MCID: SBC024
MIFTS: 39

Subcutaneous Panniculitis-Like T-Cell Lymphoma

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

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

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

Name: Subcutaneous Panniculitis-Like T-Cell Lymphoma 53 59 73
Sptcl 53 59
Subcutaneous Panniculitic T-Cell Lymphoma 59

Characteristics:

Orphanet epidemiological data:

59
subcutaneous panniculitis-like t-cell lymphoma
Inheritance: Not applicable; Age of onset: All ages;

Classifications:



External Ids:

Orphanet 59 ORPHA86884
UMLS via Orphanet 74 C0522624
MESH via Orphanet 45 C537503
ICD10 via Orphanet 34 C83.6
ICD10 33 C86.3
UMLS 73 C0522624

Summaries for Subcutaneous Panniculitis-Like T-Cell Lymphoma

MalaCards based summary : Subcutaneous Panniculitis-Like T-Cell Lymphoma, also known as sptcl, is related to panniculitis and peripheral t-cell lymphoma, and has symptoms including fatigue and fever. An important gene associated with Subcutaneous Panniculitis-Like T-Cell Lymphoma is GZMB (Granzyme B), and among its related pathways/superpathways are Apoptosis and Autophagy and Innate Lymphoid Cell Differentiation Pathways. The drugs Gemcitabine and Etoposide have been mentioned in the context of this disorder. Affiliated tissues include t cells, skin and bone, and related phenotypes are Synthetic lethal with MLN4924 (a NAE inhibitor) and Synthetic lethal with MLN4924 (a NAE inhibitor)

Related Diseases for Subcutaneous Panniculitis-Like T-Cell Lymphoma

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

(show top 50) (show all 56)
# Related Disease Score Top Affiliating Genes
1 panniculitis 29.3 GZMB MYC NAV3 NCAM1 TIA1
2 peripheral t-cell lymphoma 29.3 GZMB NCAM1 TIA1
3 lymphoma 11.2
4 lupus erythematosus 10.5
5 lupus erythematosus panniculitis 10.4
6 hematopoietic stem cell transplantation 10.4
7 t-cell large granular lymphocyte leukemia 10.3 GZMB NCAM1
8 chronic active epstein-barr virus infection 10.3 TRD TRG
9 lung lymphoma 10.2 GZMB NCAM1
10 anaplastic large cell lymphoma 10.2
11 arthritis 10.2
12 macrophage activation syndrome 10.2
13 b-cell lymphomas 10.2
14 hemophagocytic lymphohistiocytosis 10.2
15 dermatomyositis 10.2
16 dermatitis 10.2
17 plasma cell neoplasm 10.2 MYC NCAM1
18 papillon-lefevre syndrome 10.1 GZMB SLC17A5
19 peripheral nervous system neoplasm 10.1 MYC NCAM1
20 rheumatoid arthritis 10.1
21 cellulitis 10.1
22 aging 10.1
23 erythromelalgia 10.1
24 discoid lupus erythematosus 10.1
25 diffuse large b-cell lymphoma 10.1
26 cytophagic histiocytic panniculitis 10.1
27 pure red-cell aplasia 10.1
28 rheumatic disease 10.1
29 enthesopathy 10.1
30 autonomic nervous system neoplasm 10.0 MYC NCAM1
31 lichen sclerosus et atrophicus 10.0 GZMB TIA1
32 lymphomatoid granulomatosis 10.0 GZMB TIA1
33 viral esophagitis 10.0 GZMB TIA1
34 lymphocytic gastritis 10.0 GZMB TIA1
35 natural killer cell leukemia 10.0 GZMB TIA1
36 composite lymphoma 9.9 GZMB TIA1
37 hepatosplenic t-cell lymphoma 9.9 GZMB TIA1
38 lymphomatoid papulosis 9.9 GZMB TIA1
39 bile duct carcinoma 9.9 MYC NCAM1
40 choriocarcinoma of the testis 9.9 GZMB TIA1
41 precursor t-cell acute lymphoblastic leukemia 9.9 MYC TRD TRG
42 giardiasis 9.9 GZMB TIA1
43 lethal midline granuloma 9.9 NCAM1 TIA1
44 infiltrating lipoma 9.9 GZMB TIA1
45 parasitic ichthyosporea infectious disease 9.8 NCAM1 TIA1
46 necrotizing sialometaplasia 9.8 GZMB TIA1
47 rhinosporidiosis 9.8 NCAM1 TIA1
48 lymphatic system cancer 9.8 MYC TIA1
49 reticulosarcoma 9.8 GZMB TIA1
50 b-cell expansion with nfkb and t-cell anergy 9.7 GZMB TIA1

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



Diseases related to Subcutaneous Panniculitis-Like T-Cell Lymphoma

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

UMLS symptoms related to Subcutaneous Panniculitis-Like T-Cell Lymphoma:


fatigue, fever

GenomeRNAi Phenotypes related to Subcutaneous Panniculitis-Like T-Cell Lymphoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-1 9.17 GZMB NAV3 TIA1
2 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 9.17 GZMB MYC NAV3 TIA1

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

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

(show top 50) (show all 63)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Gemcitabine Approved Phase 4 95058-81-4 60750
2
Etoposide Approved Phase 4 33419-42-0 36462
3
Cisplatin Approved Phase 4 15663-27-1 84093 441203 2767
4
Ifosfamide Approved Phase 4 3778-73-2 3690
5
Vincristine Approved, Investigational Phase 4,Phase 1,Phase 2 2068-78-2, 57-22-7 5978
6
Cyclophosphamide Approved, Investigational Phase 4,Phase 1,Phase 2 50-18-0, 6055-19-2 2907
7
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
8
Epirubicin Approved Phase 4 56420-45-2 41867
9
Prednisone Approved, Vet_approved Phase 4,Phase 1,Phase 2 53-03-2 5865
10
Doxorubicin Approved, Investigational Phase 4,Phase 1,Phase 2 23214-92-8 31703
11
Mechlorethamine Approved, Investigational Phase 4 51-75-2 4033
12
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
13
leucovorin Approved, Nutraceutical Phase 4 58-05-9 143 6006
14
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
15
Doxil Approved June 1999 Phase 4,Phase 1,Phase 2 31703
16
Pirarubicin Investigational Phase 4 72496-41-4
17 Dexamethasone acetate Phase 4 1177-87-3
18
Isophosphamide mustard Phase 4 0
19 Alkylating Agents Phase 4,Phase 1,Phase 2
20 glucocorticoids Phase 4,Phase 1,Phase 2
21 Topoisomerase Inhibitors Phase 4,Phase 1,Phase 2
22 Hormone Antagonists Phase 4,Phase 1,Phase 2
23 Hormones Phase 4,Phase 1,Phase 2
24 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 1,Phase 2
25 Anti-Bacterial Agents Phase 4,Phase 1,Phase 2
26 Antibiotics, Antitubercular Phase 4,Phase 1,Phase 2
27 Etoposide phosphate Phase 4
28 Immunosuppressive Agents Phase 4,Phase 1,Phase 2
29 Anti-Inflammatory Agents Phase 4,Phase 1,Phase 2
30 Antirheumatic Agents Phase 4,Phase 1,Phase 2
31 BB 1101 Phase 4
32 Antimitotic Agents Phase 4,Phase 1,Phase 2
33 Antineoplastic Agents, Alkylating Phase 4,Phase 1,Phase 2
34 Antineoplastic Agents, Hormonal Phase 4,Phase 1,Phase 2
35 Antineoplastic Agents, Phytogenic Phase 4,Phase 1,Phase 2
36 Folic Acid Antagonists Phase 4
37 Dermatologic Agents Phase 4
38 Nucleic Acid Synthesis Inhibitors Phase 4
39 Vitamin B Complex Phase 4
40 Antimetabolites Phase 4
41 Antimetabolites, Antineoplastic Phase 4
42 Folate Nutraceutical Phase 4
43 Vitamin B9 Nutraceutical Phase 4
44
Vorinostat Approved, Investigational Phase 1, Phase 2,Phase 2 149647-78-9 5311
45
Bortezomib Approved, Investigational Phase 2,Phase 1 179324-69-7 387447 93860
46 Histone Deacetylase Inhibitors Phase 1, Phase 2,Phase 2
47
rituximab Approved Phase 1 174722-31-7 10201696
48
Miconazole Approved, Investigational, Vet_approved Phase 1 22916-47-8 4189
49
Everolimus Approved Phase 1 159351-69-6 6442177
50
Sirolimus Approved, Investigational Phase 1 53123-88-9 5284616 6436030 46835353

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 CEOP/IVE/GDP Compared With CEOP as the First-line Therapy for Newly Diagnosed Adult Patients With PTCL Unknown status NCT02533700 Phase 4 CEOP/IVE/GDP chemotherapy regimen;CEOP chemotherapy regimen for 6 cycles
2 CTOP/ITE/MTX Compared With CHOP as the First-line Therapy for Newly Diagnosed Young Patients With T Cell Lymphoma Active, not recruiting 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
3 SAHA + CHOP in Untreated T-cell Non-Hodgkin's Lymphoma Completed NCT00787527 Phase 1, Phase 2 Zolinza (vorinostat);Cyclophosphamide;Doxorubicin;Vincristine;Prednisone
4 Combination of Vorinostat and Bortezomib in Relapsed or Refractory T-Cell Non-Hodgkin's Lymphoma Terminated NCT00810576 Phase 2 Vorinostat;Bortezomib
5 Phase 1 Trial of Siplizumab and Dose-Adjusted EPOCH-Rituximab in T- and NK-Cell Lymphomas Active, not recruiting NCT01445535 Phase 1 EPOCH
6 Everolimus and Bortezomib in Treating Patients With Relapsed or Refractory Lymphoma Terminated NCT00671112 Phase 1 Bortezomib;Everolimus
7 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Recruiting NCT02652715 Not Applicable
8 Cholecalciferol in Improving Survival in Patients With Newly Diagnosed Cancer With Vitamin D Insufficiency Recruiting NCT01787409 Not Applicable

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

Genetic Tests for Subcutaneous Panniculitis-Like T-Cell Lymphoma

Anatomical Context for Subcutaneous Panniculitis-Like T-Cell Lymphoma

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

41
T Cells, Skin, Bone, Nk Cells, Bone Marrow, B Cells, Cervix

Publications for Subcutaneous Panniculitis-Like T-Cell Lymphoma

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

(show top 50) (show all 168)
# Title Authors Year
1
Composite cutaneous lymphoma of diffuse large B-cell lymphoma-leg type and subcutaneous panniculitis-like T-cell lymphoma. ( 29851123 )
2018
2
Subcutaneous Panniculitis-Like T-Cell Lymphoma Versus Lupus Erythematosus Panniculitis: Distinction by Means of the Periadipocytic Cell Proliferation Index. ( 29742552 )
2018
3
A Case of Refractory Subcutaneous Panniculitis-like T-cell Lymphoma with Bone Marrow and Lung Involvement Treated Successfully with Oral Etoposide. ( 29881869 )
2018
4
The coexistence of lupus erythematosus panniculitis and subcutaneous panniculitis-like T-cell lymphoma in the same patient. ( 29892661 )
2018
5
Clinical characteristics, differential diagnosis, and treatment outcome of subcutaneous panniculitis-like T-cell lymphoma: a literature review of published Japanese cases. ( 28120776 )
2017
6
Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting as a Local Inflammation of a Thigh in an 8-Month-Old Child. ( 29085769 )
2017
7
Subcutaneous panniculitis-like T-cell lymphoma with haemophagocytic syndrome during tocilizumab therapy for juvenile idiopathic arthritis. ( 28079502 )
2017
8
Immunohistochemistry reveals an increased proportion of MYC-positive cells in subcutaneous panniculitis-like T-cell lymphoma compared with lupus panniculitis. ( 28800143 )
2017
9
Relapsed subcutaneous panniculitis-like T cell lymphoma: role of haploidentical hematopoietic stem cell transplant. ( 28879427 )
2017
10
Recurrent mutations in epigenetic modifiers and the PI3K/AKT/mTOR pathway in subcutaneous panniculitis-like T-cell lymphoma. ( 28294301 )
2017
11
Remission of subcutaneous panniculitis-like T-cell lymphoma in a pregnant woman after treatment with oral corticosteroids as monotherapy. ( 28361107 )
2017
12
Subcutaneous panniculitis-like T-cell lymphoma. ( 28127142 )
2017
13
CCR4 and CCR5 expression in a case of subcutaneous panniculitis-like T-cell lymphoma. ( 28721932 )
2017
14
Hemophagocytic lymphohistiocytosis associated with subcutaneous panniculitis-like T-cell lymphoma. ( 27879081 )
2016
15
Subcutaneous panniculitis like T-cell lymphoma. ( 27728567 )
2016
16
Subcutaneous panniculitis-like T-cell lymphoma with macrophage activation syndrome treated by cyclosporine and prednisolone. ( 27990393 )
2016
17
Long-term remission of subcutaneous panniculitis-like T-cell lymphoma with central nervous system involvement: A case report. ( 27347188 )
2016
18
Relapsed and refractory subcutaneous panniculitis-like T-cell lymphoma with excellent response to cyclosporine: a case report and literature review. ( 26873089 )
2016
19
Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Rare Tumour. ( 27437288 )
2016
20
Subcutaneous Panniculitis-like T Cell Lymphoma Mimicking Early-Onset Nodular Panniculitis. ( 27342380 )
2016
21
Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome in a Child. ( 26764131 )
2016
22
Survival of Subcutaneous Panniculitis-Like T-Cell Lymphoma and Peripheral T-Cell Lymphoma Not Otherwise Specified: A Propensity-Matched Analysis of the Surveillance, Epidemiology, and End Results Database. ( 27349764 )
2016
23
Clinical and histopathologic overlap between subcutaneous panniculitis-like T-cell lymphoma with systemic features and small cell CD8+ ALK+ systemic anaplastic large cell lymphoma with cutaneous involvement. ( 27060878 )
2016
24
Kikuchi-Fujimoto Disease Associated With Subcutaneous Panniculitis-Like T-Cell Lymphoma. ( 26844615 )
2016
25
Pediatric subcutaneous panniculitis-like T-cell lymphoma presenting as paraparesis: An unreported manifestation. ( 27625250 )
2016
26
Subcutaneous panniculitis like T-cell lymphoma in a case of sarcoidosis: A rare association. ( 27728564 )
2016
27
Case report of subcutaneous panniculitis-like T-cell lymphoma complicated by eyelid swelling. ( 27440138 )
2016
28
Atypical lymphocytic lobular panniculitis: an overlap condition with features of subcutaneous panniculitis-like T-cell lymphoma and lupus profundus. ( 27807018 )
2016
29
Subcutaneous Panniculitis-like T cell Lymphoma: Immunosuppressive Drugs Induce Better Response than Polychemotherapy. ( 27722764 )
2016
30
Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography evaluation of subcutaneous panniculitis-like T cell lymphoma and treatment response. ( 27672640 )
2016
31
Useful Parameters for Distinguishing Subcutaneous Panniculitis-like T-Cell Lymphoma From Lupus Erythematosus Panniculitis. ( 26796503 )
2016
32
Systemic T cell lymphoma mimicking subcutaneous panniculitis-like T-cell lymphoma. ( 27444745 )
2016
33
A rare case of fever of unknown origin: subcutaneous panniculitis-like T-cell lymphoma (SPTCL). ( 26290563 )
2015
34
Progressive and painful wound as a feature of subcutaneous panniculitis-like T-cell lymphoma (SPTCL): report of a case and review of literature. ( 25755768 )
2015
35
Subcutaneous Panniculitis-like T-cell Lymphoma Presenting With Diffuse Cutaneous Edema in a 2-Year-old Child. ( 25739026 )
2015
36
CCR5 and CXCR3 expression in a case of subcutaneous panniculitis-like T-cell lymphoma. ( 26301865 )
2015
37
Fever and generalized erythema caused by subcutaneous panniculitis-like T-cell lymphoma. ( 26621148 )
2015
38
Value of positron emission tomography in diagnosing subcutaneous panniculitis-like T-cell lymphoma. ( 25691676 )
2015
39
Subcutaneous panniculitis-like T-cell lymphoma: A report of five cases with review of literature. ( 26960515 )
2015
40
Subcutaneous panniculitis-like T-cell lymphoma: Pediatric case series demonstrating heterogeneous presentation and option for watchful waiting. ( 26146844 )
2015
41
Subcutaneous Panniculitis-Like T-Cell Lymphoma in Children: A Review of the Literature. ( 25727090 )
2015
42
Subcutaneous panniculitis-like T-cell lymphoma in children: a detailed clinicopathological description of 11 multifocal cases with a high frequency of haemophagocytic syndrome. ( 25456748 )
2015
43
Subcutaneous panniculitis-like T-cell lymphoma with bone marrow involvement. ( 25596253 )
2015
44
Successful treatment of refractory pure red cell aplasia with bortezomib after allogeneic haematopoietic cell transplantation in a patient with alpha-beta subcutaneous panniculitis-like T cell lymphoma. ( 26147536 )
2015
45
Hematopoietic stem cell transplantation for primary cutaneous I^I' T-cell lymphoma and refractory subcutaneous panniculitis-like T-cell lymphoma. ( 25981001 )
2015
46
Subcutaneous panniculitis-like T-cell lymphoma with unusual eschar-like crusting. ( 24612568 )
2014
47
Systemic corticosteroids for subcutaneous panniculitis-like T-cell lymphoma. ( 24725144 )
2014
48
Subcutaneous panniculitis-like T-cell lymphoma: MRI features and literature review. ( 24719040 )
2014
49
Pediatric subcutaneous panniculitis-like T-cell lymphoma with favorable result by immunosuppressive therapy: a report of two cases. ( 24684413 )
2014
50
Local facial edema: a novel presentation of subcutaneous panniculitis-like T-cell lymphoma in a 30-year-old Iranian woman. ( 25530062 )
2014

Variations for Subcutaneous Panniculitis-Like T-Cell Lymphoma

Expression for Subcutaneous Panniculitis-Like T-Cell Lymphoma

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

Pathways for Subcutaneous Panniculitis-Like T-Cell Lymphoma

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

# Super pathways Score Top Affiliating Genes
1 11.07 GZMB MYC TIA1
2 11.06 GZMB NCAM1
3 10.5 MYC TIA1

GO Terms for Subcutaneous Panniculitis-Like T-Cell Lymphoma

Biological processes related to Subcutaneous Panniculitis-Like T-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 amino acid transport GO:0006865 8.62 MYC SLC17A5

Sources for Subcutaneous Panniculitis-Like T-Cell Lymphoma

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17 ExPASy
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34 ICD10 via Orphanet
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44 MeSH
45 MESH via Orphanet
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58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
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74 UMLS via Orphanet
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