Abstract
Adult T-cell leukemia/lymphoma (ATLL) is a peripheral T-cell lymphoma caused by human T-cell leukemia/lymphoma virus type 1 (HTLV-1). ATLL occurs in approximately 3%–5% of HTLV-1 carriers during their lifetime and follows a heterogeneous clinical course. The Shimoyama classification has been frequently used for treatment decisions in ATLL patients, and antiviral therapy has been reportedly promising, particularly in patients with indolent type ATLL; however, the prognosis continues to be dismal for patients with aggressive-type ATLL. Recent efforts to improve treatment outcomes have been focused on the development of prognostic stratification and improved dosage, timing, and combination of therapeutic modalities, such as antiviral therapy, chemotherapy, allogeneic hematopoietic stem cell transplantation, and molecular targeted therapy.
Highlights
Adult T-cell leukemia/lymphoma (ATLL) was first described in 1977 by Uchiyama et al [1], as a distinct clinical entity frequently observed in southwestern Japan
Indolent-type ATLL: Smoldering- or favorable chronic-type (1) Watchful waiting for asymptomatic patients (2) Interferon-α (IFN-α)/zidovudine (AZT) or watchful waiting for symptomatic patients (3) Skin lesion: Local therapy; Topical steroids, Ultraviolet light, Radiation Systemic therapy; Steroids, Oral retinoids, Single agent chemotherapy 2
A meta-analysis of 254 ATLL patients, including 116 patients with acute type, 100 with lymphoma type, 18 with chronic type, and 11 with smoldering type, reported that patients with acute, chronic, and smoldering leukemic-type ATLL had better outcomes with first-line antiviral therapy alone, whereas chemotherapy was more effective in patients with lymphoma-type ATLL [14]
Summary
Adult T-cell leukemia/lymphoma (ATLL) was first described in 1977 by Uchiyama et al [1], as a distinct clinical entity frequently observed in southwestern Japan. The causative agent of ATLL is the retrovirus human T-cell leukemia virus type I (HTLV-1) [2], which causes several immune-associated diseases, including HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) [3]. ATLL develops in approximately 3%–5% of HTLV-1 carriers and has a dismal prognosis. The clinical manifestations and the course of disease in ATLL patients vary to a great extent. Recent efforts to improve treatment outcomes in ATLL patients have been focused on the development of prognostic stratification and therapeutic modalities. Recent advances in ATLL treatment including antiviral therapy, chemotherapy, allogeneic hematopoietic stem cell transplantation (allo-HSCT), and molecular targeted therapy are discussed
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