Primary Cutaneous Gamma/Delta T-cell Lymphoma and Hemophagocytic Lymphohistiocytosis Associated With AIDS

Abstract

Primary cutaneous gamma delta T-cell lymphoma (PCGD-TCL) is a rare lymphoma that makes up less than 1% of all cutaneous T-cell lymphomas. Patients with PCGD-TCL typically present with rapidly progressing plaques and ulceronecrotic nodules most frequently located on extremities without lymph node or bone marrow involvement. The overall prognosis is poor with a median overall survival of approximately 15 months. This case highlights a patient with concomitant PCGD-TCL, hemophagocytic lymphohistiocytosis, and human immunodeficiency virus-1-acquired immunodeficiency syndrome. There is a paucity of case reports describing PCGD-TCL and there are no evidence-based treatment recommendations. Further studies are needed to optimize strategies to treat patients with these diseases.

Keywords: aids; hlh; pctcl.

Figure 1. Progressing Plaques and Ulceronecrotic Nodules

A: Lower extremity lesions upon initial presentation B: Lower extremity lesions after first cycle of cyclophosphamide, doxorubicin, etoposide, vincristine, prednisone (CHOEP)

Figure 2. Computed Tomography (CT) Findings

A: Mildly Prominent Axillary Lymph Nodes B: 5-mm Left Upper Lobe Pulmonary Nodule

Figure 3. Histopathology of Primary Cutaneous Gamma Delta T-Cell Lymphoma (PCGD-TCL)

Low power view demonstrating a perivascular and periadnexal infiltrate (A), Medium power view demonstrating a hyperchromatic atypical cellular infiltrate around adnexal glands and dermal vasculature (B), Higher power views demonstrating a marked amount of nuclear debris along with scattered large hyperchromatic atypical cells (C, D), CD3 immunostudy highlighting the T-Cell nature of the infiltrate (E)