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Primary Cutaneous CD4-Positive Small/Medium T-Cell Lymphoproliferative Disorder

MedGen UID:
1627444
Concept ID:
C4528627
Neoplastic Process
Synonyms: PCSM-TCL- primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder; Primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder; Primary cutaneous CD4-positive small/medium T-cell lymphoproliferative disorder; Primary Cutaneous CD4-Positive Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder; Primary Cutaneous CD4-Positive Small/Medium-Sized T-Cell Lymphoproliferative Disorder
SNOMED CT: Primary cutaneous CD4-positive small/medium T-cell lymphoproliferative disorder (788570002); PCSM-TCL- primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder (788674000); Primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder (788674000)

Definition

A primary cutaneous T-cell lymphoproliferative disorder. It usually presents with a solitary plaque or tumor on the face, neck, or upper trunk. Morphologically, it is composed of small to medium-sized CD4-positive, CD8-negative, and CD30-negative pleomorphic T-lymphocytes. A small number of large pleomorphic T-lymphocytes may also be present. The lymphocytic infiltrate is dermal. Focal epidermotropism and subcutaneous involvement may be present. The clinical behavior is almost always indolent and most patients present with localized disease. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary Cutaneous CD4-Positive Small/Medium T-Cell Lymphoproliferative Disorder

Professional guidelines

PubMed

Plumptre IR, Said JT, Sun T, Larocca C, Virgen CA, Kupper TS, Fisher DC, Devlin PM, Elco CP, Song JS, LeBoeuf NR
Leuk Lymphoma 2022 Dec;63(12):2832-2846. Epub 2022 Jul 21 doi: 10.1080/10428194.2022.2098287. PMID: 35862569
Besch-Stokes JG, Costello CM, Severson KJ, Bhullar P, Montoya J, Butterfield RJ, DiCaudo DJ, Comfere N, Sluzevich J, Rule W, Craig FE, Rosenthal A, Pittelkow MR, Mangold AR
J Am Acad Dermatol 2022 May;86(5):1167-1169. Epub 2021 Apr 26 doi: 10.1016/j.jaad.2021.04.067. PMID: 33915243
Gru AA, Wick MR, Eid M
Semin Cutan Med Surg 2018 Mar;37(1):39-48. doi: 10.12788/j.sder.2018.006. PMID: 29719019

Recent clinical studies

Etiology

Plumptre IR, Said JT, Sun T, Larocca C, Virgen CA, Kupper TS, Fisher DC, Devlin PM, Elco CP, Song JS, LeBoeuf NR
Leuk Lymphoma 2022 Dec;63(12):2832-2846. Epub 2022 Jul 21 doi: 10.1080/10428194.2022.2098287. PMID: 35862569
Ganapathi KA, Brown LE, Prakash S, Bhargava P
Pathology 2021 Apr;53(3):349-366. Epub 2021 Mar 5 doi: 10.1016/j.pathol.2021.01.002. PMID: 33685720
Shi HZ, Zhang J, Xiong JS, Gan L, Jiang YQ, Xu XL, Zhang W, Zeng XS, Sun JF, Chen H
Int J Dermatol 2021 Apr;60(4):497-502. Epub 2020 Dec 28 doi: 10.1111/ijd.15372. PMID: 33369742
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
Maurelli M, Colato C, Gisondi P, Girolomoni G
J Cutan Med Surg 2017 Nov/Dec;21(6):502-506. Epub 2017 Jun 13 doi: 10.1177/1203475417715209. PMID: 28605998

Diagnosis

Besch-Stokes JG, Costello CM, Severson KJ, Bhullar P, Montoya J, Butterfield RJ, DiCaudo DJ, Comfere N, Sluzevich J, Rule W, Craig FE, Rosenthal A, Pittelkow MR, Mangold AR
J Am Acad Dermatol 2022 May;86(5):1167-1169. Epub 2021 Apr 26 doi: 10.1016/j.jaad.2021.04.067. PMID: 33915243
Ganapathi KA, Brown LE, Prakash S, Bhargava P
Pathology 2021 Apr;53(3):349-366. Epub 2021 Mar 5 doi: 10.1016/j.pathol.2021.01.002. PMID: 33685720
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
Gru AA, Wick MR, Eid M
Semin Cutan Med Surg 2018 Mar;37(1):39-48. doi: 10.12788/j.sder.2018.006. PMID: 29719019
Maurelli M, Colato C, Gisondi P, Girolomoni G
J Cutan Med Surg 2017 Nov/Dec;21(6):502-506. Epub 2017 Jun 13 doi: 10.1177/1203475417715209. PMID: 28605998

Therapy

Davick JJ, Gaughan E, Barry M, Gru AA
Am J Dermatopathol 2018 Jan;40(1):60-63. doi: 10.1097/DAD.0000000000000960. PMID: 28719434
Alberti-Violetti S, Torres-Cabala CA, Talpur R, Corti L, Fanoni D, Venegoni L, Berti E, Duvic M
J Cutan Pathol 2016 Dec;43(12):1121-1130. Epub 2016 Sep 20 doi: 10.1111/cup.12806. PMID: 27550169
Ma H, Qiu S, Lu R, Feng P, Lu C
An Bras Dermatol 2016 May-Jun;91(3):368-71. doi: 10.1590/abd1806-4841.20163875. PMID: 27438209Free PMC Article
Garrido MC, Riveiro-Falkenbach E, Ruano Y, Ortiz P, Rodriguez-Peralto JL
Am J Dermatopathol 2015 Jun;37(6):440-3. doi: 10.1097/DAD.0000000000000231. PMID: 25357018
James E, Sokhn JG, Gibson JF, Carlson K, Subtil A, Girardi M, Wilson LD, Foss F
Leuk Lymphoma 2015 Apr;56(4):951-7. doi: 10.3109/10428194.2014.938331. PMID: 24996443

Prognosis

Plumptre IR, Said JT, Sun T, Larocca C, Virgen CA, Kupper TS, Fisher DC, Devlin PM, Elco CP, Song JS, LeBoeuf NR
Leuk Lymphoma 2022 Dec;63(12):2832-2846. Epub 2022 Jul 21 doi: 10.1080/10428194.2022.2098287. PMID: 35862569
Shi HZ, Zhang J, Xiong JS, Gan L, Jiang YQ, Xu XL, Zhang W, Zeng XS, Sun JF, Chen H
Int J Dermatol 2021 Apr;60(4):497-502. Epub 2020 Dec 28 doi: 10.1111/ijd.15372. PMID: 33369742
Salah E
J Dtsch Dermatol Ges 2019 Feb;17(2):123-136. Epub 2018 Dec 3 doi: 10.1111/ddg.13691. PMID: 30506921
Gru AA, Wick MR, Eid M
Semin Cutan Med Surg 2018 Mar;37(1):39-48. doi: 10.12788/j.sder.2018.006. PMID: 29719019
Maurelli M, Colato C, Gisondi P, Girolomoni G
J Cutan Med Surg 2017 Nov/Dec;21(6):502-506. Epub 2017 Jun 13 doi: 10.1177/1203475417715209. PMID: 28605998

Clinical prediction guides

Hu J, Zhang X, Zhao L, Zhao Q, Geng S
An Bras Dermatol 2024 Jan-Feb;99(1):27-33. Epub 2023 Aug 30 doi: 10.1016/j.abd.2023.01.003. PMID: 37657958Free PMC Article
Ward J, Prince HM, McCormack C, Lade S, Buelens O, van der Weyden C, Bhabha F, Campbell BA
Radiother Oncol 2023 Jan;178:109430. Epub 2022 Nov 28 doi: 10.1016/j.radonc.2022.11.019. PMID: 36455687
Bakr F, Wain EM, Wong S, Palmer R, Robson A
Am J Dermatopathol 2021 Dec 1;43(12):e190-e196. doi: 10.1097/DAD.0000000000001979. PMID: 33989212
Shi HZ, Zhang J, Xiong JS, Gan L, Jiang YQ, Xu XL, Zhang W, Zeng XS, Sun JF, Chen H
Int J Dermatol 2021 Apr;60(4):497-502. Epub 2020 Dec 28 doi: 10.1111/ijd.15372. PMID: 33369742
Magro CM, Momtahen S
Am J Dermatopathol 2017 Feb;39(2):95-103. doi: 10.1097/DAD.0000000000000597. PMID: 27097342

Recent systematic reviews

Surmanowicz P, Doherty S, Sivanand A, Parvinnejad N, Deschenes J, Schneider M, Hardin J, Gniadecki R
Dermatology 2021;237(4):618-628. Epub 2020 Dec 16 doi: 10.1159/000511473. PMID: 33326960
Salah E
J Dtsch Dermatol Ges 2019 Feb;17(2):123-136. Epub 2018 Dec 3 doi: 10.1111/ddg.13691. PMID: 30506921

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