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.

Autor: Plumptre IR; Imperial College Healthcare NHS Trust, London, UK., Said JT; Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.; Harvard Medical School, Boston, MA, USA., Sun T; Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.; Center for Cutaneous Oncology, Dana-Farber Cancer Institute, Boston, MA, USA., Larocca C; Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.; Harvard Medical School, Boston, MA, USA.; Center for Cutaneous Oncology, Dana-Farber Cancer Institute, Boston, MA, USA., Virgen CA; Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.; Harvard Medical School, Boston, MA, USA.; Center for Cutaneous Oncology, Dana-Farber Cancer Institute, Boston, MA, USA., Kupper TS; Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.; Harvard Medical School, Boston, MA, USA.; Center for Cutaneous Oncology, Dana-Farber Cancer Institute, Boston, MA, USA., Fisher DC; Harvard Medical School, Boston, MA, USA.; Center for Cutaneous Oncology, Dana-Farber Cancer Institute, Boston, MA, USA., Devlin PM; Harvard Medical School, Boston, MA, USA.; Center for Cutaneous Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.; Department of Radiation Oncology, Brigham and Women's Hospital, Boston, MA, USA., Elco CP; Harvard Medical School, Boston, MA, USA.; Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA., Song JS; Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.; Center for Cutaneous Oncology, Dana-Farber Cancer Institute, Boston, MA, USA., LeBoeuf NR; Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.; Harvard Medical School, Boston, MA, USA.; Center for Cutaneous Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.
Jazyk: angličtina
Zdroj: Leukemia & lymphoma [Leuk Lymphoma] 2022 Dec; Vol. 63 (12), pp. 2832-2846. Date of Electronic Publication: 2022 Jul 21.
DOI: 10.1080/10428194.2022.2098287
Abstrakt: 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.
Databáze: MEDLINE
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