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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]
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 ]
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.
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).
Anaplastic large cell lymphoma (ALCL) is a subtype of mature T-cell lymphoma involving T-cells or natural killer (NK) cells, representing 12% of patients. [9] It can be recognized by a constant expression of the tumour receptor necrosis factor CD30 , a membrane protein expressed by activated T and B cells, in the cancer cells. [ 13 ]
T-cell leukemia describes several different types of lymphoid leukemia which affect T cells. Types include: [1] Large granular lymphocytic leukemia; Adult T-cell leukemia/lymphoma; T-cell prolymphocytic leukemia; In practice, it can be hard to distinguish T-cell leukemia from T-cell lymphoma, and they are often grouped together.
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