Cutaneous and Systemic Complications in Primary CD8+ Aggressive Epidermotropic Cytotoxic T-cell Lymphoma

CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (CD8+ AECTCL) is an uncommon and highly aggressive variant of cutaneous T-cell lymphoma characterized by rapid systemic progression. We present the case of a 61-year-old man with CD8+ AECTCL who developed a methicillin-sensitive Staphylococcus aureus port-site infection while on duvelisib following prior gemcitabine/liposomal doxorubicin therapy. The patient had been diagnosed with CD8+ AECTCL 18 months prior and presented with acute erythema, swelling, and pain over his left anterior chest. Upon admission, the patient met sepsis criteria, and his port was subsequently removed in addition to the initiation of appropriate antibiotic therapy. During hospitalization, he developed nephrotic-range proteinuria and acute kidney injury requiring dialysis. Renal biopsy revealed diabetic nephropathy with findings consistent with renal-limited thrombotic microangiopathy (TMA), likely multifactorial from gemcitabine and duvelisib exposure compounded by sepsis and underlying lymphoma. Complement studies supported the activation of the alternative pathway, and after multidisciplinary discussion, eculizumab was initiated for TMA management. The patient completed inpatient eculizumab dosing and was discharged on outpatient infusions. His cutaneous lymphoma lesions improved with a short course of oral prednisone as directed by oncology. At discharge, he was clinically stable on dialysis with plans for ongoing outpatient care; subsequent follow-up showed stable renal function. This case highlights the intersection between aggressive cutaneous lymphoma, infection, and chemotherapy-induced vascular injury, emphasizing the importance of early recognition of systemic complications in CD8+ AECTCL and the need for a multidisciplinary approach to optimize patient outcomes.