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

Author:

Fattah Yasmin H.1ORCID,Crasto David1,Liu Shuo S.1,Linhares Yuliya2,Kerdel Franz3,Hanly Andrew4,Karai Laszlo J.4

Affiliation:

1. Department of Dermatology, Larkin Community Hospital, South Miami, FL;

2. Department of Oncology, Baptist Hospital, Miami, FL;

3. Skin Center of South Miami, South Miami, FL; and

4. Global Pathology, Sonic Health Care USA, Miami Lakes, FL.

Abstract

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.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Dermatology,General Medicine,Pathology and Forensic Medicine

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