Adult T-cell leukemia on the east coast of Kii Peninsula--presentation of an anti-ATLA-negative case.

Nineteen patients with adult T-cell leukemia (ATL) have been found in the last seven years along the east coast of Kii Peninsula in Japan. The leukemic cells were of the immunologically inducer/helper T-cell phenotype. The prognosis was very poor (median survival time, 85 days), and most of the patients had fatal complications of pulmonary infections. Antibody against ATL-associated antigen (anti-ATLA) was detected in sera from 9 of 10 patients who were born along the coast. However, it was not detected in one patient who was born in a district surrounded by mountains. Although he had neither superficial lymphadenopathy nor skin lesions, he showed rapid clinical deterioration. His leukemic cells appeared to be extremely bizarre with marked nuclear deformation compared with those of the other patients. In surface marker studies the leukemic cells reacted positively with OKT3, OKT4 and OKIa-1 monoclonal antibodies. The characteristics of the anti-ATLA-negative case are discussed in comparison with the other ATL cases.