SPTCL
MCID: SBC024
MIFTS: 38

Subcutaneous Panniculitis-Like T-Cell Lymphoma (SPTCL)

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases, Skin 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 fever and fatigue. 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 Cyclophosphamide and Gemcitabine 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 58)
# Related Disease Score Top Affiliating Genes
1 panniculitis 31.0 GZMB NAV3 NCAM1 TIA1 TRD
2 peripheral t-cell lymphoma 29.9 GZMB NCAM1 TIA1
3 lymphoma 11.3
4 lupus erythematosus 10.6
5 lupus erythematosus panniculitis 10.5
6 hematopoietic stem cell transplantation 10.5
7 hemophagocytic lymphohistiocytosis 10.4
8 anaplastic large cell lymphoma 10.3
9 arthritis 10.3
10 b-cell lymphomas 10.3
11 dermatomyositis 10.3
12 dermatitis 10.3
13 macrophage activation syndrome 10.3
14 nocardiosis 10.2
15 rheumatoid arthritis 10.2
16 diffuse large b-cell lymphoma 10.2
17 patulous eustachian tube 10.2
18 pure red-cell aplasia 10.2
19 cellulitis 10.2
20 erythromelalgia 10.2
21 discoid lupus erythematosus 10.2
22 cytophagic histiocytic panniculitis 10.2
23 papillon-lefevre syndrome 10.1 GZMB SLC17A5
24 chronic active epstein-barr virus infection 10.1 TRD TRG
25 leukemia 10.0
26 t-cell large granular lymphocyte leukemia 10.0 GZMB NCAM1
27 lung lymphoma 10.0 GZMB NCAM1
28 lichen sclerosus et atrophicus 10.0 GZMB TIA1
29 lymphomatoid granulomatosis 10.0 GZMB TIA1
30 viral esophagitis 10.0 GZMB TIA1
31 plasma cell neoplasm 10.0 MYC NCAM1
32 lymphocytic gastritis 10.0 GZMB TIA1
33 composite lymphoma 10.0 GZMB TIA1
34 hepatosplenic t-cell lymphoma 10.0 GZMB TIA1
35 lymphomatoid papulosis 10.0 GZMB TIA1
36 choriocarcinoma of the testis 10.0 GZMB TIA1
37 mature b-cell neoplasm 10.0 MYC NCAM1
38 giardiasis 10.0 GZMB TIA1
39 infiltrating lipoma 10.0 GZMB TIA1
40 necrotizing sialometaplasia 9.9 GZMB TIA1
41 precursor t-cell acute lymphoblastic leukemia 9.9 MYC TRD TRG
42 autonomic nervous system neoplasm 9.9 MYC NCAM1
43 angioimmunoblastic t-cell lymphoma 9.9 GZMB TIA1
44 peripheral nervous system neoplasm 9.9 MYC NCAM1
45 reticulosarcoma 9.9 GZMB TIA1
46 b-cell expansion with nfkb and t-cell anergy 9.9 GZMB TIA1
47 lethal midline granuloma 9.9 NCAM1 TIA1
48 leukocyte disease 9.9 MYC NCAM1
49 parasitic ichthyosporea infectious disease 9.9 NCAM1 TIA1
50 rhinosporidiosis 9.9 NCAM1 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:


fever, fatigue

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 64)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 4,Phase 1,Phase 2 6055-19-2, 50-18-0 2907
2
Gemcitabine Approved Phase 4 95058-81-4 60750
3
Vincristine Approved, Investigational Phase 4,Phase 1,Phase 2 57-22-7, 2068-78-2 5978
4
Etoposide Approved Phase 4 33419-42-0 36462
5
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
6
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4 1177-87-3
7
Cisplatin Approved Phase 4 15663-27-1 2767 441203 84093
8
Ifosfamide Approved Phase 4 3778-73-2 3690
9
Epirubicin Approved Phase 4 56420-45-2 41867
10
Prednisone Approved, Vet_approved Phase 4,Phase 1,Phase 2 53-03-2 5865
11
Doxorubicin Approved, Investigational Phase 4,Phase 1,Phase 2 23214-92-8 31703
12
Mechlorethamine Approved, Investigational Phase 4 51-75-2 4033
13
Methotrexate Approved Phase 4 59-05-2, 1959-05-2 126941
14
leucovorin Approved Phase 4 58-05-9 6006 143
15
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
16
Doxil Approved June 1999 Phase 4,Phase 1,Phase 2 31703
17
Pirarubicin Investigational Phase 4 72496-41-4
18
Isophosphamide mustard Phase 4 0
19 Hormone Antagonists Phase 4,Phase 1,Phase 2
20 Anti-Bacterial Agents Phase 4,Phase 1,Phase 2
21 Antineoplastic Agents, Alkylating Phase 4,Phase 1,Phase 2
22 Topoisomerase Inhibitors Phase 4,Phase 1,Phase 2
23 Antibiotics, Antitubercular Phase 4,Phase 1,Phase 2
24 Immunologic Factors Phase 4,Phase 1,Phase 2
25 Antineoplastic Agents, Hormonal Phase 4,Phase 1,Phase 2
26 Antimitotic Agents Phase 4,Phase 1,Phase 2
27 Alkylating Agents Phase 4,Phase 1,Phase 2
28 Hormones Phase 4,Phase 1,Phase 2
29 Anti-Inflammatory Agents Phase 4,Phase 1,Phase 2
30 Antineoplastic Agents, Phytogenic Phase 4,Phase 1,Phase 2
31 Immunosuppressive Agents Phase 4,Phase 1,Phase 2
32 BB 1101 Phase 4
33 Etoposide phosphate Phase 4
34 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 1,Phase 2
35 Antirheumatic Agents Phase 4,Phase 1,Phase 2
36 glucocorticoids Phase 4,Phase 1,Phase 2
37 Folic Acid Antagonists Phase 4
38 Dermatologic Agents Phase 4
39 Vitamin B Complex Phase 4
40 Antimetabolites Phase 4
41 Folate Phase 4
42 Antimetabolites, Antineoplastic Phase 4
43 Vitamin B9 Phase 4
44 Nucleic Acid Synthesis Inhibitors Phase 4
45
Vorinostat Approved, Investigational Phase 1, Phase 2,Phase 2 149647-78-9 5311
46
Bortezomib Approved, Investigational Phase 2,Phase 1 179324-69-7 387447 93860
47 Histone Deacetylase Inhibitors Phase 1, Phase 2,Phase 2
48
rituximab Approved Phase 1 174722-31-7 10201696
49
Miconazole Approved, Investigational, Vet_approved Phase 1 22916-47-8 4189
50
Everolimus Approved Phase 1 159351-69-6 6442177

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 Cholecalciferol in Improving Survival in Patients With Newly Diagnosed Cancer With Vitamin D Insufficiency Recruiting NCT01787409 Not Applicable
8 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Active, not recruiting NCT02652715 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, Bone Marrow, B Cells, Lung, Testis

Publications for Subcutaneous Panniculitis-Like T-Cell Lymphoma

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

(show top 50) (show all 180)
# 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
Hematopoietic stem cell transplantation for subcutaneous panniculitis-like T-cell lymphoma: single center experience in an Asian population. ( 30547418 )
2018
5
Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma. ( 30289114 )
2018
6
The coexistence of lupus erythematosus panniculitis and subcutaneous panniculitis-like T-cell lymphoma in the same patient. ( 29892661 )
2018
7
Relapsed subcutaneous panniculitis-like T cell lymphoma evaluated by FDG PET/CT: A clinical case report. ( 30431571 )
2018
8
Clinicopathologic, immunophenotypic, and molecular analysis of subcutaneous panniculitis-like T-cell lymphoma: A retrospective study in a tertiary care center. ( 30350476 )
2018
9
Subcutaneous Panniculitis-Like T-cell Lymphoma in Dogs: Morphologic and Immunohistochemical Classification. ( 30071780 )
2018
10
Mycophenolate Mofetil for the Treatment of Subcutaneous Panniculitis-Like T-Cell Lymphoma: Case Report and Review of the Literature. ( 30077696 )
2018
11
Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients. ( 30126736 )
2018
12
Clinical characteristics, differential diagnosis, and treatment outcome of subcutaneous panniculitis-like T-cell lymphoma: a literature review of published Japanese cases. ( 28120776 )
2017
13
Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting as a Local Inflammation of a Thigh in an 8-Month-Old Child. ( 29085769 )
2017
14
Subcutaneous panniculitis-like T-cell lymphoma with haemophagocytic syndrome during tocilizumab therapy for juvenile idiopathic arthritis. ( 28079502 )
2017
15
Immunohistochemistry reveals an increased proportion of MYC-positive cells in subcutaneous panniculitis-like T-cell lymphoma compared with lupus panniculitis. ( 28800143 )
2017
16
Relapsed subcutaneous panniculitis-like T cell lymphoma: role of haploidentical hematopoietic stem cell transplant. ( 28879427 )
2017
17
Recurrent mutations in epigenetic modifiers and the PI3K/AKT/mTOR pathway in subcutaneous panniculitis-like T-cell lymphoma. ( 28294301 )
2017
18
Remission of subcutaneous panniculitis-like T-cell lymphoma in a pregnant woman after treatment with oral corticosteroids as monotherapy. ( 28361107 )
2017
19
Subcutaneous panniculitis-like T-cell lymphoma. ( 28127142 )
2017
20
CCR4 and CCR5 expression in a case of subcutaneous panniculitis-like T-cell lymphoma. ( 28721932 )
2017
21
Hemophagocytic lymphohistiocytosis associated with subcutaneous panniculitis-like T-cell lymphoma. ( 27879081 )
2016
22
Subcutaneous panniculitis like T-cell lymphoma. ( 27728567 )
2016
23
Subcutaneous panniculitis-like T-cell lymphoma with macrophage activation syndrome treated by cyclosporine and prednisolone. ( 27990393 )
2016
24
Long-term remission of subcutaneous panniculitis-like T-cell lymphoma with central nervous system involvement: A case report. ( 27347188 )
2016
25
Relapsed and refractory subcutaneous panniculitis-like T-cell lymphoma with excellent response to cyclosporine: a case report and literature review. ( 26873089 )
2016
26
Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Rare Tumour. ( 27437288 )
2016
27
Subcutaneous Panniculitis-like T Cell Lymphoma Mimicking Early-Onset Nodular Panniculitis. ( 27342380 )
2016
28
Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome in a Child. ( 26764131 )
2016
29
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
30
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
31
Kikuchi-Fujimoto Disease Associated With Subcutaneous Panniculitis-Like T-Cell Lymphoma. ( 26844615 )
2016
32
Pediatric subcutaneous panniculitis-like T-cell lymphoma presenting as paraparesis: An unreported manifestation. ( 27625250 )
2016
33
Subcutaneous panniculitis like T-cell lymphoma in a case of sarcoidosis: A rare association. ( 27728564 )
2016
34
Case report of subcutaneous panniculitis-like T-cell lymphoma complicated by eyelid swelling. ( 27440138 )
2016
35
Atypical lymphocytic lobular panniculitis: an overlap condition with features of subcutaneous panniculitis-like T-cell lymphoma and lupus profundus. ( 27807018 )
2016
36
Subcutaneous Panniculitis-like T cell Lymphoma: Immunosuppressive Drugs Induce Better Response than Polychemotherapy. ( 27722764 )
2016
37
Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography evaluation of subcutaneous panniculitis-like T cell lymphoma and treatment response. ( 27672640 )
2016
38
Useful Parameters for Distinguishing Subcutaneous Panniculitis-like T-Cell Lymphoma From Lupus Erythematosus Panniculitis. ( 26796503 )
2016
39
Systemic T cell lymphoma mimicking subcutaneous panniculitis-like T-cell lymphoma. ( 27444745 )
2016
40
A rare case of fever of unknown origin: subcutaneous panniculitis-like T-cell lymphoma (SPTCL). ( 26290563 )
2015
41
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
42
Subcutaneous Panniculitis-like T-cell Lymphoma Presenting With Diffuse Cutaneous Edema in a 2-Year-old Child. ( 25739026 )
2015
43
CCR5 and CXCR3 expression in a case of subcutaneous panniculitis-like T-cell lymphoma. ( 26301865 )
2015
44
Fever and generalized erythema caused by subcutaneous panniculitis-like T-cell lymphoma. ( 26621148 )
2015
45
Value of positron emission tomography in diagnosing subcutaneous panniculitis-like T-cell lymphoma. ( 25691676 )
2015
46
Subcutaneous panniculitis-like T-cell lymphoma: A report of five cases with review of literature. ( 26960515 )
2015
47
Subcutaneous panniculitis-like T-cell lymphoma: Pediatric case series demonstrating heterogeneous presentation and option for watchful waiting. ( 26146844 )
2015
48
Subcutaneous Panniculitis-Like T-Cell Lymphoma in Children: A Review of the Literature. ( 25727090 )
2015
49
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
50
Subcutaneous panniculitis-like T-cell lymphoma with bone marrow involvement. ( 25596253 )
2015

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

3 CDC
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9 Cosmic
10 dbSNP
11 DGIdb
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
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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