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

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.

Keywords: adipocyte rimming; brentuximab vedotin; hemophagocytic lymphohistiocytosis; panniculitis; primary cutaneous T-cell lymphoma.

Figure 1

Large violaceous contiguous patch spreading over the anterior and right side of the abdominal wall.

Figure 2

Skin biopsy findings. Low magnification ((a), 20x) image showing a lobular, pannicular infiltrate of large lymphoid cells with pleomorphic nuclei and irregular nuclear contours that focally rim adipocytes ((b), 400x). Focal hemophagocytic lymphohistiocytosis is also noted. Tumor cells show immunostaining for CD3 ((c), 100x), CD8, with rimming of adipocytes ((d), 100x), and TCR-delta ((e), 100x). The BF-1 shows staining of small lymphocytes ((f), 100x).

Figure 3

Bone marrow biopsy findings. Bone marrow trephine biopsy at 20X magnification stained with hematoxylin and eosin (a), CD3 (b), CD20 (c), and γδ TCR (d), demonstrating rimming of fat by γδ T cells. Insets in (a), (b), and (d) at 100X magnification. Bone marrow aspirate at 100X magnification demonstrating extensive hemophagocytosis (e-f).

Figure 4

Flow cytometric analysis of CD3+ T cells from the bone marrow aspirate specimen. Green, CD8+ T cells. Red, CD4+ T cells. Blue, CD4-CD8- γδ T cells. The γδ T cells express bright CD3, normal levels of CD2, CD5, and CD7, partial CD56, partial CD57, and partial CD16. No definitive phenotypic abnormalities were detected by flow cytometry.