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- W2948582372 abstract "Prolymphocytic leukaemias B-PLL and T-PLL are rare disorders, typically with an aggressive clinical course and poor prognosis. Combining morphology, immunophenotyping, cytogenetic and molecular diagnostics reliably separates B-PLL and T-PLL from one another and other disorders. In T-PLL discovery of frequent mutations in the JAK-STAT pathway have increased understanding of disease pathogenesis. Alemtuzumab (anti-CD52) produces excellent response rates but long-term remissions are only achieved in a minority following consolidation with allogeneic stem cell transplant. Molecular abnormalities in B-PLL are less understood. Disruption of TP53 is a key finding, conveying chemotherapy resistance requiring novel therapies such as B-cell receptor inhibitors (BCRi). Both conditions require improved pathobiological knowledge to identify new treatment targets and guide therapy with novel pathway inhibitors." @default.
- W2948582372 created "2019-06-14" @default.
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- W2948582372 date "2019-09-01" @default.
- W2948582372 modified "2023-10-17" @default.
- W2948582372 title "B and T cell prolymphocytic leukaemia" @default.
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- W2948582372 doi "https://doi.org/10.1016/j.beha.2019.06.001" @default.
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