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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).
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.
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]
The T cell variations are usually caused by the prolonged use of T cell suppressant drugs, such as sirolimus, tacrolimus, or ciclosporin. [2] The Epstein-Barr virus , which infects >90% of the world population, is also a common cause of these disorders, being responsible for a wide range of non-malignant, pre-malignant, and malignant Epstein ...
The most common T-cell leukemia is precursor T-cell lymphoblastic leukemia. [1] It causes 15% of acute leukemias in childhood, and also 40% of lymphomas in childhood. [1] It is most common in adolescent males. [1] Its morphology is identical to that of precursor B-cell lymphoblastic leukemia. [1] Cell markers include TdT, CD2, CD7. [1]
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 ]
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