Secondary Gamma-Delta T-Cell Lymphoma Not Otherwise Specified (NOS) From Chronic Immunosuppression

Herein, we present the case of a 63-year-old female with a history of Behçet’s disease managed with long-term prednisone and azathioprine who initially presented for symptomatic anemia, which progressed to pancytopenia with neutropenic fever. Initial workup ruled out infectious etiologies but was indeterminate for immune-mediated or neoplastic causes. Bone marrow biopsy demonstrated a CD8+ gamma-delta T-cell neoplasm; however, imaging and skin biopsy pathology did not support hepatosplenic or cutaneous lymphoma involvement. By the 2017 World Health Organization (WHO) classifications, these findings were defined as gamma-delta peripheral T-cell lymphoma, not otherwise specified (NOS). This is suspected to be secondary to chronic immunosuppression from long-term steroid and azathioprine use. The patient was treated with one cycle of the EPOCH chemotherapy regimen ((etoposide, vincristine, cyclophosphamide, doxorubicin, and prednisone), but the treatment course was complicated by an angioinvasive fungal infection and the patient subsequently transitioned to symptom-focused therapy in a hospice facility.