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Primary Cutaneous Acral CD8+ T-Cell Lymphoma—A Single Center Review of 3 Cases and Recent Literature Review

Tjahjono, Leonardo A. BS*; Davis, Mark D. P. MD; Witzig, Thomas E. MD; Comfere, Nneka I. MD†,§

The American Journal of Dermatopathology: September 2019 - Volume 41 - Issue 9 - p 644–648
doi: 10.1097/DAD.0000000000001366
Original Study

Abstract: The recently published 2016 revision of the WHO classification of lymphoid neoplasms includes primary cutaneous acral CD8-positive T-cell lymphoma (PCATCL) as a provisional entity. This is a rare indolent lymphoma characterized by papules or nodules on the ear and a dermal infiltrate of CD8-positive T-lymphocytes with cytotoxic marker expression. A retrospective review of a single institutional experience with PCATCL identified 3 patients (mean age 54; range 49–62) with papules or nodules on the ear. Lesional biopsies demonstrated a dense diffuse dermal infiltrate of atypical lymphocytes with a Grenz zone in 2 cases and focal epidermotropism in 1 case. The atypical lymphocytes were predominantly CD3 and CD8 positive with expression of cytotoxic marker TIA1. Staging evaluation failed to reveal systemic disease. Two patients underwent local excision, and the third received local radiation therapy all with complete response and no disease recurrence at last follow-up 3 months (range 2–5 months). Our cases add to the existing limited literature on the clinical and histopathological features of PCATCL. We also performed an updated systematic literature view of the entity.

*Mayo Clinic School of Medicine, Rochester, MN;

Department of Dermatology, Mayo Clinic, Rochester, MN;

Department of Hematology and Oncology, Mayo Clinic, Rochester, MN; and

§Department of Laboratory Medicine and Pathology, Rochester, MN.

Correspondence: Nneka I. Comfere, MD, 200 First Street SW, Rochester, MN 55905 (e-mail:

The authors declare no conflicts of interest.

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