SPTCL
MCID: SBC024
MIFTS: 40

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 54 60 17 74
Sptcl 54 60
Subcutaneous Panniculitic T-Cell Lymphoma 60

Characteristics:

Orphanet epidemiological data:

60
subcutaneous panniculitis-like t-cell lymphoma
Inheritance: Not applicable; Age of onset: All ages;

Classifications:



External Ids:

ICD10 34 C86.3
MESH via Orphanet 46 C537503
ICD10 via Orphanet 35 C83.6
UMLS via Orphanet 75 C0522624
Orphanet 60 ORPHA86884
UMLS 74 C0522624

Summaries for Subcutaneous Panniculitis-Like T-Cell Lymphoma

NIH Rare Diseases : 54 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 : 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 Vincristine and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include t cells, skin and spleen, 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 30.7 GZMB NAV3 NCAM1 TIA1 TRD
2 peripheral t-cell lymphoma 30.1 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 myelofibrosis 10.2
15 nocardiosis 10.2
16 rheumatoid arthritis 10.2
17 diffuse large b-cell lymphoma 10.2
18 patulous eustachian tube 10.2
19 pure red-cell aplasia 10.2
20 cellulitis 10.2
21 erythromelalgia 10.2
22 discoid lupus erythematosus 10.2
23 cytophagic histiocytic panniculitis 10.2
24 lichen sclerosus et atrophicus 10.2 GZMB TIA1
25 lymphomatoid granulomatosis 10.1 GZMB TIA1
26 viral esophagitis 10.1 GZMB TIA1
27 lymphocytic gastritis 10.1 GZMB TIA1
28 t-cell large granular lymphocyte leukemia 10.1 GZMB NCAM1
29 composite lymphoma 10.1 GZMB TIA1
30 hepatosplenic t-cell lymphoma 10.1 GZMB TIA1
31 lymphomatoid papulosis 10.1 GZMB TIA1
32 choriocarcinoma of the testis 10.1 GZMB TIA1
33 lung lymphoma 10.1 GZMB NCAM1
34 giardiasis 10.1 GZMB TIA1
35 lethal midline granuloma 10.1 NCAM1 TIA1
36 parasitic ichthyosporea infectious disease 10.1 NCAM1 TIA1
37 infiltrating lipoma 10.1 GZMB TIA1
38 rhinosporidiosis 10.1 NCAM1 TIA1
39 necrotizing sialometaplasia 10.1 GZMB TIA1
40 leukemia 10.1
41 mature b-cell neoplasm 10.1 MYC NCAM1
42 angioimmunoblastic t-cell lymphoma 10.1 GZMB TIA1
43 papillon-lefevre syndrome 10.1 GZMB SLC17A5
44 reticulosarcoma 10.0 GZMB TIA1
45 b-cell expansion with nfkb and t-cell anergy 10.0 GZMB TIA1
46 autonomic nervous system neoplasm 10.0 MYC NCAM1
47 peripheral nervous system neoplasm 10.0 MYC NCAM1
48 primary cutaneous anaplastic large cell lymphoma 10.0 GZMB TIA1 TRG
49 mycosis fungoides 10.0 GZMB TIA1 TRG
50 leukocyte disease 9.9 MYC NCAM1

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:

27
# 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 68)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vincristine Approved, Investigational Phase 4,Phase 1,Phase 2 2068-78-2, 57-22-7 5978
2
Ifosfamide Approved Phase 4 3778-73-2 3690
3
Prednisone Approved, Vet_approved Phase 4,Phase 1,Phase 2 53-03-2 5865
4
Epirubicin Approved Phase 4 56420-45-2 41867
5
Cyclophosphamide Approved, Investigational Phase 4,Phase 1,Phase 2 6055-19-2, 50-18-0 2907
6
Cisplatin Approved Phase 4 15663-27-1 441203 84093 2767
7
Etoposide Approved Phase 4 33419-42-0 36462
8
Gemcitabine Approved Phase 4 95058-81-4 60750
9
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4 1177-87-3
10
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
11
Doxorubicin Approved, Investigational Phase 4,Phase 1,Phase 2 23214-92-8 31703
12
leucovorin Approved Phase 4 58-05-9 6006 143
13
Mechlorethamine Approved, Investigational Phase 4 51-75-2 4033
14
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
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 Anti-Bacterial Agents Phase 4,Phase 1,Phase 2
20 Anti-Inflammatory Agents Phase 4,Phase 1,Phase 2
21 Antineoplastic Agents, Alkylating Phase 4,Phase 1,Phase 2
22 Etoposide phosphate Phase 4
23 Hormones Phase 4,Phase 1,Phase 2,Not Applicable
24 BB 1101 Phase 4
25 Immunosuppressive Agents Phase 4,Phase 1,Phase 2
26 Antineoplastic Agents, Hormonal Phase 4,Phase 1,Phase 2
27 Hormone Antagonists Phase 4,Phase 1,Phase 2
28 Immunologic Factors Phase 4,Phase 1,Phase 2
29 Topoisomerase Inhibitors Phase 4,Phase 1,Phase 2
30 Alkylating Agents Phase 4,Phase 1,Phase 2
31 Antibiotics, Antitubercular Phase 4,Phase 1,Phase 2
32 Antimitotic Agents Phase 4,Phase 1,Phase 2
33 Antineoplastic Agents, Phytogenic Phase 4,Phase 1,Phase 2
34 Antirheumatic Agents Phase 4,Phase 1,Phase 2
35 glucocorticoids Phase 4,Phase 1,Phase 2
36 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 1,Phase 2
37 Folic Acid Antagonists Phase 4
38 Dermatologic Agents Phase 4
39 Folate Phase 4
40 Vitamin B9 Phase 4
41 Nucleic Acid Synthesis Inhibitors Phase 4
42 Antimetabolites, Antineoplastic Phase 4
43 Antimetabolites Phase 4
44 Vitamin B Complex 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
Everolimus Approved Phase 1 159351-69-6 6442177 70789204
50
Miconazole Approved, Investigational, Vet_approved Phase 1 22916-47-8 4189

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:

42
T Cells, Skin, Spleen, Bone, Nk Cells, Bone Marrow, B Cells

Publications for Subcutaneous Panniculitis-Like T-Cell Lymphoma

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

(show top 50) (show all 186)
# Title Authors Year
1
Hematopoietic stem cell transplantation for subcutaneous panniculitis-like T-cell lymphoma: single center experience in an Asian population. ( 30547418 )
2019
2
Subcutaneous Panniculitis-Like T-Cell Lymphoma With Granulomas as the Predominant Feature. ( 30908292 )
2019
3
Successful treatment of a rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review. ( 30884048 )
2019
4
Frequent germline mutations of HAVCR2 in sporadic subcutaneous panniculitis-like T-cell lymphoma. ( 30792187 )
2019
5
Treatment of Facial Lipoatrophy Secondary to Subcutaneous Panniculitis-Like T-Cell Lymphoma. ( 30829756 )
2019
6
Pediatric Subcutaneous Panniculitis-like T-Cell Lymphoma With Hemophagocytosis Showing Complete Resolution With the BFM90 Protocol: Case Report and Review of Literature. ( 30730380 )
2019
7
Composite cutaneous lymphoma of diffuse large B-cell lymphoma-leg type and subcutaneous panniculitis-like T-cell lymphoma. ( 29851123 )
2018
8
Subcutaneous Panniculitis-Like T-Cell Lymphoma Versus Lupus Erythematosus Panniculitis: Distinction by Means of the Periadipocytic Cell Proliferation Index. ( 29742552 )
2018
9
A Case of Refractory Subcutaneous Panniculitis-like T-cell Lymphoma with Bone Marrow and Lung Involvement Treated Successfully with Oral Etoposide. ( 29881869 )
2018
10
The coexistence of lupus erythematosus panniculitis and subcutaneous panniculitis-like T-cell lymphoma in the same patient. ( 29892661 )
2018
11
Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma. ( 30289114 )
2018
12
Subcutaneous panniculitis-like T-cell lymphoma responsive to combination therapy with methotrexate and corticosteroids. ( 30677832 )
2018
13
Relapsed subcutaneous panniculitis-like T cell lymphoma evaluated by FDG PET/CT: A clinical case report. ( 30431571 )
2018
14
Clinicopathologic, immunophenotypic, and molecular analysis of subcutaneous panniculitis-like T-cell lymphoma: A retrospective study in a tertiary care center. ( 30350476 )
2018
15
Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients. ( 30126736 )
2018
16
Subcutaneous Panniculitis-Like T-cell Lymphoma in Dogs: Morphologic and Immunohistochemical Classification. ( 30071780 )
2018
17
Mycophenolate Mofetil for the Treatment of Subcutaneous Panniculitis-Like T-Cell Lymphoma: Case Report and Review of the Literature. ( 30077696 )
2018
18
Clinical characteristics, differential diagnosis, and treatment outcome of subcutaneous panniculitis-like T-cell lymphoma: a literature review of published Japanese cases. ( 28120776 )
2017
19
Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting as a Local Inflammation of a Thigh in an 8-Month-Old Child. ( 29085769 )
2017
20
Subcutaneous panniculitis-like T-cell lymphoma with haemophagocytic syndrome during tocilizumab therapy for juvenile idiopathic arthritis. ( 28079502 )
2017
21
Immunohistochemistry reveals an increased proportion of MYC-positive cells in subcutaneous panniculitis-like T-cell lymphoma compared with lupus panniculitis. ( 28800143 )
2017
22
Relapsed subcutaneous panniculitis-like T cell lymphoma: role of haploidentical hematopoietic stem cell transplant. ( 28879427 )
2017
23
Recurrent mutations in epigenetic modifiers and the PI3K/AKT/mTOR pathway in subcutaneous panniculitis-like T-cell lymphoma. ( 28294301 )
2017
24
Remission of subcutaneous panniculitis-like T-cell lymphoma in a pregnant woman after treatment with oral corticosteroids as monotherapy. ( 28361107 )
2017
25
Subcutaneous panniculitis-like T-cell lymphoma. ( 28127142 )
2017
26
CCR4 and CCR5 expression in a case of subcutaneous panniculitis-like T-cell lymphoma. ( 28721932 )
2017
27
Hemophagocytic lymphohistiocytosis associated with subcutaneous panniculitis-like T-cell lymphoma. ( 27879081 )
2016
28
Subcutaneous panniculitis like T-cell lymphoma. ( 27728567 )
2016
29
Subcutaneous panniculitis-like T-cell lymphoma with macrophage activation syndrome treated by cyclosporine and prednisolone. ( 27990393 )
2016
30
Long-term remission of subcutaneous panniculitis-like T-cell lymphoma with central nervous system involvement: A case report. ( 27347188 )
2016
31
Relapsed and refractory subcutaneous panniculitis-like T-cell lymphoma with excellent response to cyclosporine: a case report and literature review. ( 26873089 )
2016
32
Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Rare Tumour. ( 27437288 )
2016
33
Subcutaneous Panniculitis-like T Cell Lymphoma Mimicking Early-Onset Nodular Panniculitis. ( 27342380 )
2016
34
Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome in a Child. ( 26764131 )
2016
35
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
36
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
37
Kikuchi-Fujimoto Disease Associated With Subcutaneous Panniculitis-Like T-Cell Lymphoma. ( 26844615 )
2016
38
Pediatric subcutaneous panniculitis-like T-cell lymphoma presenting as paraparesis: An unreported manifestation. ( 27625250 )
2016
39
Subcutaneous panniculitis like T-cell lymphoma in a case of sarcoidosis: A rare association. ( 27728564 )
2016
40
Case report of subcutaneous panniculitis-like T-cell lymphoma complicated by eyelid swelling. ( 27440138 )
2016
41
Atypical lymphocytic lobular panniculitis: an overlap condition with features of subcutaneous panniculitis-like T-cell lymphoma and lupus profundus. ( 27807018 )
2016
42
Subcutaneous Panniculitis-like T cell Lymphoma: Immunosuppressive Drugs Induce Better Response than Polychemotherapy. ( 27722764 )
2016
43
Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography evaluation of subcutaneous panniculitis-like T cell lymphoma and treatment response. ( 27672640 )
2016
44
Useful Parameters for Distinguishing Subcutaneous Panniculitis-like T-Cell Lymphoma From Lupus Erythematosus Panniculitis. ( 26796503 )
2016
45
Systemic T cell lymphoma mimicking subcutaneous panniculitis-like T-cell lymphoma. ( 27444745 )
2016
46
A rare case of fever of unknown origin: subcutaneous panniculitis-like T-cell lymphoma (SPTCL). ( 26290563 )
2015
47
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
48
Subcutaneous Panniculitis-like T-cell Lymphoma Presenting With Diffuse Cutaneous Edema in a 2-Year-old Child. ( 25739026 )
2015
49
CCR5 and CXCR3 expression in a case of subcutaneous panniculitis-like T-cell lymphoma. ( 26301865 )
2015
50
Fever and generalized erythema caused by subcutaneous panniculitis-like T-cell lymphoma. ( 26621148 )
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 8.32 SLC17A5

Sources for Subcutaneous Panniculitis-Like T-Cell Lymphoma

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10 dbSNP
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17 EFO
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35 ICD10 via Orphanet
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45 MeSH
46 MESH via Orphanet
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59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
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75 UMLS via Orphanet
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