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Adult T-cell leukemia/lymphoma (ATL or ATLL) is a rare cancer of the immune system's T-cells [1] [2] [3] caused by human T cell leukemia/lymphotropic virus type 1 (). [4] All ATL cells contain integrated HTLV-1 provirus further supporting that causal role of the virus in the cause of the neoplasm. [4]
Although the cause of T-cell lymphoma is not definitive, it has been associated with various risk factors and viruses such as Epstein–Barr virus (EBV) and human T-cell leukemia virus-1 (HTLV1). [2] The prognosis and treatment of T-cell lymphoma can vary drastically based on the specific type of lymphoma and its growth patterns.
Human T-cell lymphotropic virus type 1 or human T-lymphotropic virus (HTLV-I), also called the adult T-cell lymphoma virus type 1, is a retrovirus of the human T-lymphotropic virus (HTLV) family. Most people with HTLV-1 infection do not appear to develop health conditions that can be directly linked to the infection.
In childhood, T-cell acute lymphoblastic leukemia (T-ALL) patients can expect a 5-year event-free survival (EFS) rate of 70% and an overall survival (OS) rate of 80%. [1] Among the approximately 25% of children who relapse, survival rates drop to 30-50%, with patients generally showing a much poorer prognosis. [ 1 ]
Angioimmunoblastic T-cell lymphoma (AITL) is a fast-growing form of mature T-cell lymphoma, accounting for 18.5% of patients. [9] It is characterised by systemic disorders, polymorphous lymphoid infiltrate and a significant increase in proliferation of follicular dendritic cells and high endothelial venules. [10]
Large granular lymphocytic (LGL) leukemia is a chronic lymphoproliferative disorder that exhibits an unexplained, chronic (> 6 months) elevation in large granular lymphocytes (LGLs) in the peripheral blood. [1] It is divided in two main categories: T-cell LGL leukemia (T-LGLL) and natural-killer (NK)-cell LGL leukemia (NK-LGLL).
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