| Research Abstract |
This study was performed to identify and establish a type of adult T-cell leukemia/lymphoma (ATLL), named as cutaneous type of ATLL (cATLL). Fifty eight cases satisfied our criteria of the cATLL, which is characterized by involvement of the skin alone for a long time period of the disease process and by the detection of monoclonal integration for HTLV-1 provirus in the skin lesion. This type could be histologically, immunohistochemically subdivided into the tumoral and erythemato-papular types by depth and size of a main tumor cell mass within the skin. The former showed various number of tumors or of eruptive nodules, composed of tumor cell infiltration and proliferation through almost layers of the skin, and the latter did disseminated plaques, indistinguishable clinically from early stage of mycosis fungoides, localized papules or generalized papules, major population of cases of which were characterized by tumor cell infiltration limited to epidermis and papillary dermis, to the appendages and periappendage, and to upper dermis around the vessels, respectively.
The prognosis of patients with cATLL were made a survey among 6 types ; acute, chronic, lymphomatous, cutaneous tumoral, cutaneous erythemato- papular and smoldering type, and confirmed that it differs from type to type. The tumoral type was better than the acute type, but almost equal to the chronic or lymphomatous type, and worse than the erythemato-papular or smoldering type, whereas the erythemato-papular type was worse than the smoldering one. These data suggest two cutaneous types of ATLL might be a distinct clinical entity on diagnosis and therapeutics. Whereas, Langerhans cells and their cytokines might play an important role in the formation of cATLL skin lesions. Following the establishment of the diagnosis and therapy for cATLL, we have found more than 10 cases of non-ATLL malignant lymphoma positeve for HTLV-1 antibody, prognosis of which were similar to one of the diagnosed disease.
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