ABSTRACT
General description 1384 Historical perspective 1384 HTLV-1 and its role in leukemogenesis 1384 Epidemiology 1385 Subtypes 1386 Diagnostic evaluation 1386 Clinical features 1387 Clinical features commonly observed in adult 1387
T cell leukemia Laboratory features commonly observed in cases 1388
of adult T cell leukemia
Clinical and laboratory findings restricted to specific subtypes 1388 Management 1389 Therapeutic principles for indolent disease 1389 Therapeutic approaches for patients critically debilitated 1389
at presentation Therapeutic principles for aggressive disease 1390 Follow-up 1395 Indolent types (smoldering and chronic ATL) 1395 Aggressive types (lymphoma and acute ATL) 1395 Key points 1396 References 1396
The development of immunologic techniques to classify lymphocytes into T cells and B cells revealed that the frequency of T cell leukemia was considerably higher in Japan than in Western countries. As many Japanese with T cell leukemia shared characteristic clinical features which were previously undescribed and, surprisingly, the birthplace of these patients clustered in southwestern Japan, it was proposed that adult T cell leukemia (ATL) is a distinct clinical entity.1 The patients described in the original report had acute or chronic leukemia with rapidly progressive terminal phases. Subsequently, sera from these patients were shown to contain antibodies against ATL-associated antigens which were found in a cell line derived from a patient with ATL.2 The introduction of an assay which easily detected the presence of antibodies against ATL-associated antigens expanded the disease spectrum of ATL. Sera from a significant proportion of Japanese patients with T cell lymphoma tested positive. In addition, histologic features of lymph nodes derived from patients with ATL and most of the seropositive T cell lymphomas were shown to be identical.3 The clinical course was frequently characterized
from classical ATL could not be made. The term lymphomatype ATL was given.
