Adult T-cell leukemia/lymphoma (ATLL) is a malignant proliferation of mature helper T lymphocytes, and is caused by human T-lymphotropic virus type I (HTLV-I); an HTLV-I infection endemic in the Caribbean, south-weste...Adult T-cell leukemia/lymphoma (ATLL) is a malignant proliferation of mature helper T lymphocytes, and is caused by human T-lymphotropic virus type I (HTLV-I); an HTLV-I infection endemic in the Caribbean, south-western Japan, South America and Africa. Seroepidemiological studies suggest that it is also endemic in Brazil. Although carriers of HTLV-I show polyclonal integration of virus in T lymphocytes, only patients with ATLL of various subtypes show monoclonal integration of HTLV-I in tumor cells. Cutaneous T-cell lymphomas (CTCL) are a group of primary cutaneous lymphoproliferative diseases unknown etiology. The two most common presentations of CTCL are mycosis fungoides (MF) and Sézary syndrome (SS). However, both CTCL categories can easily resemble ATLL. Therefore, in HTLV-I endemic areas, differentiation between ATLL and CTCL must be performed, as they have different prognoses and treatment approaches.展开更多
文摘Adult T-cell leukemia/lymphoma (ATLL) is a malignant proliferation of mature helper T lymphocytes, and is caused by human T-lymphotropic virus type I (HTLV-I); an HTLV-I infection endemic in the Caribbean, south-western Japan, South America and Africa. Seroepidemiological studies suggest that it is also endemic in Brazil. Although carriers of HTLV-I show polyclonal integration of virus in T lymphocytes, only patients with ATLL of various subtypes show monoclonal integration of HTLV-I in tumor cells. Cutaneous T-cell lymphomas (CTCL) are a group of primary cutaneous lymphoproliferative diseases unknown etiology. The two most common presentations of CTCL are mycosis fungoides (MF) and Sézary syndrome (SS). However, both CTCL categories can easily resemble ATLL. Therefore, in HTLV-I endemic areas, differentiation between ATLL and CTCL must be performed, as they have different prognoses and treatment approaches.