primary cutaneous T-cell non-Hodgkin lymphoma

Summary

primary cutaneous T-cell non-Hodgkin lymphoma (MONDO:0000607) is a cancer (an umbrella term covering 5 Mondo subtypes) and 235 clinical trials. Top therapeutic interventions include romidepsin, mogamulizumab, and bexarotene. A subtype of primary cutaneous lymphoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Umbrella term: 5 Mondo subtypes
• Clinical trials: 235

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	primary cutaneous T-cell non-Hodgkin lymphoma
Mondo ID	MONDO:0000607
EFO	EFO:0002913
MeSH	D016410
DOID	DOID:0060061
NCIT	C3467
GARD	0022811
Is cancer (heuristic)	yes

Also known as: CTCL · cutaneous T cell lymphoma · cutaneous T-cell lymphoma · cutaneous T-cell non-Hodgkin lymphoma · cutaneous T-cell non-Hodgkin’s lymphoma · PCTCL · primary cutaneous T-cell non-Hodgkin lymphoma · primary cutaneous T-cell non-Hodgkin’s lymphoma · skin T-cell non-Hodgkin’s lymphoma · T-cell non-Hodgkin’s lymphoma of skin · T-cell non-Hodgkin’s lymphoma of the skin

Data availability: 15 cell lines.

Disease family

An umbrella term covering 5 Mondo subtypes.

Classification path: disease › human disease › disease by body system or component › integumentary system disorder › integumentary system cancer › skin cancer › primary cutaneous lymphoma › primary cutaneous T-cell non-Hodgkin lymphoma

Related subtypes (2): primary cutaneous T-cell lymphoma, primary cutaneous B-cell lymphoma

Subtypes (5): mycosis fungoides, primary cutaneous anaplastic large cell lymphoma, Sezary syndrome, mycosis fungoides variant, primary cutaneous gamma-delta 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

Drugs indicated for this disease

4 approved, 4 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Bexarotene	Approved (phase 4)
Denileukin Diftitox	Approved (phase 4)
Romidepsin	Approved (phase 4)
Vorinostat	Approved (phase 4)
Brentuximab Vedotin	Phase 3 (in late-stage trials)
Hypericin	Phase 3 (in late-stage trials)
Methotrexate	Phase 3 (in late-stage trials)
Mogamulizumab	Phase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Atezolizumab, Belinostat, Curcumin, Dimethyl Fumarate, Enzastaurin, Everolimus, Forodesine, Lenalidomide, Mechlorethamine, Panobinostat, Sapacitabine, Sintilimab, Sirolimus, Tucidinostat, Zanolimumab.

Clinical trials & evidence

Clinical trials

Clinical trials: 235.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE1	80
PHASE2	76
Not specified	40
PHASE1/PHASE2	23
PHASE3	7
PHASE4	5
EARLY_PHASE1	4

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06285370	PHASE4	ACTIVE_NOT_RECRUITING	A Study to Evaluate the Efficacy and Safety of KW-0761 in Chinese Subjects With Mycosis Fungoides or Sézary Syndrome Previously Treated With Systemic Therapy
NCT00221039	PHASE4	COMPLETED	Photopheresis as an Interventional Therapy for the Treatment of CTCL (Cutaneous T-Cell Lymphoma, Mycosis Fungoides) Stage 1A, 1B, 2A
NCT00630903	PHASE4	TERMINATED	PUVA Versus PUVA + IFN Alpha 2a in Mycosis Fungoides
NCT01007448	PHASE4	COMPLETED	Study Evaluating Two Dose Levels of Targretin Capsules in Participants With Refractory Cutaneous T-Cell Lymphoma (CTCL)
NCT01569724	PHASE4	COMPLETED	Carbohydrate Metabolism Disorder Frequency in Hypertriglyceridemia Induced by Bexarotene of Cutaneous T Cell Lymphoma
NCT05205902	PHASE3	NOT_YET_RECRUITING	TOtal Skin Electron Beam Therapy (Low-dose) for Tumor Clone Eradication in Early-stage Mycosis Fungoides
NCT06470451	PHASE3	RECRUITING	Confirmatory Study of Topical HyBryte™ vs. Placebo for the Treatment of CTCL
NCT07178457	PHASE3	NOT_YET_RECRUITING	Brentuximab Vedotin in CutAneous T-cell Lymphomas (CTCL): Post-allogeneic Hematopoietic Stem Cell Transplant Maintenance
NCT00799461	PHASE3	COMPLETED	Internet-Based Program With or Without Telephone-Based Problem-Solving Training in Helping Long-Term Survivors of Hematopoietic Stem Cell Transplant Cope With Late Complications
NCT01578499	PHASE3	COMPLETED	A Phase 3 Trial of Brentuximab Vedotin(SGN-35) Versus Physician’s Choice (Methotrexate or Bexarotene) in Participants With CD30-Positive Cutaneous T-Cell Lymphoma (ALCANZA Study)
NCT01728805	PHASE3	COMPLETED	Study of KW-0761 Versus Vorinostat in Relapsed/Refractory CTCL
NCT02448381	PHASE3	COMPLETED	FLASH [Fluorescent Light Activated Synthetic Hypericin] Clinical Study: Topical SGX301 (Synthetic Hypericin) for the Treatment of Cutaneous T-Cell Lymphoma (Mycosis Fungoides)
NCT02978625	PHASE2	ACTIVE_NOT_RECRUITING	Talimogene Laherparepvec and Nivolumab in Treating Patients With Refractory Lymphomas or Advanced or Refractory Non-melanoma Skin Cancers
NCT03011814	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Durvalumab With or Without Lenalidomide in Treating Patients With Relapsed or Refractory Cutaneous or Peripheral T Cell Lymphoma
NCT03278782	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Study of Pembrolizumab (MK-3475) in Combination With Romidepsin
NCT03385226	PHASE2	ACTIVE_NOT_RECRUITING	A Trial Assessing the Effect of Pembrolizumab Combined with Radiotherapy in Patients with Relapsed, Refractory, Specified Stages of Cutaneous T-cell Lymphoma (CTCL) Mycosis Fungoides (MF)/Sezary Syndrome (SS)
NCT04930653	PHASE2	RECRUITING	Extracorporeal Photopheresis and Mogamulizumab for the Treatment of Erythrodermic Cutaneous T Cell Lymphoma
NCT05680558	PHASE2	RECRUITING	Photopheresis in Early-stage Mycosis Fungoides
NCT05956041	PHASE2	RECRUITING	Pembrolizumab and Mogamulizumab in Advanced-stage, Relapsed/Refractory Cutaneous T-cell Lymphomas
NCT06485219	PHASE2	ACTIVE_NOT_RECRUITING	A Phase IIa Clinical Study of Purinostat Mesylate for Injection in Patients With Peripheral T-Cell Lymphoma and Cutaneous T-Cell Lymphoma
NCT06698822	PHASE2	RECRUITING	A Phase 2 Trial to Assess Safety and Efficacy of Tofacitinib 2% Cream in the Treatment of Cutaneous T-cell Lymphoma (CTCL), Stages IA, IB, and IIA
NCT06716658	PHASE2	RECRUITING	JAK1 Inhibitor Golidocitnib for the Treatment of Relapsed/Refractory Indolent T/NK-cell Lymphomas
NCT06810778	PHASE1/PHASE2	RECRUITING	Duvelisib and Venetoclax in Patients With Relapsed or Refractory Peripheral T-cell Lymphoma (PTCL)
NCT06854653	PHASE2	RECRUITING	A Phase 2 Study of PTX 100 in Patients With Relapsed/Refractory CTCL
NCT07283822	PHASE2	RECRUITING	Amping up With PemJAK
NCT07356245	PHASE2	RECRUITING	Ruxolitinib Maintenance Post-Hematopoietic Stem Cell Transplant T-Cell Lymphoma
NCT07535710	PHASE1/PHASE2	NOT_YET_RECRUITING	Aclarubicin Plus Cyclophosphamide, Vincristine, and Prednisone (CAOP) in Patients With Previously Treated Cutaneous T-cell Lymphoma
NCT00003210	PHASE2	COMPLETED	Interleukin-12 in Treating Patients With Previously Treated Non-Hodgkin’s Lymphoma or Hodgkin’s Disease
NCT00005080	PHASE2	COMPLETED	506U78 in Treating Patients With Lymphoma
NCT00005982	PHASE2	TERMINATED	506U78 in Treating Patients With Recurrent or Refractory Cutaneous T-cell Lymphoma
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
NCT00007345	PHASE2	COMPLETED	Depsipeptide to Treat Patients With Cutaneous T-Cell Lymphoma and Peripheral T-Cell Lymphoma
NCT00038025	PHASE2	COMPLETED	A Study Of Deoxycoformycin(DCF)/Pentostatin In Lymphoid Malignancies
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
NCT00052377	PHASE1/PHASE2	TERMINATED	Interleukin-12 and Interleukin-2 in Treating Patients With Mycosis Fungoides
NCT00071071	PHASE2	COMPLETED	Clinical Trial of HuMax-CD4, a New Drug to Treat Early Stage T-Cell Lymphoma in the Skin.
NCT00071084	PHASE2	COMPLETED	Clinical Trial of HuMax-CD4, a New Drug to Treat Advanced Stage T-Cell Lymphoma in the Skin.
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

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
ROMIDEPSIN	4	14
MOGAMULIZUMAB	4	9
BEXAROTENE	4	7
BRENTUXIMAB VEDOTIN	4	7
BELINOSTAT	4	5
PANOBINOSTAT	4	5
VORINOSTAT	4	4
BENDAMUSTINE HYDROCHLORIDE	4	3
PRALATREXATE	4	3
ALEMTUZUMAB	4	2
CYANOCOBALAMIN	4	2
DEFERASIROX	4	2
NELARABINE	4	2
ROSIGLITAZONE	4	2
BECLOMETHASONE DIPROPIONATE	4	1
CEFEPIME HYDROCHLORIDE	4	1
CEMIPLIMAB	4	1
COPANLISIB HYDROCHLORIDE	4	1
DENILEUKIN DIFTITOX	4	1
DEXRAZOXANE	4	1
DEXRAZOXANE HYDROCHLORIDE	4	1
DIMETHYL FUMARATE	4	1
DUVELISIB	4	1
FOSCARNET	4	1
FOSCARNET SODIUM	4	1
GANCICLOVIR	4	1
ISOTRETINOIN	4	1
LETERMOVIR	4	1
LITHIUM CARBONATE	4	1
MECHLORETHAMINE	4	1