Clinical features and treatment outcomes for primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder: a retrospective cohort study from the Dana-Farber Cancer Institute and updated literature review
- PMID: 35862569
- DOI: 10.1080/10428194.2022.2098287
Clinical features and treatment outcomes for primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder: a retrospective cohort study from the Dana-Farber Cancer Institute and updated literature review
Abstract
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSM-TCLPD) was reclassified in 2016 as a rare benign entity with an excellent prognosis, yet its clinical features and best treatments remain poorly defined. We collected clinical data, treatments, and treatment-responses from our institution's patients with PCSM-TCLPD through September 2018 and an identical PubMed review through June 2021. Among 36 cases (median-age 54 years; 58.3% head/neck), diagnostic biopsy resulted in sustained complete remission (CR) in 13/33 punch/shave biopsies and 3/3 excisional biopsies. The remaining 20 patients further required topical corticosteroids (n = 5); intralesional corticosteroids (n = 1); surgical-excision (n = 5); electron-beam-radiation (n = 6); or brachytherapy (n = 3). All patients ultimately achieved CR, excluding one patient continuing treatment at end-of-study. 57/59 (96.6%) of institutional and literature-reported radiation-treated patients experienced CR. No institutional cases progressed beyond skin; 5/209 (2.4%) literature-reported cases progressed to systemic/extracutaneous involvement, all pre-reclassification. PCSM-TCLPD responds well to local-directed therapy including radiation, and only rarely if ever progresses.
Keywords: PCSM-TCLPD; cutaneous lymphoproliferative disorder; lymphoproliferative disorder; peripheral T-cell lymphoproliferative disorder; primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder; radiation.
Similar articles
-
Radiotherapy in the treatment of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder.Int J Dermatol. 2025 Feb;64(2):385-389. doi: 10.1111/ijd.17352. Epub 2024 Jul 2. Int J Dermatol. 2025. PMID: 38955474
-
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder in children: A case report and review of the literature.Pediatr Blood Cancer. 2022 Sep;69(9):e29862. doi: 10.1002/pbc.29862. Epub 2022 Jul 11. Pediatr Blood Cancer. 2022. PMID: 35730956 Review.
-
The Clinical Spectrum of Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder: An Updated Systematic Literature Review and Case Series.Dermatology. 2021;237(4):618-628. doi: 10.1159/000511473. Epub 2020 Dec 16. Dermatology. 2021. PMID: 33326960
-
Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoproliferative disorder: Report of a case and review of the literature.J Cutan Pathol. 2017 Nov;44(11):944-947. doi: 10.1111/cup.13011. Epub 2017 Aug 30. J Cutan Pathol. 2017. PMID: 28749588 Review.
-
Primary cutaneous small- to medium-sized CD4+ pleomorphic T-cell lymphoma: a retrospective case series and review of the provisional cutaneous lymphoma category.Am J Clin Dermatol. 2011 Dec 1;12(6):389-401. doi: 10.2165/11590390-000000000-00000. Am J Clin Dermatol. 2011. PMID: 21863906 Review.
Cited by
-
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder nodule successfully treated with laser Co2 ablation: a case report and literature review.Dermatol Reports. 2024 Feb 6;16(4):9884. doi: 10.4081/dr.2024.9884. eCollection 2024 Nov 21. Dermatol Reports. 2024. PMID: 39669877 Free PMC article.
-
Radiotherapy in the treatment of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder.Int J Dermatol. 2025 Feb;64(2):385-389. doi: 10.1111/ijd.17352. Epub 2024 Jul 2. Int J Dermatol. 2025. PMID: 38955474
-
Clinical features, treatment options and outcomes in primary cutaneous B-cell lymphomas: a real-world, multicenter, retrospective study.Int J Dermatol. 2025 May;64(5):882-889. doi: 10.1111/ijd.17564. Epub 2024 Nov 11. Int J Dermatol. 2025. PMID: 39526550 Free PMC article.
-
Primary Cutaneous CD4 Small/Medium T-Cell Lymphoproliferative Disorder Following COVID-19 Vaccination-What Do We Know about Lymphoproliferative Disorders and Cutaneous Lymphomas after COVID-19 Vaccination? A Report of an Atypical Case and a Review of the Literature.Life (Basel). 2024 Mar 14;14(3):386. doi: 10.3390/life14030386. Life (Basel). 2024. PMID: 38541710 Free PMC article.
Publication types
MeSH terms
Grants and funding
LinkOut - more resources
Full Text Sources
Medical
Research Materials
Miscellaneous