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.
Highlights
Human immunodeficiency virus-1 (HIV-1) increases the risk for developing various malignancies due to chronic antigenic stimulation of B-cells, cytokine dysregulation, and chronic inflammation [1,2]
To differentiate PCGD-TCL from subcutaneous panniculitis like-T cell lymphoma (SPTL), it is important to note that the malignant CD8+ T cells express T-cell receptor (TCR) alpha/beta receptors in contrast to the gamma/delta receptors found on PCGD-TCL
The loss of TIM-3 expression on mature T cells is most probably driving oncogenesis with ultimate development of T cell lymphoma and chronic auto-inflammatory status resulting in hemophagocytic lymphohistiocytosis (HLH) [6]
Summary
Human immunodeficiency virus-1 (HIV-1) increases the risk for developing various malignancies due to chronic antigenic stimulation of B-cells, cytokine dysregulation, and chronic inflammation [1,2]. How to cite this article Khadilkar A C, Adashek J J, Riddle N D, et al (September 11, 2020) Primary Cutaneous Gamma/Delta T-cell Lymphoma and Hemophagocytic Lymphohistiocytosis Associated With AIDS. A 53-year-old Haitian woman with known AIDS, CD4 count of 37, and HIV-1 (low viral load with
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