hepatosplenic T-cell lymphoma
diseaseOn this page
Also known as Hepatosplenic gamma/Delta T-cell lymphomaHSTCL
Summary
hepatosplenic T-cell lymphoma (MONDO:0019474) is a cancer and 68 clinical trials. Top therapeutic interventions include cyclophosphamide anhydrous, dexrazoxane, and doxorubicin. A subtype of T-cell non-Hodgkin lymphoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Prevalence: <1 / 1 000 000 (United States) [Orphanet-validated]
- Clinical trials: 68
Clinical features
Epidemiology
Prevalence records
1 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.03 | United States | Validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | hepatosplenic T-cell lymphoma |
| Mondo ID | MONDO:0019474 |
| Orphanet | 86882 |
| DOID | DOID:0081049 |
| ICD-10-CM | C86.1 |
| ICD-11 | 1301206942 |
| NCIT | C8459 |
| SNOMED CT | 445406001 |
| UMLS | C1333984 |
| MedGen | 272711 |
| GARD | 0019077 |
| MedDRA | 10066957 |
| Is cancer (heuristic) | yes |
Also known as: Hepatosplenic gamma/Delta T-cell lymphoma · HSTCL
Data availability: 2 cell lines.
Disease family
This is a subtype of T-cell non-Hodgkin lymphoma. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › hematopoietic and lymphoid system neoplasm › hematopoietic and lymphoid cell neoplasm › lymphoid neoplasm › lymphoma › non-Hodgkin lymphoma › T-cell non-Hodgkin lymphoma › hepatosplenic T-cell lymphoma
Related subtypes (8): mature T-cell and NK-cell non-Hodgkin lymphoma, primary cutaneous T-cell lymphoma, CD4+/CD56+ hematodermic neoplasm, adult T-cell leukemia/lymphoma, enteropathy-associated T-cell lymphoma, T-lymphoblastic lymphoma, Nodal T-follicular helper cell lymphoma, follicular type, monomorphic epitheliotropic intestinal T-cell lymphoma
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 68.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 23 |
| PHASE1 | 20 |
| Not specified | 11 |
| PHASE1/PHASE2 | 10 |
| PHASE4 | 2 |
| PHASE2/PHASE3 | 1 |
| EARLY_PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT01746992 | PHASE4 | UNKNOWN | CTOP/ITE/MTX Compared With CHOP as the First-line Therapy for Newly Diagnosed Young Patients With T Cell Lymphoma |
| NCT01804166 | PHASE4 | COMPLETED | 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) |
| NCT04021082 | PHASE2/PHASE3 | WITHDRAWN | CELTIC-1: A Phase 2B Study of Cerdulatinib in Patients With Relapsed/Refractory Peripheral T-Cell Lymphoma (PTCL) |
| NCT03113500 | PHASE2 | ACTIVE_NOT_RECRUITING | Brentuximab Vedotin and Combination Chemotherapy in Treating Patients With CD30-Positive Peripheral T-cell Lymphoma |
| NCT03598998 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Pembrolizumab and Pralatrexate in Treating Patients With Relapsed or Refractory Peripheral T-Cell Lymphomas |
| NCT05475925 | PHASE1/PHASE2 | RECRUITING | A Study of DR-01 in Subjects With Large Granular Lymphocytic Leukemia or Cytotoxic Lymphomas |
| NCT00006251 | PHASE1/PHASE2 | COMPLETED | Fludarabine Phosphate, Low-Dose Total-Body Irradiation, and Donor Stem Cell Transplant Followed by Cyclosporine, Mycophenolate Mofetil, Donor Lymphocyte Infusion in Treating Patients With Hematopoietic Cancer |
| NCT00040846 | PHASE2 | COMPLETED | Alemtuzumab, Fludarabine Phosphate, and Low-Dose Total Body Irradiation Before Donor Stem Cell Transplantation in Treating Patients With Hematological Malignancies |
| NCT00049504 | PHASE2 | COMPLETED | Haploidentical Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancer |
| NCT00072514 | PHASE2 | COMPLETED | Gemcitabine Hydrochloride, Carboplatin, Dexamethasone, and Rituximab in Treating Patients With Previously Treated Lymphoid Malignancies |
| NCT00078858 | PHASE1/PHASE2 | COMPLETED | Mycophenolate Mofetil and Cyclosporine in Reducing Graft-Versus-Host Disease in Patients With Hematologic Malignancies or Metastatic Kidney Cancer Undergoing Donor Stem Cell Transplant |
| NCT00089011 | PHASE2 | COMPLETED | Tacrolimus and Mycophenolate Mofetil in Preventing Graft-Versus-Host Disease in Patients Who Have Undergone Total-Body Irradiation With or Without Fludarabine Phosphate Followed by Donor Peripheral Blood Stem Cell Transplant for Hematologic Cancer |
| NCT00118352 | PHASE2 | COMPLETED | Alemtuzumab, Fludarabine Phosphate, and Total-Body Irradiation Followed by Cyclosporine and Mycophenolate Mofetil in Treating Patients Who Are Undergoing Donor Stem Cell Transplant for Hematologic Cancer |
| NCT00131937 | PHASE2 | COMPLETED | Sorafenib Tosylate in Treating Patients With Recurrent Aggressive Non-Hodgkin’s Lymphoma |
| NCT00901147 | PHASE2 | COMPLETED | Study of Bortezomib and Panobinostat in Treating Patients With Relapsed/Refractory Peripheral T-cell Lymphoma or NK/T-cell Lymphoma |
| NCT00918333 | PHASE1/PHASE2 | COMPLETED | Panobinostat and Everolimus in Treating Patients With Recurrent Multiple Myeloma, Non-Hodgkin Lymphoma, or Hodgkin Lymphoma |
| NCT01075321 | PHASE1/PHASE2 | COMPLETED | Everolimus and Lenalidomide in Treating Patients With Relapsed or Refractory Non-Hodgkin or Hodgkin Lymphoma |
| NCT01177371 | PHASE2 | COMPLETED | High-Dose Busulfan and High-Dose Cyclophosphamide Followed By Donor Bone Marrow Transplant in Treating Patients With Leukemia, Myelodysplastic Syndrome, Multiple Myeloma, or Recurrent Hodgkin or Non-Hodgkin Lymphoma |
| NCT01258998 | PHASE2 | COMPLETED | Study of Akt Inhibitor MK2206 in Patients With Relapsed Lymphoma |
| NCT01261247 | PHASE2 | COMPLETED | Panobinostat in Treating Patients With Relapsed or Refractory Non-Hodgkin Lymphoma |
| NCT01273766 | PHASE2 | COMPLETED | Deferasirox in Treating Iron Overload Caused By Blood Transfusions in Patients With Hematologic Malignancies |
| NCT01326702 | PHASE1/PHASE2 | COMPLETED | Veliparib, Bendamustine Hydrochloride, and Rituximab in Treating Patients With Relapsed or Refractory Lymphoma, Multiple Myeloma, or Solid Tumors |
| NCT01336933 | PHASE2 | COMPLETED | Combination Chemotherapy and Pralatrexate as First-Line Therapy in Treating Patients With Non-Hodgkin Lymphoma |
| NCT01419795 | PHASE2 | TERMINATED | Lenalidomide With or Without Rituximab in Treating Patients With Progressive or Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, Prolymphocytic Leukemia, or Non-Hodgkin Lymphoma Previously Treated With Donor Stem Cell Transplant |
| NCT01427881 | PHASE2 | COMPLETED | Cyclophosphamide for Prevention of Graft-Versus-Host Disease After Allogeneic Peripheral Blood Stem Cell Transplantation in Patients With Hematological Malignancies |
| NCT01466881 | PHASE2 | COMPLETED | Alisertib in Treating Patients With Relapsed or Refractory Peripheral T-Cell Non-Hodgkin Lymphoma |
| NCT01529827 | PHASE2 | COMPLETED | Fludarabine Phosphate, Melphalan, and Low-Dose Total-Body Irradiation Followed by Donor Peripheral Blood Stem Cell Transplant in Treating Patients With Hematologic Malignancies |
| NCT01652014 | PHASE2 | WITHDRAWN | Single or Double Donor Umbilical Cord Blood Transplant in Treating Patients With High-Risk Hematologic Malignancies |
| NCT01805037 | PHASE1/PHASE2 | TERMINATED | Brentuximab Vedotin + Rituximab as Frontline Therapy for Pts w/ CD30+ and/or EBV+ Lymphomas |
| NCT01839916 | PHASE2 | COMPLETED | Donor T Cells After Donor Stem Cell Transplant in Treating Patients With Hematologic Malignancies |
| NCT02232516 | PHASE2 | UNKNOWN | Romidepsin and Lenalidomide in Treating Patients With Previously Untreated Peripheral T-Cell Lymphoma |
| NCT02533700 | PHASE2 | UNKNOWN | CEOP/IVE/GDP Compared With CEOP as the First-line Therapy for Newly Diagnosed Adult Patients With PTCL |
| NCT02561273 | PHASE1/PHASE2 | COMPLETED | Combination Chemotherapy & Lenalidomide in Newly Diagnosed Stage II-IV Peripheral T-cell Non-Hodgkin’s Lymphoma |
| NCT02742727 | PHASE1/PHASE2 | UNKNOWN | CAR-pNK Cell Immunotherapy in CD7 Positive Leukemia and Lymphoma |
| NCT03075553 | PHASE2 | TERMINATED | Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma |
| NCT04795869 | PHASE2 | WITHDRAWN | Brentuximab Vedotin and Pembrolizumab in Treating Patients With Recurrent Peripheral T-Cell Lymphoma |
| NCT05377827 | PHASE1 | ACTIVE_NOT_RECRUITING | Dose-Escalation and Dose-Expansion Study to Evaluate the Safety and Tolerability of Anti-CD7 Allogeneic CAR T-Cells (WU-CART-007) in Patients With CD7+ Hematologic Malignancies |
| NCT07055477 | PHASE1 | RECRUITING | A Phase I Trial Anti-CC Chemokine Receptor 4 Chimeric Antigen Receptor T Cells (CCR4 CAR T Cells) for CCR4 Expressing T-cell Malignancies Including Peripheral T-cell Non-Hodgkin Lymphoma (PTCL) and Cutaneous T-cell Non-Hodgkin Lymphoma (CTCL) |
| NCT00101205 | PHASE1 | TERMINATED | Oxaliplatin, Ifosfamide and Etoposide in Treating Young Patients With Recurrent or Refractory Solid Tumors or Lymphoma |
| NCT00458731 | PHASE1 | COMPLETED | Bevacizumab and Cediranib Maleate in Treating Patients With Metastatic or Unresectable Solid Tumor, Lymphoma, Intracranial Glioblastoma, Gliosarcoma or Anaplastic Astrocytoma |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| CYCLOPHOSPHAMIDE ANHYDROUS | 4 | 3 |
| DEXRAZOXANE | 4 | 3 |
| DOXORUBICIN | 4 | 3 |
| PANOBINOSTAT | 4 | 3 |
| ROMIDEPSIN | 4 | 3 |
| ALEMTUZUMAB | 4 | 2 |
| BENDAMUSTINE HYDROCHLORIDE | 4 | 2 |
| BRENTUXIMAB VEDOTIN | 4 | 2 |
| IFOSFAMIDE | 4 | 2 |
| PRALATREXATE | 4 | 2 |
| VINCRISTINE | 4 | 2 |
| DEFERASIROX | 4 | 1 |
| DUVELISIB | 4 | 1 |
| ETOPOSIDE | 4 | 1 |
| ETOPOSIDE PHOSPHATE | 4 | 1 |
| GOLIMUMAB | 4 | 1 |
| INFLIXIMAB | 4 | 1 |
| METHOTREXATE | 4 | 1 |
| PREDNISONE | 4 | 1 |
| SORAFENIB | 4 | 1 |
| ALISERTIB | 3 | 2 |
| VELIPARIB | 3 | 2 |
| 6-O-BENZYLGUANINE | 3 | 1 |
| CEDIRANIB MALEATE | 3 | 1 |
| CPI 613 | 3 | 1 |
| OBATOCLAX MESYLATE | 3 | 1 |
| PIRARUBICIN | 3 | 1 |
| METHOXYAMINE | 2 | 2 |
| AR-42 | 2 | 1 |
| CERDULATINIB | 2 | 1 |
Related Atlas pages
- Drugs: Cyclophosphamide, Dexrazoxane, Doxorubicin, Panobinostat, Romidepsin, Alemtuzumab, Bendamustine, Brentuximab Vedotin, Ifosfamide, Pralatrexate, Vincristine, Deferasirox, Duvelisib, Etoposide, Etoposide Phosphate, Golimumab, Infliximab, Methotrexate, Prednisone, Sorafenib, Alisertib, Veliparib, 6-O-BENZYLGUANINE, Cediranib, CPI 613, Obatoclax, Pirarubicin