Abstract
Adult T-cell leukemia/lymphoma (ATL) is an aggressive malignancy of mature activated T cells caused by human T-cell lymphotropic virus type I. ATL carries a bad prognosis because of intrinsic chemoresistance and severe immunosuppression. In acute ATL, Japanese trials demonstrated that although combinations of chemotherapy improved response rate, they failed to achieve a significant impact on survival. Patients with chronic and smoldering ATL have a better prognosis, but long-term survival is poor when these patients are managed with a watchful-waiting policy or with chemotherapy. Recently, a worldwide meta-analysis revealed that the combination of zidovudine and IFN-alpha is highly effective in the leukemic subtypes of ATL and should be considered as standard first-line therapy in that setting. This combination has changed the natural history of the disease through achievement of significantly improved long-term survival in patients with smoldering and chronic ATL as well as a subset of patients with acute ATL. ATL lymphoma patients still benefit from chemotherapy induction with concurrent or sequential antiretroviral therapy with zidovudine/IFN. To prevent relapse, clinical trials assessing consolidative targeted therapies such as arsenic/IFN combination or novel monoclonal antibodies are needed. Finally, allogeneic BM transplantation should be considered in suitable patients.
| Original language | English |
|---|---|
| Pages (from-to) | 1736-1745 |
| Number of pages | 10 |
| Journal | Blood |
| Volume | 118 |
| Issue number | 7 |
| DOIs | |
| Publication status | Published - 18 Aug 2011 |
Keywords
- VIRUS TYPE-I
- KAPPA-B ACTIVATION
- LEUKEMIA-LYMPHOMA
- HTLV-I
- INTERFERON-ALPHA
- MONOCLONAL-ANTIBODY
- ARSENIC TRIOXIDE
- TRANSFORMED-CELLS
- COMBINATION CHEMOTHERAPY
- MOLECULAR-MECHANISMS
