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
• lymphoproliferative disorder
• primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder
• peripheral T-cell lymphoproliferative disorder
• radiation
• cutaneous lymphoproliferative disorder

Acknowledgements

The authors thank the Dana-Farber Cancer Institute Center for Cutaneous Oncology for their support. The patients in this manuscript have given informed consent to publication of their deidentified case details. The authors acknowledge that care has been taken to not include patient-identifying information or images in this manuscript, and that data reporting was consistent with the IRB-approved protocol for deidentified reporting of patient data.

IRB approval

This study was performed in accordance with the Declaration of Helsinki and approved by the Brigham and Women's Hospital IRB Committee (ref: 2014P000098).

Disclosure statement

Thomas S. Kupper is on the Scientific Advisory Board at Adaptive Biotechnologies and receives no compensation. Nicole R. LeBoeuf is a consultant and has received honoraria from Bayer, Seattle Genetics, Sanofi, Silverback and Synox Therapeutics outside the submitted work. All other authors have no competing interests to declare.

Data availability statement

The data that support the findings of this study are available from the corresponding author, N.R.L., upon reasonable request.

Supplemental online material

This manuscript is supported by Supplemental Tables S1–S2.