# Primary Cutaneous Gamma–Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male

## TL;DR

A rare skin lymphoma in a young Polynesian man is reported, emphasizing diagnostic challenges and poor prognosis.

## Contribution

This case highlights unique demographic and clinical features of PCGD-TCL with HLH in a young patient.

## Key findings

- PCGD-TCL is rare and often mimics other conditions, requiring high clinical suspicion for diagnosis.
- HLH is associated with more aggressive disease and poor prognosis, with 5-year survival under 20%.
- Treatment typically involves anthracycline-based chemotherapy and stem cell transplants, with limited guidelines.

## Abstract

Primary cutaneous gamma–delta T-cell lymphoma (PCGD-TCL) is a very rare subtype of cutaneous T-cell lymphoma. We report the case of a young Polynesian male who presented with fever and an abdominal wall rash and highlight the workup leading to the diagnosis of PCGD-TCL. As PCGD-TCL is rare and mimics other medical conditions, its diagnosis requires a high index of suspicion and can be challenging. Hemophagocytic lymphohistiocytosis (HLH) occurs with PCGD-TCL and can be a marker of more invasive disease. There are no well-defined treatment guidelines, but the most common treatment approach is anthracycline-based multiagent chemotherapy followed by allogeneic stem cell transplant. Targeted therapies are being increasingly used as well. Prognosis remains poor and 5‐year survival is < 20%, particularly in more invasive disease. We highlight how this patient's demographic varies from the published literature and discuss some unique particulars of the diagnostic evaluation and treatment, especially in the presence of concurrent HLH.