Adult T-cell leukemia/lymphoma: “A fatal presentation of a challenging disease”

Abstract

Adult T-cell leukemia/lymphoma (ATL) is a rare aggressive peripheral T cell neoplasm caused by a retrovirus human T-lymphotropic virus, type 1 (HTLV-I). Although commonly described in HTLV-I endemic regions, it has rarely been described in non-endemic regions, like the Indian subcontinent. We present the case of a 45-year-old south indian male who presented with generalized lymphadenopathy and diffuse erythematous scaly lesions which had progressed over 2 months. Skin biopsy revealed dermal lymphoid infiltrates with epidermotropism and epidermal destruction. Supportive immunohistochemistry and serological evidence of antibodies to HTLV-I confirmed the diagnosis of ATL. Our patient was initiated on chemotherapy, but eventually succumbed to fatal pulmonary hemorrhage. Aggressive ATL presents a therapeutic challenge due to intrinsic chemo-resistance, large tumor burden and severe immunocompromise.

Keywords

• Adult T-cell leukemia/lymphoma
• HTLV-I
• rash