Adult T cell leukemia: a case with massive hyperimmunoglobulinemia E

Adult T cell leukemia, an endemic disease in the southwestern part of Japan, is characterized (1) by a short survival, (2) by leukemic T cells in peripheral blood that have lobulated nuclei and helper/inducer surface phenotypes, and (3) by cutaneous involvement. A 34-year-old man who had a history of atopic dermatitis was seen at our clinic because of generalized erythroderma and lymphadenopathy. His clinical course was rather chronic as compared with that of prototypic adult T cell leukemia; however, typical leukemia cells were observed in specimens taken from his peripheral blood and skin. The diagnosis of adult T cell leukemia was established by the patient's positive serum antibody reaction to adult T cell leukemia-associated antigen and monoclonal integration of virus genome in the patient's leukemia cell DNA. Interesting and characteristic of the patient were the very high levels of serum immunoglobulin E. With the use of an in vitro immunoglobulin production system with mitogen, the patient's T lymphocytes enhanced the differentiation of B cells, both from the patient and from a normal adult, into immunoglobulin E-producing cells. Therefore it may be speculated that T cells functioning as immunoglobulin E-specific helpers were transformed to leukemia cells by human T-lymphotropic virus type I. Continuous antigen stimulation of the patient's atopic diathesis also may be a factor.