CD30+ primary cutaneous tumoral adult T-cell leukemia/lymphoma successfully treated with zidovudine and interferon

Abstract

Adult T-cell leukemia/lymphoma (ATL) is an aggressive neoplasia of mature peripheral T lymphocytes associated with human T-cell lymphotropic virus type 1 (HTLV-1). The primary cutaneous tumoral (PCT) type of adult T-cell leukemia/lymphoma presents as nodules and tumors without internal organ involvement, but with a bad prognosis. Here, we report a middle aged woman, who manifested a nodular lesion on the right leg. Histopathology showed moderately dense, lymphoid infiltrate of atypical, medium sized cells in the dermis and subcutaneous tissue. The cells were positive for CD3, CD4, CD30, and CD25, and negative for CD8, CD20, and anaplastic lymphoma kinase. The biopsy specimen showed a Ki-67 proliferation index of 90%. Serological testing for HTLV-1 was positive. The patient was diagnosed to have CD30+ PCT type ATL and was treated initially with localized radiation. The lesion regressed, however, there was a recurrence of the disease, which was treated with low dose interferon-alfa (IFN-α) and zidovudine, following which patient attained complete resolution and remained symptom free during the follow up period of 2 years. Our case highlights the usefulness of low dose IFN-α and zidovudine in the management of PCT type of CD30+ ATL.