経過中臓器浸潤が興味ある変遷を示し，著明な低ガンマグロブリン血症を伴ったATLの1剖検例

抄録

A 43-year-old male born in kagoshima prefecture was admitted to Kyoto University Hospital because of general malaise and cough in June, 1982. Hematological examinaton revealed leucocytosis (26,700/ul) with abnormal lymphoid cells (26.5%), which were compatible with ATL cells in morphological charcteristics. Serum ATLA test gave a titer of 1:20. The ATL cells had an E⁺ OKT3^-4⁺8^- surface phenotype and possessed potent suppressor activity on PWM-induced normal B-cell differentiation. These immunological chracteristics showed some alteration on other occasions during the course. Serum immunoglobulin concentration was markedly reduced since initial visit and fluctuated with an inverse correlation with the number of circulating ATL cells. Cytogenetic studies of the ATL cells revealed 46, XY, t (9; 14), (p12; q13), 6q^- with other many additional abnormalities.
The patient was treated with combined chemotherapy regimen including cyclophosphamide, vincristin, adriamycin and prednisolone intermittently, with partial reduction of ATL cells. During the course, pulmonary involvement devoloped and almost paralleled the number of circulating ATL cells. In contrast, marked intestinal involvement was found by endoscopical examination when the circulating ATL cell count was quite low and pulmonary involvement was improved without any specific treatment, and disseminated intravascular coagulating was associated causing massive intestinal bleeding. Finally, meningeal involvement was noticed and the patient died of disseminated herpes zoster in March, 1984.
These clinical and laboratory findings suggest that the ATL cells of this patient consisted of a few subclones which are heterogenous in organ affinity, surface phenotype and functional activity.