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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

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPrimary cutaneous T-cell lymphoma

Disorder by Site
- Hematopoietic and Lymphoid System Disorder
  - Hematologic disorder
    - Hematopoietic and lymphoid cell neoplasm
      - Cutaneous Hematopoietic and Lymphoid Cell Neoplasm
        Cutaneous Mature T-Cell and NK-Cell Neoplasm
        Primary cutaneous T-cell lymphoma
        Advanced Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
        Early Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
        Lymphomatoid papulosis
        Lymphomatoid Papulosis with DUSP22-IRF4 Gene Rearrangement
        Type A Lymphomatoid Papulosis
        Type B Lymphomatoid Papulosis
        Type C Lymphomatoid Papulosis
        Type D Lymphomatoid Papulosis
        Type E Lymphomatoid Papulosis
        Mycosis fungoides
        Mycosis Fungoides by AJCC v7 Stage
        Stage I Mycosis Fungoides AJCC v7
        Stage II Mycosis Fungoides AJCC v7
        Stage III Mycosis Fungoides AJCC v7
        Stage IV Mycosis Fungoides AJCC v7
        Pagetoid Reticulosis
        Recurrent Mycosis Fungoides
        Recurrent Transformed Mycosis Fungoides
        Refractory Mycosis Fungoides
        Refractory Transformed Mycosis Fungoides
        Transformed Mycosis Fungoides
        Mycosis fungoides variant
        Folliculotropic mycosis fungoides
        Granulomatous slack skin disease
        Primary cutaneous anaplastic large cell lymphoma
        Adult Primary Cutaneous Anaplastic Large Cell Lymphoma
        Childhood Primary Cutaneous Anaplastic Large Cell Lymphoma
        Recurrent Primary Cutaneous Anaplastic Large Cell Lymphoma
        Refractory Primary Cutaneous Anaplastic Large Cell Lymphoma
        Primary Cutaneous Peripheral T-Cell Lymphoma, Rare Subtype
        Primary Cutaneous Acral CD8-Positive T-Cell Lymphoma
        Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma
        Primary cutaneous gamma-delta t-cell lymphoma
        Recurrent Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
        Recurrent Subcutaneous Panniculitis-Like T-Cell Lymphoma
        Refractory Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
        Refractory Subcutaneous Panniculitis-Like T-Cell Lymphoma
        Sezary syndrome
        Recurrent Sezary Syndrome
        Refractory Sezary Syndrome
        Subcutaneous panniculitis-like T-cell lymphoma

Follow this link to review classifications for Primary cutaneous T-cell lymphoma in Orphanet.

Professional guidelines

PubMed

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

Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions.

Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C
J Am Acad Dermatol 2014 Feb;70(2):223.e1-17; quiz 240-2. doi: 10.1016/j.jaad.2013.08.033. PMID: 24438970

Primary cutaneous T-cell lymphoma: review and current concepts.

Siegel RS, Pandolfino T, Guitart J, Rosen S, Kuzel TM
J Clin Oncol 2000 Aug;18(15):2908-25. doi: 10.1200/JCO.2000.18.15.2908. PMID: 10920140

See all (13)

Recent clinical studies

Etiology

Pathogenesis and Therapy of Primary Cutaneous T-Cell Lymphoma: Collegium Internationale Allergologicum (CIA) Update 2020.

Bobrowicz M, Fassnacht C, Ignatova D, Chang YT, Dimitriou F, Guenova E
Int Arch Allergy Immunol 2020;181(10):733-745. Epub 2020 Jul 20 doi: 10.1159/000509281. PMID: 32690848

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

Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions.

Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C
J Am Acad Dermatol 2014 Feb;70(2):223.e1-17; quiz 240-2. doi: 10.1016/j.jaad.2013.08.033. PMID: 24438970

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

See all (81)

Diagnosis

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

Subcutaneous panniculitis-like T-cell lymphoma.

Tomasini D, Berti E
G Ital Dermatol Venereol 2013 Aug;148(4):395-411. PMID: 23900161

See all (103)

Therapy

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 advances in primary cutaneous T-cell lymphoma.

DeSimone JA, Sodha P, Ignatova D, Dummer R, Cozzio A, Guenova E
Curr Opin Oncol 2015 Mar;27(2):128-33. doi: 10.1097/CCO.0000000000000161. PMID: 25594623

Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions.

Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C
J Am Acad Dermatol 2014 Feb;70(2):223.e1-17; quiz 240-2. doi: 10.1016/j.jaad.2013.08.033. PMID: 24438970

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

See all (59)

Prognosis

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: 34583709 Free 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 II. Prognosis, management, and future directions.

Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C
J Am Acad Dermatol 2014 Feb;70(2):223.e1-17; quiz 240-2. doi: 10.1016/j.jaad.2013.08.033. PMID: 24438970

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

See all (98)

Clinical prediction guides

Thirty Years of Experience With Ocular Adnexal T-Cell Lymphoma.

Manta AI, Wilson-Pogmore AA, Sullivan TJ
Ophthalmic Plast Reconstr Surg 2023 May-Jun 01;39(3):266-274. Epub 2023 Jan 24 doi: 10.1097/IOP.0000000000002309. PMID: 36727764

Single-cell RNA sequencing reveals markers of disease progression in primary cutaneous T-cell lymphoma.

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: 29166501 Free PMC Article

Telomerase functions beyond telomere maintenance in primary cutaneous T-cell lymphoma.

Chevret E, Andrique L, Prochazkova-Carlotti M, Ferrer J, Cappellen D, Laharanne E, Idrissi Y, Boettiger A, Sahraoui W, Ruiz F, Pham-Ledard A, Vergier B, Belloc F, Dubus P, Beylot-Barry M, Merlio JP
Blood 2014 Mar 20;123(12):1850-9. Epub 2014 Jan 27 doi: 10.1182/blood-2013-05-500686. PMID: 24470590

Expression of follicular helper T-cell markers in primary cutaneous T-cell lymphoma.

Park JH, Han JH, Kang HY, Lee ES, Kim YC
Am J Dermatopathol 2014 Jun;36(6):465-70. doi: 10.1097/DAD.0b013e3182a72f8c. PMID: 24162385

See all (42)

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: 23986525 Free 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

See all (4)

MedGen

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Primary cutaneous T-cell lymphoma

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

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