SPTCL
MCID: TCL023
MIFTS: 50

T-Cell Lymphoma, Subcutaneous Panniculitis-Like (SPTCL)

Categories: Blood diseases, Cancer diseases, Genetic diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

MalaCards integrated aliases for T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

Name: T-Cell Lymphoma, Subcutaneous Panniculitis-Like 56 73
Subcutaneous Panniculitis-Like T-Cell Lymphoma 52 58 6 17 32
Sptcl 56 52 58 73
Subcutaneous Panniculitic T-Cell Lymphoma 58
Lymphoma, T-Cell 43

Characteristics:

Orphanet epidemiological data:

58
subcutaneous panniculitis-like t-cell lymphoma
Inheritance: Not applicable; Age of onset: All ages;

OMIM:

56
Inheritance:
autosomal recessive

Miscellaneous:
variable severity
variable age at onset (median 36 years)
immunosuppressive drugs and hematopoietic bone marrow transplant are effective treatments


HPO:

31
t-cell lymphoma, subcutaneous panniculitis-like:
Inheritance autosomal recessive inheritance
Onset and clinical course variable expressivity


Classifications:

Orphanet: 58  
Rare skin diseases
Rare haematological diseases


External Ids:

OMIM 56 618398
ICD10 32 C86.3
MESH via Orphanet 44 C537503
ICD10 via Orphanet 33 C83.6
UMLS via Orphanet 72 C0522624
Orphanet 58 ORPHA86884
MedGen 41 C0522624

Summaries for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

NIH Rare Diseases : 52 Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of lymphoma that mainly affects the skin. Lymphomas are cancers that result from a type of white blood cell that grows out of control. In SPTCL, people develop multiple painless nodules in the fat layer under the skin and can these cause swelling. This is known as panniculitis. The cause of this type of cancer is unknown, but genetic factors may be involved. It can be diagnosed by a skin biopsy of the affected area. Treatment for SPTCL depends on the symptoms but often involves taking a steroid medication or a medication that lessens the immune response. Some people with more aggressive SPTCL may need chemotherapy . SPTCL can occur in children or adults, but is typically diagnosed in the 30s. For unknown reasons, it occurs more often in women than men. SPTCL is a very slow-growing cancer and does not usually spread beyond the subcutaneous fat layer. About 20% of people with SPTCL will also develop another condition called hemophagocytic lymphohistiocytosis (HLH) in which the body makes too many immune cells. These immune cells attack other cells in the body and cause an enlarged spleen and a decreased level of blood cells in the body.

MalaCards based summary : T-Cell Lymphoma, Subcutaneous Panniculitis-Like, also known as subcutaneous panniculitis-like t-cell lymphoma, is related to panniculitis and peripheral t-cell lymphoma. An important gene associated with T-Cell Lymphoma, Subcutaneous Panniculitis-Like is HAVCR2 (Hepatitis A Virus Cellular Receptor 2), and among its related pathways/superpathways are NF-kappaB Signaling and IL12-mediated signaling events. The drugs Denileukin diftitox and Epirubicin have been mentioned in the context of this disorder. Affiliated tissues include t cells, b cells and skin, and related phenotypes are panniculitis and erythematous papule

OMIM : 56 Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon form of T-cell non-Hodgkin lymphoma in which cytotoxic CD8 (see 186910)+ T cells infiltrate adipose tissue forming subcutaneous nodules. Both children and adults can be affected, with a median age at diagnosis of 36 years and a female gender bias. Most patients have accompanying systemic features such as fever or flank pain. A subset (about 20%) of patients develop hemophagocytic lymphohistiocytosis (HLH), usually associated with CD8+ T cells rimming adipocytes in the bone marrow. An infectious agent is not identified, and the disorder is believed to result from improperly activated inflammation. Immunosuppressive therapy may be helpful; hematopoietic bone marrow transplantation is usually curative (summary by Gayden et al., 2018). For a general discussion of genetic heterogeneity of HLH, see HLH1 (267700). (618398)

UniProtKB/Swiss-Prot : 73 T-cell lymphoma, subcutaneous panniculitis-like: An uncommon form of T-cell non-Hodgkin lymphoma, in which cytotoxic CD8+ T-cells infiltrate subcutaneous adipose tissue, and rimming adipocytes in a lace-like pattern. Affected individuals typically present with multiple subcutaneous nodules, systemic B-cell symptoms, and, in a subset of cases, autoimmune disorders, most commonly systemic lupus erythematosus. A subset of patients develop hemophagocytic lymphohistiocytosis. SPTCL transmission pattern is consistent with autosomal recessive inheritance with incomplete penetrance.

Related Diseases for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Diseases related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 172)
# Related Disease Score Top Affiliating Genes
1 panniculitis 30.8 TRG TNFRSF8 TIA1 HAVCR2 GZMB
2 peripheral t-cell lymphoma 30.6 TNFRSF8 TIA1 GZMB ALK
3 alk-positive anaplastic large cell lymphoma 30.5 TNFRSF8 ALK
4 pediatric lymphoma 30.4 TNFRSF8 ALK
5 composite lymphoma 30.4 TNFRSF8 TIA1 GZMB
6 lymphomatoid granulomatosis 30.2 TIA1 GZMB
7 anaplastic large cell lymphoma 30.1 TNFRSF8 TIA1 GZMB ALK
8 lymphoma, hodgkin, classic 30.0 TNFRSF8 TIA1 GZMB ALK
9 cutaneous t cell lymphoma 30.0 TRB TNFRSF8 TIA1 GZMB
10 lymphoma 30.0 TNFRSF8 TIA1 NFKB2 HAVCR2 GZMB ALK
11 lymphoblastic lymphoma 29.9 TRB TNFRSF8 CD7
12 angioimmunoblastic t-cell lymphoma 29.8 TNFRSF8 TIA1 GZMB
13 sezary's disease 29.7 TRB TNFRSF8 CD7
14 lymphomatoid papulosis 29.6 TNFRSF8 TIA1 GZMB ALK
15 b-cell lymphoma 29.5 TNFRSF8 TIA1 NFKB2 ALK
16 histiocytosis 29.4 TNFRSF8 ALK
17 hepatosplenic t-cell lymphoma 29.3 TIA1 GZMB CD7
18 primary cutaneous anaplastic large cell lymphoma 29.3 TRG TIA1 GZMB ALK
19 precursor t-cell acute lymphoblastic leukemia 29.1 TRG TRB CD7
20 lymphoma, non-hodgkin, familial 29.1 TNFRSF8 TIA1 GZMB CD7 ALK
21 mycosis fungoides 28.7 TRG TNFRSF8 TIA1 GZMB CD7 ALK
22 t-cell adult acute lymphocytic leukemia 28.7 TNFRSF8 TIA1 CD7 ALK
23 t-cell/histiocyte rich large b cell lymphoma 11.8
24 t-cell non-hodgkin lymphoma 11.8
25 adult t-cell leukemia 11.8
26 lupus erythematosus panniculitis 10.7
27 pancytopenia 10.7
28 hemophagocytic lymphohistiocytosis 10.6
29 rapidly involuting congenital hemangioma 10.6
30 autoimmune disease 10.6
31 primary cutaneous t-cell lymphoma 10.5
32 hydrops, lactic acidosis, and sideroblastic anemia 10.4
33 exanthem 10.4
34 dermatomyositis 10.4
35 disseminated intravascular coagulation 10.4
36 cellulitis 10.4
37 primary cutaneous lymphoma 10.4
38 cytophagic histiocytic panniculitis 10.4
39 hypertriglyceridemia, familial 10.4
40 ovarian cancer 10.4
41 cutaneous lupus erythematosus 10.4
42 pyoderma 10.4
43 pyoderma gangrenosum 10.4
44 vasculitis 10.4
45 splenomegaly 10.4
46 rare tumor 10.4
47 lichen sclerosus et atrophicus 10.3 TIA1 GZMB
48 lymphocytic gastritis 10.3 TIA1 GZMB
49 nocardiosis 10.3
50 exophthalmos 10.3

Graphical network of the top 20 diseases related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:



Diseases related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Symptoms & Phenotypes for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Human phenotypes related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

58 31 (show all 19)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 panniculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0012490
2 erythematous papule 58 31 hallmark (90%) Very frequent (99-80%) HP:0030350
3 fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012378
4 fever 58 31 frequent (33%) Frequent (79-30%) HP:0001945
5 skin ulcer 58 31 frequent (33%) Frequent (79-30%) HP:0200042
6 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
7 hepatosplenomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0001433
8 abnormality of the coagulation cascade 58 31 frequent (33%) Frequent (79-30%) HP:0003256
9 chills 58 31 frequent (33%) Frequent (79-30%) HP:0025143
10 hemophagocytosis 58 31 very rare (1%) Frequent (79-30%) HP:0012156
11 erythematous plaque 58 31 frequent (33%) Frequent (79-30%) HP:0025474
12 splenomegaly 31 very rare (1%) HP:0001744
13 anemia 31 very rare (1%) HP:0001903
14 pancytopenia 31 very rare (1%) HP:0001876
15 autoimmunity 31 very rare (1%) HP:0002960
16 facial edema 31 very rare (1%) HP:0000282
17 hypertriglyceridemia 31 HP:0002155
18 increased serum ferritin 31 HP:0003281
19 hypofibrinogenemia 31 HP:0011900

Symptoms via clinical synopsis from OMIM:

56
Metabolic Features:
fever

Hematology:
anemia (in some patients)
pancytopenia (in some patients)

Abdomen Spleen:
splenomegaly (in some patients)

Neoplasia:
t-cell non-hodgkin lymphoma, subcutaneous

Skin Nails Hair Skin:
panniculitis
erythematous plaques
hemophagocytosis (in some patients)
subcutaneous nodules, diffuse
atypical cytotoxic cd8+ t cells rimming adipocytes

Head And Neck Face:
facial swelling (in some patients)

Immunology:
autoimmunity (in some patients)
increased levels of inflammatory cytokines, including tnf-alpha, il2, and il1b
hemophagocytic lymphohistiocytosis (hlh) (in some patients)
atypical cytotoxic cd8+ t cells rimming adipocytes in bone marrow (in some patients)

Laboratory Abnormalities:
increased triglycerides (in patients with hlh)
hypofibrinogenemia (in patients with hlh)
hyperferritinemia (in patients with hlh)

Clinical features from OMIM:

618398

Drugs & Therapeutics for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

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

(show top 50) (show all 288)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Denileukin diftitox Approved, Investigational Phase 4 173146-27-5
2
Epirubicin Approved Phase 4 56420-45-2 41867
3
Vindesine Approved, Investigational Phase 4 53643-48-4, 59917-39-4 40839
4
Infliximab Approved Phase 4 170277-31-3
5
Methoxsalen Approved Phase 4 298-81-7 4114
6
Aspartic acid Approved, Nutraceutical Phase 4 56-84-8 5960
7
Tyrosine Approved, Investigational, Nutraceutical Phase 4 60-18-4 6057
8
Pirarubicin Investigational Phase 4 72496-41-4
9 Clorazepate Dipotassium Phase 4
10 N-Methylaspartate Phase 4
11 Antibodies, Blocking Phase 4
12 Interferon-alpha Phase 4
13 Interferon alpha-2 Phase 4
14 interferons Phase 4
15 Photosensitizing Agents Phase 4
16
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
17
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
18
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
19
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
20 Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
21
Daunorubicin Approved Phase 3 20830-81-3 30323
22
Mercaptopurine Approved Phase 3 50-44-2 667490
23
Thioguanine Approved Phase 3 154-42-7 2723601
24
Bleomycin Approved, Investigational Phase 3 11056-06-7 5360373
25
Dacarbazine Approved, Investigational Phase 3 4342-03-4 5351166
26
Vinblastine Approved Phase 3 865-21-4 13342 241903
27
Darbepoetin alfa Approved, Investigational Phase 3 209810-58-2, 11096-26-7
28
Ifosfamide Approved Phase 3 3778-73-2 3690
29
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
30
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
31
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
32
Naloxone Approved, Vet_approved Phase 3 465-65-6 5284596
33
Levoleucovorin Approved, Investigational Phase 3 68538-85-2
34
Lenalidomide Approved Phase 3 191732-72-6 216326
35
Azacitidine Approved, Investigational Phase 3 320-67-2 9444
36
Decitabine Approved, Investigational Phase 3 2353-33-5 451668
37
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
38
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
39
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
40
Vincristine Approved, Investigational Phase 2, Phase 3 2068-78-2, 57-22-7 5978
41
Oxaliplatin Approved, Investigational Phase 3 61825-94-3 5310940 9887054 6857599 43805
42
Methylcobalamin Approved, Investigational Phase 3 13422-55-4
43
Hydroxocobalamin Approved Phase 3 13422-51-0 11953898 15589840
44
Etoposide Approved Phase 2, Phase 3 33419-42-0 36462
45
Pegaspargase Approved, Investigational Phase 3 130167-69-0
46
Cyanocobalamin Approved, Nutraceutical Phase 3 68-19-9 44176380
47
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
48
Hypericin Investigational Phase 3 548-04-9 5281051
49
Cobalamin Experimental Phase 3 13408-78-1 6857388
50 Methylprednisolone Acetate Phase 3

Interventional clinical trials:

(show top 50) (show all 589)
# Name Status NCT ID Phase Drugs
1 Comparison of Methotrexate Versus Interferon-alfa 2b on Efficacy, Safety and Quality of Life in Patients With Primary Cutaneous T-cell Lymphomas Unknown status NCT02323659 Phase 4 Methotrexate;Interferon Alfa-2b
2 A Randomized Controlled Multi-center Clinical Trial on Treatment of Stage Ⅲ/Ⅳ NK/T Cell Lymphoma With DDGP Regiment (Gemcitabine,Pegaspargase,Cisplatin,Dexamethasone) Unknown status NCT01501149 Phase 4 DDGP(cisplatin,dexamethasone,gemcitabine,pegaspargase);Modified SMILE (MTX,DEX,IFO,L-ASP,Etoposide,Mesna)
3 A Randomized Controlled Multi-center Clinical Trial on Treatment of Stage I/II NK/T Cell Lymphoma With DDGP Regiment (Gemcitabine,Pegaspargase,Cisplatin,Dexamethasone) Unknown status NCT01501136 Phase 4
4 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
5 A Multicenter Open-Label Study to Evaluate the Safety and Efficacy of DAB389IL-2 in Cutaneous T-Cell Lymphoma (CTCL) Patients Following Protocol 93-04-10, Protocol 93-04-11, or Protocol 92-04-01 or Who Meet the Requirements for Protocol 93-04-11 Except Have Biopsy-Documented CTCL That Does Not Express CD25 Completed NCT00051012 Phase 4 ONTAK
6 A Multicenter Phase III Randomized Double-Blind Placebo-Controlled Study to Evaluate the Efficacy of Two Dose Levels of DAB389IL-2 (9 and 18 Mcg/kg/Day) in Cutaneous T-Cell Lymphoma (CTCL) Patients With Stage Ia-III Disease Who, Following Less Than or Equal to 3 Previous Therapies, Have Recurrent or Persistent Disease That Has Been Biopsy-Documented to Express CD25 Completed NCT00050999 Phase 4 ONTAK
7 Open Label Study of ONTAK® (Denileukin Diftitox, DAB389IL-2) to Estimate Response in Cutaneous T-Cell Lymphoma (CTCL) According to CD25 Status Completed NCT00211198 Phase 4 ONTAK (denileukin difitox, DAB389IL-2)
8 Phase IV Randomized Study of Two Dose Levels of Targretin® Capsules in Subjects With Refractory Cutaneous T-Cell Lymphoma Completed NCT01007448 Phase 4 Bexarotene
9 Estimation of Carbohydrate Metabolism Disorder Frequency in Hypertriglyceridemia Induced by Bexarotene Treatment of Cutaneous T Cell Lymphoma - Pilote Study Completed NCT01569724 Phase 4
10 A Randomized Controlled Multi-center Clinical Trial on Treatment of Peripheral T-cell Lymphoma With DGPT Regiment (Gemcitabine,Cisplatin,Prednisone ,Thalidomide ) Completed NCT01664975 Phase 4 GDPT regimen;CHOP regimen
11 UVADEX Sterile Solution in Conjunction With the UVAR XTS Photopheresis System as an Interventional Therapy for the Treatment Of CTCL (Mycosis Fungoides) in Patients With TMN Classification Stage 1A, 1B, 2A Completed NCT00221039 Phase 4 Methoxsalen+ECP
12 A Randomized Controlled Clinical Trial of Apatinib Combined With CHOP Regimen in the Treatment of Newly Diagnosed Peripheral T-cell Lymphoma Recruiting NCT03631862 Phase 4 Apatinib;CHOP Regimen
13 Single-arm, Multi-center Clinical Study of PD-1 Antibody, Chidamide, Lenalidomide and Etoposide for Peripheral T-cell Lymphoma Recruiting NCT04040491 Phase 4 PD-1 blocking antibody, chidamide, lenalidomide and etoposide
14 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) Recruiting NCT01804166 Phase 4 Infliximab;Golimumab
15 A Multi-Center, Open-Labelled, Pralatrexate Study in Asian Patients With Peripheral T-cell Lymphoma After Prior Therapy Recruiting NCT03150602 Phase 4 Pralatrexate
16 Single-arm, Multi-center Clinical Study of PD-1 Antibody, Chidamide, Lenalidomide and Etoposide for Relapsed or Refractory Natural Killer/T Cell Lymphoma Recruiting NCT04038411 Phase 4 PD-1 Antibody, chidamide, lenalidomide and etoposide
17 Combination Chemotherapy Including Cisplatin, Ifosfamide, Gemcitabine, L-asparaginase, Etoposide and Dexamethasone (PIGLETS Regimen) as Treatment of Newly Diagnosed and Relapsed/Refractory Peripheral T Cell Lymphomas (PTCLs) Recruiting NCT03071822 Phase 4 cisplatin;ifosfamide;gemcitabine;L-asparaginase;etoposide;dexamethasone
18 A Single-arm Study of Brentuximab Vedotin in Patients With Relapsed or Refractory Hodgkin Lymphoma Who Are Not Suitable for Stem Cell Transplantation or Multiagent Chemotherapy Active, not recruiting NCT01990534 Phase 4 Brentuximab vedotin
19 Prospective, Randomized Multicentic to Compare PUVA+IFN Alpha 2a vs PUVA Alone in Mycosis Fungoides Stages Ia, Ib or IIa. Terminated NCT00630903 Phase 4 PUVA (8MOP + UVA) + IFN;PUVA (8-MOP + UVA)
20 Phase III Study of CHOP-14 Plus or Minus Alemtuzumab in Peripheral T-cell Lymphoma of the Elderly Unknown status NCT00725231 Phase 3 chemotherapy
21 Compared the Efficacy and Safety of CDOP Combined With Chidamide and CDOP in de Novo Peripheral T Cell Lymphoma Patients Unknown status NCT03023358 Phase 3 chidamide;cyclophosphamide;liposomal doxorubicin;vincristine;prednisone
22 Non-Hodgkin's Lymphoma T Cell Protocol Unknown status NCT00003423 Phase 3 asparaginase;cyclophosphamide;cytarabine;daunorubicin hydrochloride;dexamethasone;etoposide;mercaptopurine;methotrexate;prednisolone;thioguanine;vincristine sulfate
23 A Prospective , Multicenter, RandomizedPhase III Study of Improving the Efficacy of Treatment in High Risk T Cell Lymphoma Patients Unknown status NCT01788137 Phase 3 chemotherapy (CHOP);chemotherapy(c-ATT)
24 A Phase II Study of Oral LBH589 in Adult Patients With Refractory Cutaneous T-Cell Lymphoma Completed NCT00425555 Phase 2, Phase 3 Panobinostat
25 A Phase II Study of Oral LBH589 in Adult Patients With Cutaneous T-Cell Lymphoma Who Are Intolerant to or Have Progressed on or After Prior HDAC Inhibitor Completed NCT00490776 Phase 2, Phase 3 LBH589
26 A Randomized Phase III Study to Evaluate the Efficacy of Chemoimmunotherapy With the Monoclonal Antibody Campath-1H (Alemtuzumab) Given in Combination With 2-weekly CHOP Versus 2-weekly CHOP Alone and Consolidated by Autologous Stem Cell Transplant, in Young Patients With Previously Untreated Systemic Peripheral T-cell Lymphomas Completed NCT00646854 Phase 3 CHOP14 chemotherapy (cyclophosphamide, hydroxydaunorubicin, vincristin, prednison) plus G-CSF, combined with alemtuzumab;CHOP14 chemotherapy (see specification under Arm B) plus G-CSF
27 Multicentric Study About Treatment of High Grade Peripheral T Cell Lymphoma in Adults. LTP Study Comparison Between VIP ABVD Versus CHOP Completed NCT00970385 Phase 3 CHOP21;VIP/ABVD
28 An Open-Label Therapeutic Exploratory Clinical Trial of HuMax-CD4, a Fully Human Monoclonal Anti-CD4 Antibody, in Patients With Refractory or Relapsed Non-Cutaneous CD4+ T-Cell Lymphoma Completed NCT00877656 Phase 2, Phase 3
29 A Phase 3, Randomized, Two-Arm, Open-Label, Multicenter, International Trial of Alisertib (MLN8237) or Investigator's Choice (Selected Single Agent) in Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma Completed NCT01482962 Phase 3 Alisertib;Pralatrexate;Gemcitabine;Romidepsin
30 A Randomized, Open-Label, Phase 3 Trial of Brentuximab Vedotin(SGN-35) Versus Physician's Choice (Methotrexate or Bexarotene) in Patients With CD30-Positive Cutaneous T-Cell Lymphoma Completed NCT01578499 Phase 3 Brentuximab Vedotin;Methotrexate;Bexarotene
31 A Multi-center, Single Arm, Safety and Efficacy Study of Pralatrexate With Vitamin B12 and Folic Acid Supplementation in Subjects With Relapsed or Refractory Peripheral T-cell Lymphoma Completed NCT03349333 Phase 3 pralatrexate
32 INSPIRE: An Internet-based RCT for Long-term Survivors of Hematopoietic Stem Cell Transplantation Completed NCT00799461 Phase 3
33 A Randomized, Open-Label, Multicenter Study Of Darbepoetin Alfa Administered Once Every Two Weeks (Q2W) Compared With rHuEPO Administered Once Every Week (QW) For The Treatment Of Anemia In Subjects With Non-Myeloid Malignancies Receiving Multiple Chemotherapy Completed NCT00070382 Phase 3 darbepoetin alfa;epoetin alfa
34 Randomized Phase 3 Study Evaluation the Efficacy and Safety of Oral Azacitidine(CC-486) Compared to Investigator's Choice Therapy in Patients With Relapsed or Refractory Angioimmunoblastic T Cell Lymphoma Recruiting NCT03703375 Phase 3 Azacitidine;Romidepsin;Gemcitabine
35 The Efficacy and Safety of Etoposide, Dexamethasone, Peg-asparaginase or Plus Methotrexate With Sandwiched Radiotherapy in the Treatment of Stage I to II Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type Recruiting NCT02631239 Phase 3 Methotrexate;Etoposide;Dexamethasone;Pegaspargase
36 Open-label Multi-center Randomized Non-inferiority Study to Compare Efficacy and Safety of Pegylated Liposomal Doxorubicin Versus Doxorubicin for Newly Diagnosed Peripheral T-cell Lymphoma Recruiting NCT03952572 Phase 3 Cyclophosphamide;pegylated liposomal doxorubicin;Vincristine;Prednisone;Doxorubicin
37 Randomized Phase 3 Study Evaluating the Efficacy and the Safety of Oral Azacitidine (CC-486) Compared to Investigator's Choice Therapy in Patient With Relapsed or Refractory Angioimmunoblastic T Cell Lymphoma Recruiting NCT03593018 Phase 3 Oral azacitidine;Romidepsin;Bendamustine;Gemcitabine
38 The Efficacy and Safety of Gemcitabine, Etoposide, Dexamethasone and Pegaspargase Chemotherapy (GELAD) With Sandwiched Radiotherapy in the Treatment of Stage IE/II Natural Killer/T-Cell Lymphoma: A Multicenter, Prospective Study Recruiting NCT02733458 Phase 3 GELAD
39 A Double Blind Randomized Vehicle Controlled Crossover Study to Evaluate the Safety and Efficacy of Topical Naloxone Hydrochloride Lotion 0.5% for the Relief of Pruritus in Patients With the MF or SS Forms of Cutaneous T-Cell Lymphoma Recruiting NCT02811783 Phase 3 Naloxone Hydrochloride Lotion, 0.5%;Placebo Lotion
40 PA-Gemox Regimen Followed by Thalidomide Versus AspaMetDex Regimen in NKTCL Patients:a Randomized, Open-label, Phase 3 Study Recruiting NCT02085655 Phase 3 pegaspargase;Gemcitabine;Oxaliplatin;Methotrexate;Dexamethasone;Thalidomide
41 Combination Chemotherapy Using Cisplatin, Gemcitabine, Ifosfamide, Etoposide, L-asparaginase and Dexamethasone (SIMPLE) for Newly Diagnosed and Relapsed/Refractory NK/T Cell Malignancies Recruiting NCT03623087 Phase 3 Cisplatin;Gemcitabine;Etoposide (VP-16);Dexamethasone;L-asparaginase
42 A Phase 3 Multicenter, Randomized, Double-Blind, Placebo Controlled Study to Determine the Efficacy of Topical SGX301 (Synthetic Hypericin) and Fluorescent Bulb-Light Irradiation for the Treatment of Cutaneous T-Cell Lymphoma Active, not recruiting NCT02448381 Phase 3 SGX301 (synthetic hypericin);Placebo
43 Open-Label, Multi-Center, Randomized Study of Anti-CCR4 Monoclonal Antibody KW-0761 (Mogamulizumab) Versus Vorinostat in Subjects With Previously Treated Cutaneous T-Cell Lymphoma Active, not recruiting NCT01728805 Phase 3 Vorinostat
44 A Randomized, Double-blind, Placebo-controlled, Phase 3 Study of Brentuximab Vedotin and CHP (A+CHP) Versus CHOP in the Frontline Treatment of Patients With CD30-positive Mature T-cell Lymphomas Active, not recruiting NCT01777152 Phase 3 brentuximab vedotin;doxorubicin;prednisone;vincristine;cyclophosphamide
45 A Clinical Study to Demonstrate Safety and Efficacy of E7777 in Persistent or Recurrent Cutaneous T-Cell Lymphoma Active, not recruiting NCT01871727 Phase 3 E7777 9 mcg/kg
46 Phase 3 Multi-center Randomized Study to Compare Efficacy and Safety of Romidepsin CHOP (Ro-CHOP) Versus CHOP in Patients With Previously Untreated Peripheral T-cell Lymphoma Active, not recruiting NCT01796002 Phase 3 Romidepsin + CHOP;CHOP
47 Phase 3 Randomized, Double-Blind, Placebo Controlled, Multicenter Study to Compare the Efficacy and Safety of Lenalidomide (CC-5013) Plus R-CHOP Chemotherapy (R2-CHOP) Versus Placebo Plus R-CHOP Chemotherapy in Subjects With Previously Untreated Activated B-cell Type Diffuse Large B-cell Lymphoma Active, not recruiting NCT02285062 Phase 3 lenalidomide;Placebo;Rituximab;Cyclophosphamide;Doxorubicin;prednisone;vincristine
48 CELTIC-1 (Clinical Evaluation of T-cell NHL With Cerdulatinib): A Phase 2b, Open Label, Multidose, Multinational Study of Cerdulatinib (PRT062070) in Patients With Relapsed/Refractory Peripheral T-cell Lymphoma (PTCL) Not yet recruiting NCT04021082 Phase 2, Phase 3 Cerdulatinib
49 Multi-center Randomized Study to Compare Efficacy and Safety of Decitabine Plus CHOP (D-CHOP) Versus CHOP in Patients With Previously Untreated Peripheral T-cell Lymphoma Not yet recruiting NCT03553537 Phase 3 Decitabine;Cyclophosphamide;Doxorubicin;Vincristine;Prednisone
50 A Multi-center, Randomized, Phase 3 Study of Sequential Pralatrexate Versus Observation in Patients With Previously Undiagnosed Peripheral T-cell Lymphoma Who Have Achieved an Objective Response Following Initial Treatment With CHOP-based Chemotherapy Terminated NCT01420679 Phase 3 Pralatrexate Injection

Search NIH Clinical Center for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Cochrane evidence based reviews: lymphoma, t-cell

Genetic Tests for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Anatomical Context for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

MalaCards organs/tissues related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

40
T Cells, B Cells, Skin, Bone, Adipocyte, Bone Marrow, Nk Cells

Publications for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Articles related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

(show top 50) (show all 635)
# Title Authors PMID Year
1
Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome. 61 52 56 6
30374066 2018
2
Frequent germline mutations of HAVCR2 in sporadic subcutaneous panniculitis-like T-cell lymphoma. 61 56 6
30792187 2019
3
Rare Cutaneous T-Cell Lymphomas. 61 52
30497671 2019
4
Immunohistochemical detection of BCL-3 in lymphoid neoplasms: a survey of 353 cases. 54 61
15105810 2004
5
Immunohistochemical detection of ZAP-70 in 341 cases of non-Hodgkin and Hodgkin lymphoma. 54 61
15133473 2004
6
Treatment of T-cell non-Hodgkin's lymphoma. 54 61
15233906 2004
7
A retrospective review of cutaneous lymphoma in Botswana. 61
31647120 2020
8
Activated-cytotoxic TCRαβ+CD4+ peripheral T-cell lymphoma with hypodermal localization: Case report of a lymphoproliferative disorder probably evolved from the CD4+ cytotoxic T-cell subpopulation. 61
31595546 2020
9
18F-FDG PET/CT-Guided Diagnosis and Evaluation of Treatment Response to Cyclosporine A in Maldistributed Subcutaneous Panniculitis-like T-Cell Lymphoma Without Typical Skin Rash. 61
31833924 2020
10
Pralatrexate for refractory or recurrent subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome. 61
31319088 2020
11
HAVCR2 mutations are associated with severe hemophagocytic syndrome in subcutaneous panniculitis-like T-cell lymphoma. 61
32005988 2020
12
Subcutaneous panniculitis-like T-cell lymphoma. 61
31975700 2020
13
Non-mycosis fungoides cutaneous lymphomas in a referral center in Taiwan: A retrospective case series and literature review. 61
31978091 2020
14
C-C chemokine receptor 4 expression in CD8+ cutaneous T-cell lymphomas and lymphoproliferative disorders, and its implications for diagnosis and treatment. 61
31355940 2020
15
Clinical features of infantile subcutaneous panniculitis-like T-cell lymphoma. 61
31808238 2019
16
[Clinical Comparative Analysis of Lupus Panniculitis and Subcutaneous Panniculitis-like T-cell Lymphoma]. 61
31880127 2019
17
High-throughput Sequencing of Subcutaneous Panniculitis-like T-Cell Lymphoma Reveals Candidate Pathogenic Mutations. 61
31702703 2019
18
Is cyclosporine a good option for the treatment of subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic syndrome? 61
31571617 2019
19
Not all swellings are lymph nodes! A case of subcutaneous panniculitis-like T-cell lymphoma. 61
31649225 2019
20
Subcutaneous Panniculitis-Like T Cell Lymphoma: Approach to Differential Diagnosis on Cytology. 61
31580741 2019
21
Positron emission tomography-computed tomography in subcutaneous panniculitis-like T-cell lymphoma. 61
31933563 2019
22
Epidemiology of primary cutaneous γδ T-cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma in the U.S.A. from 2006 to 2015: a Surveillance, Epidemiology, and End Results-18 analysis. 61
30951189 2019
23
Subcutaneous Panniculitis-Like T-Cell Lymphoma With Granulomas as the Predominant Feature. 61
30908292 2019
24
Pralatrexate Induces Long-Term Remission in Relapsed Subcutaneous Panniculitis-Like T-Cell Lymphoma. 61
31737895 2019
25
Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma. 61
30289114 2019
26
Subcutaneous panniculitis-like T-cell lymphoma in a 25-year-old male patient with sickle cell disease. 61
30944066 2019
27
Pediatric Subcutaneous Panniculitis-like T-Cell Lymphoma With Hemophagocytosis Showing Complete Resolution With the BFM90 Protocol: Case Report and Review of Literature. 61
30730380 2019
28
Cutaneous mantle cell lymphoma histomorphologically mimicking subcutaneous panniculitis-like T-cell lymphoma: Case report. 61
30957249 2019
29
Panniculitis-like T-cell-lymphoma in the mesentery associated with hemophagocytic syndrome: autopsy case report. 61
31311562 2019
30
Fever with Positive EBV IgM Antibody Combined Multiple Subcutaneous Nodules on Lower Extremities and Cervical Lymphadenopathy Masquerading as Infectious Mononucleosis Proven as Subcutaneous Panniculitis-like T-cell Lymphoma by Subcutaneous Nodule Biopsy Consultation: a Case Report and Literature Review. 61
31307165 2019
31
Utility of CD123 immunohistochemistry in differentiating lupus erythematosus from cutaneous T cell lymphoma. 61
30597607 2019
32
Successful treatment of a rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review. 61
30884048 2019
33
Hematopoietic stem cell transplantation for subcutaneous panniculitis-like T-cell lymphoma: single center experience in an Asian population. 61
30547418 2019
34
Treatment of Facial Lipoatrophy Secondary to Subcutaneous Panniculitis-Like T-Cell Lymphoma. 61
30829756 2019
35
Local radiation for cutaneous T-cell lymphoma other than mycosis fungoides and Sézary syndrome. 61
30180750 2019
36
[Concurrent subcutaneous panniculitis-like T-cell lymphoma and myelodysplastic syndrome with fibrosis]. 61
31167997 2019
37
Clinicopathologic, immunophenotypic, and molecular analysis of subcutaneous panniculitis-like T-cell lymphoma: A retrospective study in a tertiary care center. 61
30350476 2019
38
The Challenge of Identifying Identical Twins: Advances in Differentiating Lupus Erythematosus Panniculitis from Subcutaneous Panniculitis-Like T-Cell Lymphoma. 61
31145063 2019
39
Necrotizing Granulomatous Dermatitis and Panniculitis Masquerading as T Cell Lymphoma. 61
31904333 2019
40
Rare T-Cell Subtypes. 61
30596220 2019
41
Diagnosis of acquired generalized lipodystrophy in a single patient with T-cell lymphoma and no exposure to Metreleptin. 61
30923630 2019
42
Subcutaneous Panniculitis-Like T-cell Lymphoma in Dogs: Morphologic and Immunohistochemical Classification. 61
30071780 2018
43
Relapsed subcutaneous panniculitis-like T cell lymphoma evaluated by FDG PET/CT: A clinical case report. 61
30431571 2018
44
Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients. 61
30126736 2018
45
T-cell receptor-δ expression and γδ+ T-cell infiltrates in primary cutaneous γδ T-cell lymphoma and other cutaneous T-cell lymphoproliferative disorders. 61
29893430 2018
46
Mycophenolate Mofetil for the Treatment of Subcutaneous Panniculitis-Like T-Cell Lymphoma: Case Report and Review of the Literature. 61
30077696 2018
47
Subcutaneous panniculitis-like T-cell lymphoma responsive to combination therapy with methotrexate and corticosteroids. 61
30677832 2018
48
Subcutaneous Panniculitis-Like T-Cell Lymphoma Versus Lupus Erythematosus Panniculitis: Distinction by Means of the Periadipocytic Cell Proliferation Index. 61
29742552 2018
49
A Case of Refractory Subcutaneous Panniculitis-like T-cell Lymphoma with Bone Marrow and Lung Involvement Treated Successfully with Oral Etoposide. 61
29881869 2018
50
Recurrent mutations in epigenetic modifiers and the PI3K/AKT/mTOR pathway in subcutaneous panniculitis-like T-cell lymphoma. 61
28294301 2018

Variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

ClinVar genetic disease variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

6 ‎‎ ‎‎ ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 HAVCR2 NM_032782.5(HAVCR2):c.245A>G (p.Tyr82Cys)SNV risk factor 626252 rs184868814 5:156533787-156533787 5:157106776-157106776
2 HAVCR2 NM_032782.5(HAVCR2):c.291A>G (p.Ile97Met)SNV risk factor 626253 rs35960726 5:156533741-156533741 5:157106730-157106730
3 HAVCR2 NM_032782.5(HAVCR2):c.302C>T (p.Thr101Ile)SNV risk factor 626254 rs147827860 5:156533730-156533730 5:157106719-157106719

UniProtKB/Swiss-Prot genetic disease variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

73
# Symbol AA change Variation ID SNP ID
1 HAVCR2 p.Tyr82Cys VAR_082211 rs184868814
2 HAVCR2 p.Ile97Met VAR_082212 rs35960726
3 HAVCR2 p.Thr101Ile VAR_082213 rs147827860

Copy number variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like from CNVD:

7 (show top 50) (show all 868)
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 13770 1 1 34400000 Loss T-cell lymphomas
2 24155 1 176443955 176516990 Amplification MGC16664 T-cell lymphomas
3 24159 1 176545610 176578591 Amplification LOC163590 T-cell lymphomas
4 24161 1 176565573 176565968 Amplification IFRG15 T-cell lymphomas
5 24164 1 176583077 176620868 Amplification LAP1B T-cell lymphomas
6 24170 1 176655781 176814172 Amplification CAP350 T-cell lymphomas
7 24182 1 176855625 176898824 Amplification QSCN6 T-cell lymphomas
8 24187 1 176931099 176975845 Amplification LHX4 T-cell lymphomas
9 25717 1 193520555 193532978 Amplification HFL1 T-cell lymphomas
10 25718 1 193520567 193660013 Amplification HFL3 T-cell lymphomas
11 25732 1 193739978 193768023 Amplification F13B T-cell lymphomas
12 25737 1 193785111 193847224 Amplification ASPM T-cell lymphomas
13 25742 1 193859229 193901308 Amplification FRBZ1 T-cell lymphomas
14 25749 1 193969065 194179242 Amplification CRB1 T-cell lymphomas
15 25760 1 194253042 194475981 Amplification FAM31B T-cell lymphomas
16 25775 1 194601217 194608154 Amplification LOC388722 T-cell lymphomas
17 25778 1 194618174 194630930 Amplification LHX9 T-cell lymphomas
18 25796 1 194933334 195020426 Amplification NEK7 T-cell lymphomas
19 25985 1 195224011 195241732 Amplification ATP6V1G3 T-cell lymphomas
20 25999 1 195339882 195457987 Amplification PTPRC T-cell lymphomas
21 26165 1 197500000 241700000 Gain T-cell lymphomas
22 27801 1 213064983 213651371 Amplification ESRRG T-cell lymphomas
23 27839 1 213992229 214192804 Amplification GPATC2 T-cell lymphomas
24 27849 1 214193090 214428879 Amplification LOC128153 T-cell lymphomas
25 27883 1 214847047 214893312 Amplification CGI-115 T-cell lymphomas
26 35102 1 6020646 6094789 Deletion KCNAB2 T-cell lymphomas
27 35200 1 6096119 6174449 Deletion CHD5 T-cell lymphomas
28 35249 1 6180833 6193921 Deletion RPL22 T-cell lymphomas
29 35283 1 6199801 6202638 Deletion FLJ46380 T-cell lymphomas
30 35297 1 6215521 6230298 Deletion ICMT T-cell lymphomas
31 35311 1 6236858 6239833 Deletion LOC390992 T-cell lymphomas
32 35323 1 6243122 6255301 Deletion GPR153 T-cell lymphomas
33 35343 1 6258599 6388087 Deletion BACH T-cell lymphomas
34 38878 10 105700000 135374737 Loss T-cell lymphomas
35 41380 10 17300000 40300000 Loss T-cell lymphomas
36 41720 10 22674405 22746545 Deletion SPAG6 T-cell lymphomas
37 42050 10 27727122 27743303 Deletion FLJ44037 T-cell lymphomas
38 42820 10 40300000 82000000 Loss T-cell lymphomas
39 47063 10 89502855 89567897 Deletion ATAD1 T-cell lymphomas
40 47157 10 89613175 89716382 Deletion PTEN T-cell lymphomas
41 47181 10 90023601 90332988 Deletion C10orf59 T-cell lymphomas
42 47203 10 90414196 90428542 Deletion LIPF T-cell lymphomas
43 47225 10 90572282 90601495 Deletion ANKRD22 T-cell lymphomas
44 47245 10 90651413 90673218 Deletion AMSH-LP T-cell lymphomas
45 47253 10 90740268 90765522 Deletion TNFRSF6 T-cell lymphomas
46 47265 10 90955674 90957051 Deletion CH25H T-cell lymphomas
47 47270 10 90963312 91001512 Deletion LIPA T-cell lymphomas
48 47272 10 91051783 91058661 Deletion IFIT2 T-cell lymphomas
49 47275 10 91077733 91090288 Deletion IFIT3 T-cell lymphomas
50 47279 10 91127793 91134942 Deletion LOC439996 T-cell lymphomas

Expression for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Search GEO for disease gene expression data for T-Cell Lymphoma, Subcutaneous Panniculitis-Like.

Pathways for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Pathways related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.44 TNFRSF8 NFKB2 HAVCR2 GZMB
2
Show member pathways
11.18 TRB NFKB2 GZMB
3 11.02 TNFRSF8 HAVCR2
4 10.89 TRB NFKB2

GO Terms for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Cellular components related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immunological synapse GO:0001772 8.62 HAVCR2 GZMB

Sources for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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