Adult T-cell lymphoma leukemia in Western countries

A new T-cell disorder has recently emerged: the so-called adult T-cell lymphoma leukemia (ATLL) initially described in Japan. Subsequently, ATLL cases were recognized in patients from the Caribbean. We summarize the clinical and hematological features of 19 published cases from Western countries, in addition to a new case we encountered. The leukemic cells display characteristic morphological features and a T3+T4+T8-T6- surface antigenic phenotype. Overall survival is of short duration, but remission could be obtained in our case despite a subsequent relapse in skin and CNS. Geographic clusters of ATLL cases have led to the discovery of the possible role of a new retrovirus, HTLV, in the genesis of this rare malignancy.