Abstract:

A 56-year-old female with a prior history of mycosis fungoides and a CD30+ lymphoproliferative disorder clinically consistent with lymphomatoid papulosis (LyP) presented six years after being lost to follow up with multiple nodules and ulcerations on the bilateral legs and abdomen. A biopsy of an ulcer showed an atypical pleomorphic lymphoid infiltrate with scattered large cells involving the epidermis, dermis and subcutis with angiocentricity and angiodestruction. Immunohistochemical studies showed diffuse expression of CD2, CD3, CD7, CD56, granzyme B and TCR-?, and negative expression of CD4, CD5, CD8, TCR-beta and TIA1. CD30 was positive in > 50% of cells. The biopsy was interpreted as primary cutaneous gamma-delta T-cell lymphoma (PCGDTCL) and the patient began treatment with the anti-CD30 agent brentuximab vedotin and romidepsin. The patient was re-biopsied after a recurrence of ulcerations following initial treatment, with similar biopsy findings to the prior. Additionally, the patient endorsed spontaneous resolution of some her clinical lesions within 4-8 weeks. Additional molecular testing detected the same monoclonal TCR-? rearrangement peaks in multiple biopsies, and OncoScan chromosomal microarray showed a complete lack of significant copy number variation alterations. Given the self-remitting course of the disease and relative molecular stability, a diagnosis of LyP was rendered. Very rarely, LyP can have a ?? TCR phenotype. The patient has been treated with methotrexate for one year with excellent control. In conclusion, we present a rare case of ??-LyP that demonstrates an important pitfall when considering a diagnosis of PCGDTCL.

Financial Disclosure:
No current or relevant financial relationships exist.

Menu

Lisa Borretta, MD