DUSP22-IRF4 Rearranged CD30-Positive Primary Cutaneous Lymphoproliferative Disorder With Gamma/Delta Phenotype

Am J Dermatopathol. 2023 Dec 1;45(12):831-834. doi: 10.1097/DAD.0000000000002573.

Abstract

CD30-positive primary cutaneous lymphoproliferative disorders (CD30 + PCLPD) are a heterogeneous group of cutaneous T-cell lymphoma (CTCL) that includes lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma. They exist as a clinical and pathological spectrum, which display significant overlap and variability. The diagnosis is made based on correlation between clinical and histopathologic findings. LyP with 6p25.3 rearrangement subtype represents <5% of LyP cases and is defined by DUSP22-IRF4 rearrangement on 6p25.3 locus. The reported cases express the alpha/beta T-cell receptor and follow an indolent clinical behavior typical of LyP. The same rearrangement is detected in 28% of anaplastic large cell lymphoma. We hereby present an extraordinary case of CD30 + PCLPD with DUSP22-IRF4 rearrangement and novel expression of gamma/delta T-cell immunophenotype in a young patient. Although the gamma/delta T-cell immunophenotype has been described in many other T-cell lymphomas, this is the first reported association with CD30 + PCLPD with DUSP22-IRF4 rearrangement.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Gene Rearrangement
  • Humans
  • Intraepithelial Lymphocytes
  • Ki-1 Antigen
  • Lymphoma, T-Cell, Cutaneous* / diagnosis
  • Lymphoma, T-Cell, Cutaneous* / genetics
  • Lymphomatoid Papulosis* / diagnosis
  • Lymphomatoid Papulosis* / genetics

Substances

  • Ki-1 Antigen
  • DUSP22 protein, human
  • interferon regulatory factor-4