Human T-lymphotropic virus type I (HTLV-I) is etiologically linked with adult T cell leukemia lymphoma (ATLL) and HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP). The diagnostic criteria and classification of clinical subtypes of ATLL were proposed by Shimoyama in 1991. They are referred to as acute, chronic, lymphoma, and smouldering types of ATLL. The final diagnosis of ATLL is made by the demonstration of HTLV-I proviral DNA in peripheral blood, lymph nodes, and skin. ATLL commonly involves the skin in up to 50% of patients. The skin manifestations of ATLL are markedly varied. Dermatologists should investigate whether abnormal lymphoid cells are involved in the skin lesion with biopsies. The smouldering type of ATLL with skin manifestations does not always have a better prognosis than the smouldering one without skin involvement. The standard chemotherapy for acute and lymphoma types of ATLL has not been established and the prognosis for each type is very poor. However, fair results using allogenic-hematopoietic stem cell transplantation (allo-HSCT) to treat the acute type of ATLL have been recently reported by some authors. It is likely that allo-HSCT can improve the survival in ATLL patients in the future.
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