Second Malignancy in Young Adult Patient With Previously Undiagnosed Ataxia-Telangiectasia. 62
31259827 2020
33
How Does It Look in an Autopsy? Hepatosplenic T-Cell Lymphoma in a Patient with Crohn’s Disease on Azathioprine 62
32270949 2020
34
Intensive Induction Therapy Compared With CHOP for Hepatosplenic T-cell Lymphoma. 62
32284297 2020
35
Hepatosplenic T-Cell Lymphoma in an Immunocompetent Young Male: A Challenging Diagnosis. 62
32775075 2020
36
Causes of double-negative T-cell lymphocytosis in children and adults. 62
31810993 2020
37
Effect of chidamide on treating hepatosplenic T-cell lymphoma: A case report. 62
32775395 2020
38
A case of hepatosplenic T-cell lymphoma successfully treated by HLA haploidentical stem cell transplantation. 62
32404572 2020
39
[Cell morphological analysis of hepatosplenic T-cell lymphoma gamma-delta type]. 62
32536127 2020
40
Hepatosplenic T-Cell Lymphoma Diagnosed by Endoscopic Ultrasound-Guided Fine-Needle Biopsy. 62
32106363 2020
41
A Skin Plaque Preceding Systemic Relapse of Gamma-Delta Hepatosplenic T-Cell Lymphoma. 62
31725483 2020
42
Targeting EZH2 for the treatment of hepatosplenic T-cell lymphoma. 62
32232478 2020
43
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. 62
31990422 2020
44
Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas. 62
31709579 2020
45
Complete Spontaneous Regression of Hepatosplenic T-Cell Lymphoma After Surgical Biopsy. 62
31652159 2020
46
T-cell lymphoproliferative processes in the spleen. 62
31879048 2020
47
Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-α Inhibitors for Ankylosing Spondylitis. 62
31764502 2019
48
Hepatosplenic T-Cell Lymphoma in an Immunocompetent Male with Central Nervous System Invasion: A Rare Clinical Entity. 62
30500103 2019
49
Tetrasomy 8 and isochromosome 7q in CD5-positive hepatosplenic T-cell lymphoma with leukemic presentation. 62
31342408 2019
50
Refractory α/β-Hepatosplenic T-cell Lymphoma With Secondary CNS Dissemination and Severe Morbidity Related to Intrathecal Chemotherapy: A Case Report and Literature Review. 62
31362868 2019

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 TOX-DT TOX divergent transcript 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

Cellular components related to Hepatosplenic T-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016021 10.07 TNFRSF8 TNF NCAM1 DERL2 CD8A CD7
2 membrane GO:0016020 10.07 TNFRSF8 TNF NCAM1 DERL2 CD8A CD7
3 external side of plasma membrane GO:0009897 9.4 TNF NCAM1 CD8A CD5 CD4 CD2

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

# Name GO ID Score Top Affiliating Genes
1 regulation of cell population proliferation GO:0042127 9.8 TNF STAT5B DUSP22 ALK
2 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.76 CD8A CD7 CD4 ALK
3 natural killer cell mediated cytotoxicity GO:0042267 9.43 STAT5B GZMB CD2
4 T cell activation GO:0042110 9.23 CD8A CD7 CD4 CD2

Sources for Hepatosplenic T-Cell Lymphoma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 24-Oct-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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