[T-cell leukemia-lymphoma in adults and other blood diseases in HTLV-I infection]

Several clinical aspects are recognized in adult T cell leukemia in ATL which was initially described in 1977 : a) smoldering, b) chronic, c) lymphomatous and d) acute. Median survival time is different according to these types: not yet reached in smoldering type, 24 months in chronic type, 10 months in lymphomatous type, 6 months in acute type. The classical phenotype of this mature T-cell proliferation is CD2+, CD3+, CD5+, CD7-, CD4+, CD8-. Activation markers are expressed: CD25+, CD30 +/-, MHC class II. HTLV-1 proviral DNA is integrated in the host genome. A region of the viral genome codes for a protein tax which transactivates viral and cellular genes, especially cytokines genes. There are no specific cytogenetic abnormalities. The genetic lesions mainly involved P53 gene where mutations are detected in 25 to 30% of cases. Conventional chemotherapy is unsuccessful in acute type. New therapeutic strategies are under investigation, especially monoclonal antibodies against RIL2 and interferon alpha associated with antiretroviral (AZT) therapy.