Adult T-cell leukaemia/lymphoma (ATLL) is an aggressive malignancy of mature activated T cells caused by human T-cell lymphotropic virus type I (HTLV-1). Prognosis is severe because of intrinsic chemoresistance and severe immuosuppression. Four different subtypes are described with different outcomes, and treatment strategies vary according to the different clinical courses. Japanese trials show that combinations of chemotherapy can increase the response rates especially in the lymphoma subtype. However, patients have a high rate of relapse and the outcome remains extremely poor. Recently, a worldwide meta-analysis demonstrated that the combination of Zidovudine and Interferon-alpha (IFN) is effective in the leukemic subtypes (smoldering, chronic, and acute) and influences favorably the course of the disease. In order to prevent relapse, clinical trials testing new drugs such as monoclonal antibodies or combinations such as arsenic/IFN are needed. Finally, allogeneic stem cell transplantation is a feasible option but bears a very high rate of complications. 1. ATL Classification and Response Criteria The classification first described by Shimoyama (1991) used for the initial staging distinguishes four subtypes, which differ regarding their presentation and outcome. This classification has been very useful for comparison between different studies [1]. The complex presentation with both leukemic and lymphomatous components makes response assessment difficult. Recently, an international consensus meeting established new response criteria [2]. Complete response (CR) is defined as the disappearance of all measurable tumor lesions (including normalization of lymph node size) and normalization of absolute lymphocyte (including flower cells less than 5%) count below 4 × 109/L. Unconfirmed CR is defined as a reduction of 75% of the tumor size and normalization of absolute lymphocyte (including flower cells) count below 4 × 109/L. Partial response (PR) is defined as a reduction of 50% of tumor size and absolute lymphocyte count. Progressive disease is defined as an increase of 50% of the tumor size and/or absolute lymphocyte count. These response criteria require that each criterion is present for at least 4 weeks. Treatment of ATL is usually dependent on the ATL subtype. Patients with aggressive forms (acute and lymphoma) have a very poor prognosis because of intrinsic chemoresistance, a large tumor burden, hypercalcemia, and/or frequent infectious complications due to profound immune deficiency. Multiple Japanese trials in aggressive ATL clearly demonstrated
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