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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

Primary Cutaneous Lymphomas in Thailand: A 10-Year Retrospective Study.
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
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part II: Prognosis and management.
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
NK/T-cell lymphoma, nasal type, γδ T-cell lymphoma, and CD8-positive epidermotropic T-cell lymphoma-clinical and histopathologic features, differential diagnosis, and treatment.
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

Classifications of cutaneous lymphomas and lymphoproliferative disorders: An update from the EORTC cutaneous lymphoma histopathology group.
Kempf W, Mitteldorf C, Cerroni L, Willemze R, Berti E, Guenova E, Scarisbrick JJ, Battistella M
J Eur Acad Dermatol Venereol 2024 Aug;38(8):1491-1503. Epub 2024 Apr 6 doi: 10.1111/jdv.19987. PMID: 38581201
Aggressive Cutaneous Lymphomas and Their Mimics.
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
Primary cutaneous gamma/delta T-cell lymphoma in Taiwan: A series of six cases with frequent solitary presentation and relatively indolent behavior.
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
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
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
Gamma-delta t-cell lymphomas.
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

Classifications of cutaneous lymphomas and lymphoproliferative disorders: An update from the EORTC cutaneous lymphoma histopathology group.
Kempf W, Mitteldorf C, Cerroni L, Willemze R, Berti E, Guenova E, Scarisbrick JJ, Battistella M
J Eur Acad Dermatol Venereol 2024 Aug;38(8):1491-1503. Epub 2024 Apr 6 doi: 10.1111/jdv.19987. PMID: 38581201
Aggressive Cutaneous Lymphomas and Their Mimics.
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
Gamma/Delta Phenotype in Primary Cutaneous T-cell Lymphomas and Lymphoid Proliferations: Challenges for Diagnosis and Classification.
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
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
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
Gamma-delta t-cell lymphomas.
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

Bendamustine Monotherapy for Primary Cutaneous Gamma-Delta T-Cell Lymphoma.
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
Primary Cutaneous Gamma-Delta T-cell Lymphoma: Not an Aggressive Disease in All Cases.
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
Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.
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
Gamma-delta t-cell lymphomas.
Foppoli M, Ferreri AJ
Eur J Haematol 2015 Mar;94(3):206-18. Epub 2014 Oct 1 doi: 10.1111/ejh.12439. PMID: 25154298
Systemic corticosteroids for subcutaneous panniculitis-like T-cell lymphoma.
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

Aggressive Cutaneous Lymphomas and Their Mimics.
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
Primary cutaneous gamma/delta T-cell lymphoma in Taiwan: A series of six cases with frequent solitary presentation and relatively indolent behavior.
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
The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) T-cell lymphoma: A systematic review and meta-analysis.
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
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
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
Recent Advances in Cutaneous T-cell Lymphoma: Diagnostic and Prognostic Considerations.
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

The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) T-cell lymphoma: A systematic review and meta-analysis.
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
Florid splenic γ/δ T-cell proliferation in patients with splenomegaly and cytopenias: a "high stakes" diagnostic challenge.
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
Primary Cutaneous Gamma-Delta (γ/δ) T-cell Lymphoma: An Unusual Case With Very Subtle Histopathological Findings.
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
Human myxovirus resistance protein 1 (MxA) as a useful marker in the differential diagnosis of subcutaneous lymphoma vs. lupus erythematosus profundus.
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

The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) T-cell lymphoma: A systematic review and meta-analysis.
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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