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

Area of Science:

• Dermatology
• Hematology
• Oncology

Background:

• CD30-positive primary cutaneous lymphoproliferative disorders (CD30+ PCLPD) are a diverse group of cutaneous T-cell lymphomas (CTCL).
• Lymphomatoid papulosis (LyP) with 6p25.3 rearrangement, defined by DUSP22-IRF4 rearrangement, accounts for less than 5% of LyP cases and typically involves alpha/beta T-cell receptors.
• DUSP22-IRF4 rearrangement is also found in 28% of anaplastic large cell lymphomas.

Observation:

• A unique case of CD30+ PCLPD was identified in a young patient.
• This case presented with the characteristic DUSP22-IRF4 rearrangement.
• Notably, the tumor cells expressed a gamma/delta T-cell immunophenotype, which is uncommon in this context.

Findings:

• The presented case is the first documented instance of CD30+ PCLPD with DUSP22-IRF4 rearrangement exhibiting a gamma/delta T-cell immunophenotype.
• This finding expands the known immunophenotypic spectrum of DUSP22-IRF4 rearranged CD30+ PCLPD.
• While gamma/delta T-cell immunophenotype is seen in other T-cell lymphomas, its association with this specific PCLPD subtype is novel.

Implications:

• This case highlights the heterogeneity within CD30+ PCLPD and the DUSP22-IRF4 rearranged subtype.
• It suggests that gamma/delta T-cell lymphomas can present as CD30+ PCLPD with this specific genetic alteration.
• Further research is warranted to understand the clinical behavior and treatment implications of gamma/delta T-cell immunophenotype in DUSP22-IRF4 rearranged CD30+ PCLPD.