2017
DOI: 10.18632/oncotarget.22272
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Advances in the understanding and management of T-cell prolymphocytic leukemia

Abstract: T-prolymphocytic leukemia (T-PLL) is a rare T-cell neoplasm with an aggressive clinical course. Leukemic T-cells exhibit a post-thymic T-cell phenotype (Tdt−, CD1a−, CD5+, CD2+ and CD7+) and are generally CD4+/CD8−, but CD4+/CD8+ or CD8+/CD4− T-PLL have also been reported. The hallmark of T-PLL is the rearrangement of chromosome 14 involving genes for the subunits of the T-cell receptor (TCR) complex, leading to overexpression of the proto-oncogene TCL1. In addition, molecular analysis shows that T-PLL exhibit… Show more

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Cited by 24 publications
(29 citation statements)
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“…) . Lymphopoiesis starts with progenitor cells expressing CD2, CD5, CD7, and cytoplasmic CD3, which have the capacity to enter the cortex of the thymus . At this point, TCRs are formed after rearrangements of α, β, γ, and δ chains resulting in T cells with γδ‐chains (T‐γδ) and T cells with αβ‐chains (T‐αβ).…”
mentioning
confidence: 99%
“…) . Lymphopoiesis starts with progenitor cells expressing CD2, CD5, CD7, and cytoplasmic CD3, which have the capacity to enter the cortex of the thymus . At this point, TCRs are formed after rearrangements of α, β, γ, and δ chains resulting in T cells with γδ‐chains (T‐γδ) and T cells with αβ‐chains (T‐αβ).…”
mentioning
confidence: 99%
“…The leukemic lymphocytes in T-PLL are usually CD4+/CD8-, however, significant number are doubly positive for CD4 and CD8, or are positive for CD8 and rarely are doubly negative for CD4 and CD8. CD7 is usually expressed in T-PLL [6]. CD7 expression help in diagnosis of T-PLL as it is usually downregulated or lost in most other T cell neoplasms.…”
Section: Discussionmentioning
confidence: 99%
“…Alemtuzumab (Campath-1H) is a chimeric humanized antibody that binds to a glycoprotein (CD52), which is highly expressed on benign and malignant B and T-lymphocytes and monocytes (Buggins et al, 2002). Alemtuzumab showed an antitumor activity in various cancer types such as T-cell prolymphocytic leukemia and B-cell chronic lymphocytic leukemia (Laribi et al, 2017). In ATL, a phase 2 clinical study using Alemtuzumab in acute, chronic, and lymphoma subtypes resulted in an ORR of 52%, mostly in the blood, but unfortunately with a short duration of response (Sharma et al, 2017, reviewed in Hermine et al, 2018.…”
Section: Alemtuzumabmentioning
confidence: 99%