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Subcutaneous panniculitis-like T-cell lymphoma(SPTCL)

MedGen UID:
99306
Concept ID:
C0522624
Neoplastic Process
Synonym: SPTCL
SNOMED CT: Subcutaneous panniculitic T-cell lymphoma (103682005); Subcutaneous panniculitic cutaneous T-cell lymphoma (404133000); Subcutaneous panniculitis-like T-cell lymphoma (103682005); Subcutaneous panniculitis-like T-cell lymphoma (404133000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Gene (location): HAVCR2 (5q33.3)
 
HPO: HP:0034403
Monarch Initiative: MONDO:0019475
OMIM®: 618398
Orphanet: ORPHA86884

Definition

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). [from OMIM]

Clinical features

From HPO
Subcutaneous panniculitis-like T-cell lymphoma
MedGen UID:
99306
Concept ID:
C0522624
Neoplastic Process
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).
Anemia
MedGen UID:
1526
Concept ID:
C0002871
Disease or Syndrome
A reduction in erythrocytes volume or hemoglobin concentration.
Pancytopenia
MedGen UID:
18281
Concept ID:
C0030312
Disease or Syndrome
An abnormal reduction in numbers of all blood cell types (red blood cells, white blood cells, and platelets).
Hypofibrinogenemia
MedGen UID:
107511
Concept ID:
C0553681
Disease or Syndrome
Decreased concentration of fibrinogen in the blood.
Autoimmunity
MedGen UID:
2136
Concept ID:
C0004368
Pathologic Function
The occurrence of an immune reaction against the organism's own cells or tissues.
Panniculitis
MedGen UID:
45301
Concept ID:
C0030326
Disease or Syndrome
Inflammation of subcutaneous adipose tissue.
Splenomegaly
MedGen UID:
52469
Concept ID:
C0038002
Finding
Abnormal increased size of the spleen.
Hemophagocytosis
MedGen UID:
163750
Concept ID:
C0876991
Disease or Syndrome
Phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors in bone marrow and other tissues.
Fever
MedGen UID:
5169
Concept ID:
C0015967
Sign or Symptom
Body temperature elevated above the normal range.
Increased circulating ferritin concentration
MedGen UID:
69130
Concept ID:
C0241013
Finding
Increased concentration of ferritin in the blood circulation.
Hypertriglyceridemia
MedGen UID:
167238
Concept ID:
C0813230
Finding
An abnormal increase in the level of triglycerides in the blood.
Facial edema
MedGen UID:
154241
Concept ID:
C0542571
Pathologic Function
Swelling due to an excessive accumulation of fluid in facial tissues.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSubcutaneous panniculitis-like T-cell lymphoma

Conditions with this feature

Subcutaneous panniculitis-like T-cell lymphoma
MedGen UID:
99306
Concept ID:
C0522624
Neoplastic Process
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).

Professional guidelines

PubMed

Oh Y, Stoll JR, Moskowitz A, Pulitzer M, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1093-1106. Epub 2021 May 1 doi: 10.1016/j.jaad.2021.04.081. PMID: 33945836
Roschewski M, Wilson WH
Oncology (Williston Park) 2010 Jan;24(1):94-100. PMID: 20187328Free PMC Article
Evens AM, Gartenhaus RB
Curr Treat Options Oncol 2004 Aug;5(4):289-303. doi: 10.1007/s11864-004-0020-8. PMID: 15233906

Recent clinical studies

Etiology

Koh J, Jang I, Mun S, Lee C, Cha HJ, Oh YH, Kim JM, Han JH, Paik JH, Cho J, Ko YH, Park CS, Go H, Huh J, Kim K, Jeon YK
Blood Adv 2021 Oct 26;5(20):3919-3930. doi: 10.1182/bloodadvances.2021004562. PMID: 34535012Free PMC Article
Machan S, Rodríguez M, Alonso-Alonso R, Manso R, Pérez-Buira S, Borregón J, Rodríguez-Peralto JL, Cerroni L, Haro R, García C, García Toro E, Estrach T, García-Herrera A, Ferrer B, González-Cruz C, Segues N, Afonso-Martin JL, Peñate Y, Monteagudo C, Limeres-Gonzalez MÁ, González-Núñez MÁ, Torres MÁT, Cereceda L, Córdoba R, Piris MÁ, Requena L, María Rodríguez-Pinilla S
Leuk Lymphoma 2021 Sep;62(9):2130-2140. Epub 2021 May 8 doi: 10.1080/10428194.2021.1901098. PMID: 33966586
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
van der Weyden C, McCormack C, Lade S, Johnstone RW, Prince HM
Cancer Treat Res 2019;176:195-224. doi: 10.1007/978-3-319-99716-2_10. PMID: 30596220
Tomasini D, Berti E
G Ital Dermatol Venereol 2013 Aug;148(4):395-411. PMID: 23900161

Diagnosis

Alsomali DY, Bakshi N, Kharfan-Dabaja M, El Fakih R, Aljurf M
Hematol Oncol Stem Cell Ther 2023 Jan 17;16(2):110-116. doi: 10.1016/j.hemonc.2021.04.001. PMID: 34015273
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
van der Weyden C, McCormack C, Lade S, Johnstone RW, Prince HM
Cancer Treat Res 2019;176:195-224. doi: 10.1007/978-3-319-99716-2_10. PMID: 30596220
Damasco F, Akilov OE
Hematol Oncol Clin North Am 2019 Feb;33(1):135-148. doi: 10.1016/j.hoc.2018.08.004. PMID: 30497671
Tomasini D, Berti E
G Ital Dermatol Venereol 2013 Aug;148(4):395-411. PMID: 23900161

Therapy

Moonla C, Polprasert C, Komvilaisak P, Rattanathammethee T, Kongkiatkamon S, Wudhikarn K, Kobbuaklee S, Boonyabaramee P, Tangcheewinsirikul N, Pakakasama S, Rujkijyanont P, Choed-Amphai C, Phuakpet K, Pongudom S, Bunworasate U, Sukswai N, Sosothikul D, Rojnuckarin P
Haematologica 2023 Oct 1;108(10):2743-2752. doi: 10.3324/haematol.2022.282419. PMID: 37051767Free PMC Article
Cavanna L, Grassi SO, Ruffini L, Michieletti E, Carella E, Palli D, Zangrandi A, Inzerilli N, Bernuzzi P, Di Nunzio C, Citterio C
Medicina (Kaunas) 2023 Jan 12;59(1) doi: 10.3390/medicina59010157. PMID: 36676781Free PMC Article
Grinich E, Koon SM, Cascio MJ, Fett N
Dermatol Online J 2018 Sep 15;24(9) PMID: 30677832
Ohtsuka M, Miura T, Yamamoto T
Eur J Dermatol 2017 Feb 1;27(1):34-41. doi: 10.1684/ejd.2016.2914. PMID: 28120776
Tomasini D, Berti E
G Ital Dermatol Venereol 2013 Aug;148(4):395-411. PMID: 23900161

Prognosis

Koh J, Jang I, Mun S, Lee C, Cha HJ, Oh YH, Kim JM, Han JH, Paik JH, Cho J, Ko YH, Park CS, Go H, Huh J, Kim K, Jeon YK
Blood Adv 2021 Oct 26;5(20):3919-3930. doi: 10.1182/bloodadvances.2021004562. PMID: 34535012Free PMC Article
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
Damasco F, Akilov OE
Hematol Oncol Clin North Am 2019 Feb;33(1):135-148. doi: 10.1016/j.hoc.2018.08.004. PMID: 30497671
Hathuc VM, Hristov AC, Smith LB
Arch Pathol Lab Med 2017 Nov;141(11):1469-1475. doi: 10.5858/arpa.2017-0230-RA. PMID: 29072952
Tomasini D, Berti E
G Ital Dermatol Venereol 2013 Aug;148(4):395-411. PMID: 23900161

Clinical prediction guides

Moonla C, Polprasert C, Komvilaisak P, Rattanathammethee T, Kongkiatkamon S, Wudhikarn K, Kobbuaklee S, Boonyabaramee P, Tangcheewinsirikul N, Pakakasama S, Rujkijyanont P, Choed-Amphai C, Phuakpet K, Pongudom S, Bunworasate U, Sukswai N, Sosothikul D, Rojnuckarin P
Haematologica 2023 Oct 1;108(10):2743-2752. doi: 10.3324/haematol.2022.282419. PMID: 37051767Free PMC Article
Guitart J, Mangold AR, Martinez-Escala ME, Walker CJ, Comfere NI, Pulitzer M, Rieger KE, Torres-Cabala CA, Pincus LB, Kumar ES, Wang EBK, Park KE, Espinosa ML, Duvic M, Kim YH, Horwitz S
JAMA Dermatol 2022 Oct 1;158(10):1167-1174. doi: 10.1001/jamadermatol.2022.3347. PMID: 36001337Free PMC Article
Bellahsen-Harrar Y, Badrignans M, Cherif K, Papouin B, Ingen-Housz-Oro S, Ortonne N
Am J Dermatopathol 2022 Sep 1;44(9):675-676. Epub 2022 Apr 11 doi: 10.1097/DAD.0000000000002203. PMID: 35475979
Ohtsuka M, Miura T, Yamamoto T
Eur J Dermatol 2017 Feb 1;27(1):34-41. doi: 10.1684/ejd.2016.2914. PMID: 28120776
Guitart J
Dermatol Ther 2010 Jul-Aug;23(4):350-5. doi: 10.1111/j.1529-8019.2010.01335.x. PMID: 20666822

Recent systematic reviews

Alsomali DY, Bakshi N, Kharfan-Dabaja M, El Fakih R, Aljurf M
Hematol Oncol Stem Cell Ther 2023 Jan 17;16(2):110-116. doi: 10.1016/j.hemonc.2021.04.001. PMID: 34015273
Sun X, Ji L, Li G, Nong L, Zhang W, Xie W, Zhang Z
Clin Rheumatol 2022 Mar;41(3):929-934. Epub 2021 Nov 16 doi: 10.1007/s10067-021-05992-1. PMID: 34786628

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