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Primary cutaneous T-cell lymphoma

MedGen UID:
36336
•Concept ID:
C0079773
•
Neoplastic Process
Synonym: Cutaneous T-cell lymphoma
SNOMED CT: Primary cutaneous T-cell lymphoma (400122007); Cutaneous T-cell lymphoma (400122007); CTCL - Cutaneous T-cell lymphoma (400122007); Cutaneous T-cell lymphoma (1162973007); Cutaneous lymphoma (1162973007); Primary cutaneous T-cell lymphoma (1187136005)
 
HPO: HP:0012192
Monarch Initiative: MONDO:0015758
Orphanet: ORPHA171901

Definition

A type of T-cell lymphoma that exhibits malignant infiltration of the skin. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary cutaneous T-cell lymphoma
Follow this link to review classifications for Primary cutaneous T-cell lymphoma in Orphanet.

Professional guidelines

PubMed

Evaluation of mortality, prognostic parameters, and treatment efficacy in mycosis fungoides.
Porkert S, Griss J, Hudelist-Venz M, Steiner I, Valencak J, Weninger W, Brunner PM, Jonak C
J Dtsch Dermatol Ges 2024 Apr;22(4):532-550. Epub 2024 Mar 5 doi: 10.1111/ddg.15331. PMID: 38444271
An update on mechanisms of pruritus and their potential treatment in primary cutaneous T-cell lymphoma.
Hu M, Scheffel J, Elieh-Ali-Komi D, Maurer M, Hawro T, Metz M
Clin Exp Med 2023 Dec;23(8):4177-4197. Epub 2023 Aug 9 doi: 10.1007/s10238-023-01141-x. PMID: 37555911Free PMC Article
Brentuximab vedotin therapy for CD30-positive cutaneous T-cell lymphoma: a targeted approach to management.
Scarisbrick JJ
Future Oncol 2017 Nov;13(27):2405-2411. Epub 2017 Aug 14 doi: 10.2217/fon-2017-0263. PMID: 28805086

Recent clinical studies

Etiology

Genomic profiling of mycosis fungoides identifies patients at high risk of disease progression.
Fléchon L, Arib I, Dutta AK, Hasan Bou Issa L, Sklavenitis-Pistofidis R, Tilmont R, Stewart C, Dubois R, Poulain S, Copin MC, Javed S, Nudel M, Cavalieri D, Escure G, Gower N, Chauvet P, Gazeau N, Saade C, Thiam MB, Ouelkite-Oumouchal A, Gaggero S, Cailliau É, Faiz S, Carpentier O, Duployez N, Idziorek T, Mortier L, Figeac M, Preudhomme C, Quesnel B, Mitra S, Morschhauser F, Getz G, Ghobrial IM, Manier S
Blood Adv 2024 Jun 25;8(12):3109-3119. doi: 10.1182/bloodadvances.2023012125. PMID: 38513135Free PMC Article
Primary cutaneous T-cell lymphoma: a review of the most common entities with focus on recent updates.
Saleh JS, Subtil A, Hristov AC
Hum Pathol 2023 Oct;140:75-100. Epub 2023 Oct 4 doi: 10.1016/j.humpath.2023.09.009. PMID: 37802757
Rare T-Cell Subtypes.
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
Cutaneous lymphomas.
Klemke CD
J Dtsch Dermatol Ges 2014 Jan;12(1):7-28; quiz 29-30. doi: 10.1111/ddg.12237. PMID: 24393310
Subcutaneous panniculitis-like T-cell lymphoma.
Tomasini D, Berti E
G Ital Dermatol Venereol 2013 Aug;148(4):395-411. PMID: 23900161

Diagnosis

Primary cutaneous T-cell lymphoma: a review of the most common entities with focus on recent updates.
Saleh JS, Subtil A, Hristov AC
Hum Pathol 2023 Oct;140:75-100. Epub 2023 Oct 4 doi: 10.1016/j.humpath.2023.09.009. PMID: 37802757
Rare T-Cell Subtypes.
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
Clinicopathologic Variants of Mycosis Fungoides.
Muñoz-González H, Molina-Ruiz AM, Requena L
Actas Dermosifiliogr 2017 Apr;108(3):192-208. Epub 2016 Nov 18 doi: 10.1016/j.ad.2016.08.009. PMID: 27871620
Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers.
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C
J Am Acad Dermatol 2014 Feb;70(2):205.e1-16; quiz 221-2. doi: 10.1016/j.jaad.2013.07.049. PMID: 24438969
Cutaneous lymphomas.
Klemke CD
J Dtsch Dermatol Ges 2014 Jan;12(1):7-28; quiz 29-30. doi: 10.1111/ddg.12237. PMID: 24393310

Therapy

Primary Cutaneous T-cell Lymphoma with Aberrant Expression of CD20.
Frings VG, Roth S, Riedmiller AL, Schäfer K, Goebeler M, Rosenwald A, Geissinger E, Wobser M
Acta Derm Venereol 2017 Apr 6;97(4):534-536. doi: 10.2340/00015555-2576. PMID: 27840885
Therapeutic Update: Update on Cutaneous and Systemic Therapy for Primary Cutaneous T Cell Lymphoma, Mycosis Fungoides.
Lo Sicco K, Latkowski JA
J Drugs Dermatol 2015 Dec;14(12):1381-3. PMID: 26659928
Cutaneous lymphomas.
Klemke CD
J Dtsch Dermatol Ges 2014 Jan;12(1):7-28; quiz 29-30. doi: 10.1111/ddg.12237. PMID: 24393310
Subcutaneous panniculitis-like T-cell lymphoma.
Tomasini D, Berti E
G Ital Dermatol Venereol 2013 Aug;148(4):395-411. PMID: 23900161
Pegylated doxorubicin for primary cutaneous T cell lymphoma: a report on ten patients with follow-up.
Wollina U, Graefe T, Kaatz M
Ann N Y Acad Sci 2001 Sep;941:214-6. PMID: 11594577

Prognosis

Primary cutaneous T-cell lymphoma: a review of the most common entities with focus on recent updates.
Saleh JS, Subtil A, Hristov AC
Hum Pathol 2023 Oct;140:75-100. Epub 2023 Oct 4 doi: 10.1016/j.humpath.2023.09.009. PMID: 37802757
Single-cell RNA sequencing reveals markers of disease progression in primary cutaneous T-cell lymphoma.
Rindler K, Jonak C, Alkon N, Thaler FM, Kurz H, Shaw LE, Stingl G, Weninger W, Halbritter F, Bauer WM, Farlik M, Brunner PM
Mol Cancer 2021 Sep 28;20(1):124. doi: 10.1186/s12943-021-01419-2. PMID: 34583709Free PMC Article
Clinicopathologic Variants of Mycosis Fungoides.
Muñoz-González H, Molina-Ruiz AM, Requena L
Actas Dermosifiliogr 2017 Apr;108(3):192-208. Epub 2016 Nov 18 doi: 10.1016/j.ad.2016.08.009. PMID: 27871620
Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers.
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C
J Am Acad Dermatol 2014 Feb;70(2):205.e1-16; quiz 221-2. doi: 10.1016/j.jaad.2013.07.049. PMID: 24438969
Cutaneous lymphomas.
Klemke CD
J Dtsch Dermatol Ges 2014 Jan;12(1):7-28; quiz 29-30. doi: 10.1111/ddg.12237. PMID: 24393310

Clinical prediction guides

Characterization of cells and mediators associated with pruritus in primary cutaneous T-cell lymphomas.
Hu M, Scheffel J, Frischbutter S, Steinert C, Reidel U, Spindler M, Przybyłowicz K, Hawro M, Maurer M, Metz M, Hawro T
Clin Exp Med 2024 Jul 28;24(1):171. doi: 10.1007/s10238-024-01407-y. PMID: 39068637Free PMC Article
Phase I/II clinical trial of brentuximab vedotin for pretreated Japanese patients with CD30-positive cutaneous T-cell lymphoma.
Hirai Y, Sakurai J, Yoshida S, Kikuchi T, Mitsuhashi T, Miyake T, Fujimura T, Abe R, Fujikawa H, Boki H, Suga H, Shibata S, Miyagaki T, Shimauchi T, Kiyohara E, Kawakami Y, Morizane S
J Dermatol 2024 Aug;51(8):1037-1049. Epub 2024 Jun 14 doi: 10.1111/1346-8138.17324. PMID: 38874430Free PMC Article
Evaluation of mortality, prognostic parameters, and treatment efficacy in mycosis fungoides.
Porkert S, Griss J, Hudelist-Venz M, Steiner I, Valencak J, Weninger W, Brunner PM, Jonak C
J Dtsch Dermatol Ges 2024 Apr;22(4):532-550. Epub 2024 Mar 5 doi: 10.1111/ddg.15331. PMID: 38444271
Single-cell RNA sequencing reveals markers of disease progression in primary cutaneous T-cell lymphoma.
Rindler K, Jonak C, Alkon N, Thaler FM, Kurz H, Shaw LE, Stingl G, Weninger W, Halbritter F, Bauer WM, Farlik M, Brunner PM
Mol Cancer 2021 Sep 28;20(1):124. doi: 10.1186/s12943-021-01419-2. PMID: 34583709Free PMC Article
Primary cutaneous T-cell lymphoma: experience from the Peruvian National Cancer Institute.
Ruiz R, Morante Z, Mantilla R, Mas L, Casanova L, Gomez HL
An Bras Dermatol 2017 Sep-Oct;92(5):649-654. doi: 10.1590/abd1806-4841.20176825. PMID: 29166501Free PMC Article

Recent systematic reviews

What are the supportive and palliative care needs of patients with cutaneous T-cell lymphoma and their caregivers? A systematic review of the evidence.
Beynon T, Radcliffe E, Child F, Orlowska D, Whittaker S, Lawson S, Selman L, Harding R
Br J Dermatol 2014 Mar;170(3):599-608. doi: 10.1111/bjd.12644. PMID: 24116897
Allogeneic stem cell transplantation versus conventional therapy for advanced primary cutaneous T-cell lymphoma.
Schlaak M, Pickenhain J, Theurich S, Skoetz N, von Bergwelt-Baildon M, Kurschat P
Cochrane Database Syst Rev 2013 Aug 29;2013(8):CD008908. doi: 10.1002/14651858.CD008908.pub3. PMID: 23986525Free PMC Article
Allogeneic stem cell transplantation for advanced primary cutaneous T-cell lymphoma: a systematic review.
Schlaak M, Theurich S, Pickenhain J, Skoetz N, Kurschat P, von Bergwelt-Baildon M
Crit Rev Oncol Hematol 2013 Jan;85(1):21-31. Epub 2012 Jul 18 doi: 10.1016/j.critrevonc.2012.06.002. PMID: 22819279
Allogeneic stem cell transplantation versus conventional therapy for advanced primary cutaneous T-cell lymphoma.
Schlaak M, Pickenhain J, Theurich S, Skoetz N, von Bergwelt-Baildon M, Kurschat P
Cochrane Database Syst Rev 2012 Jan 18;1:CD008908. doi: 10.1002/14651858.CD008908.pub2. PMID: 22258991

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