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Primary cutaneous gamma-delta t-cell lymphoma

MedGen UID:
313069
Concept ID:
C1707547
Neoplastic Process
Synonyms: Cutaneous Gamma/Delta T-Cell Lymphoma; Peripheral Gamma-Delta T-Cell Lymphoma; Primary Cutaneous Gamma-Delta T-Cell Lymphoma; Primary cutaneous gamma-delta T-cell lymphoma; primary cutaneous gamma-delta t-cell lymphoma; Primary cutaneous gamma-delta-positive T-cell lymphoma
SNOMED CT: Primary cutaneous gamma-delta T-cell lymphoma (450908002); Primary cutaneous gamma-delta-positive T-cell lymphoma (733627006); Primary cutaneous gamma-delta T-cell lymphoma (733627006)
 
Monarch Initiative: MONDO:0850459

Definition

A rare usually aggressive subtype of cutaneous T-cell lymphoma with characteristics of infiltration of the epidermis, dermis or subcutaneous tissue by a clonal population of mature, gamma/delta positive cytotoxic T-cells. Typically it presents with ulcerating plaques on the skin of the extremities, however, frequent involvement of mucosal and extranodal sites (such as the nasal cavity, gastrointestinal tract or lungs) is also observed. Infiltration of lymph nodes, spleen and bone marrow is uncommon and resistance to multilineage chemotherapy is reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary cutaneous gamma-delta t-cell lymphoma

Professional guidelines

PubMed

Pruksaeakanan C, Teyateeti P, Patthamalai P, Thumrongtharadol J, Chairatchaneeboon M
Biomed Res Int 2021;2021:4057661. Epub 2021 Jun 11 doi: 10.1155/2021/4057661. PMID: 34235215Free PMC Article
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
Geller S, Myskowski PL, Pulitzer M
Semin Cutan Med Surg 2018 Mar;37(1):30-38. doi: 10.12788/j.sder.2018.020. PMID: 29719018Free PMC Article

Recent clinical studies

Etiology

Moy AP, Pulitzer MP
Surg Pathol Clin 2023 Jun;16(2):361-383. Epub 2023 Mar 10 doi: 10.1016/j.path.2023.01.009. PMID: 37149363
Chen BJ, Wang RC, Jhuang JY, Chen SW, Su YZ, Tseng CE, Chiang CT, Wu YT, Chuang SS
J Cutan Pathol 2022 Apr;49(4):350-357. Epub 2021 Nov 30 doi: 10.1111/cup.14169. PMID: 34778979
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
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
Foppoli M, Ferreri AJ
Eur J Haematol 2015 Mar;94(3):206-18. Epub 2014 Oct 1 doi: 10.1111/ejh.12439. PMID: 25154298

Diagnosis

Moy AP, Pulitzer MP
Surg Pathol Clin 2023 Jun;16(2):361-383. Epub 2023 Mar 10 doi: 10.1016/j.path.2023.01.009. PMID: 37149363
Torres-Cabala CA, Huen A, Iyer SP, Miranda RN
Surg Pathol Clin 2021 Jun;14(2):177-194. Epub 2021 Apr 29 doi: 10.1016/j.path.2021.03.001. PMID: 34023099
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
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
Foppoli M, Ferreri AJ
Eur J Haematol 2015 Mar;94(3):206-18. Epub 2014 Oct 1 doi: 10.1111/ejh.12439. PMID: 25154298

Therapy

Kreuter A, Koushk-Jalali B, Mitrakos G, Oellig F, Assaf C, Cerroni L, Tigges C
JAMA Dermatol 2020 Oct 1;156(10):1029-1030. doi: 10.1001/jamadermatol.2020.1231. PMID: 32584930
Khallaayoune M, Grange F, Condamina M, Szablewski V, Guillot B, Dereure O
Acta Derm Venereol 2020 Jan 23;100(1):adv00035. doi: 10.2340/00015555-3340. PMID: 31612963Free PMC Article
Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145
Foppoli M, Ferreri AJ
Eur J Haematol 2015 Mar;94(3):206-18. Epub 2014 Oct 1 doi: 10.1111/ejh.12439. PMID: 25154298
Guenova E, Schanz S, Hoetzenecker W, DeSimone JA, Mehra T, Voykov B, Urosevic-Maiwald M, Berneburg M, Dummer R, French LE, Kerl K, Kamarashev J, Fierlbeck G, Cozzio A
Br J Dermatol 2014 Oct;171(4):891-4. Epub 2014 Sep 2 doi: 10.1111/bjd.13053. PMID: 24725144

Prognosis

Moy AP, Pulitzer MP
Surg Pathol Clin 2023 Jun;16(2):361-383. Epub 2023 Mar 10 doi: 10.1016/j.path.2023.01.009. PMID: 37149363
Goyal A, O'Leary D, Duncan LM
J Cutan Pathol 2021 Dec;48(12):1449-1454. Epub 2021 Jul 2 doi: 10.1111/cup.14082. PMID: 34151455
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
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
Cocks M, Porcu P, Wick MR, Gru AA
Surg Pathol Clin 2019 Sep;12(3):783-803. doi: 10.1016/j.path.2019.03.006. PMID: 31352988

Clinical prediction guides

Goyal A, O'Leary D, Duncan LM
J Cutan Pathol 2021 Dec;48(12):1449-1454. Epub 2021 Jul 2 doi: 10.1111/cup.14082. PMID: 34151455
Zhang S, Bayerl MG
Hum Pathol 2017 Aug;66:216-221. Epub 2017 Feb 22 doi: 10.1016/j.humpath.2017.01.015. PMID: 28235627
Ramani NS, Curry JL, Merrill ED, Aung PP, Prieto V, Tetzlaff MT, Duvic M, Miranda RN, Torres-Cabala C
Am J Dermatopathol 2016 Oct;38(10):e147-9. doi: 10.1097/DAD.0000000000000608. PMID: 27533072
Wang X, Magro CM
Eur J Dermatol 2012 Sep-Oct;22(5):629-33. doi: 10.1684/ejd.2012.1790. PMID: 23026440

Recent systematic reviews

Goyal A, O'Leary D, Duncan LM
J Cutan Pathol 2021 Dec;48(12):1449-1454. Epub 2021 Jul 2 doi: 10.1111/cup.14082. PMID: 34151455

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