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dc.identifier.citation2012, pp. 538 - 551en_US
dc.description.abstractT- and B-cell subtypes of prolymphocytic leukemia (PLL) are rare, aggressive lymphoid malignancies with characteristic morphologic, immunophenotypic, cytogenetic, and molecular features. Recent studies have highlighted the role of specific oncogenes, such as TCL-1, MTCP-1, and ATM in the case of T-cell and TP53 mutations in the case of B-cell prolymphocytic leukemia. Despite the advances in the understanding of the biology of these conditions, the prognosis for these patients remains poor with short survival and no curative therapy. The advent of monoclonal antibodies has improved treatment options. Currently, the best treatment for T-PLL is intravenous alemtuzumab, which has resulted in very high response rates of more than 90% when given as first-line treatment and a significant improvement in survival. Consolidation of remissions with autologous or allogeneic stem cell transplantation further prolongs survival, and the latter may offer potential cure. In B-PLL, rituximab-based combination chemo-immunotherapy is effective in fitter patients. TP53 abnormalities are common and, as for chronic lymphocytic leukemia, these patients should be managed using an alemtuzumab-based therapy. The role of allogeneic transplant with nonmyeloablative conditioning needs to be explored further in both T-and B-cell PLL to broaden the patient eligibility for what may be a curative treatment. (Blood. 2012;120(3):538-551)en_US
dc.format.extent538 - 551en_US
dc.subjectchronic lymphocytic-leukemia stem-cell transplantation t-cell marrow-transplantation ataxia-telangiectasia laboratory features b-pll expression gene abnormalitiesen_US
dc.titleHow I treat prolymphocytic leukemiaen_US
pubs.notesISI Document Delivery No.: 987TH Times Cited: 0 Cited Reference Count: 75 Dearden, Claire Amer soc hematology Washingtonen_US
pubs.notesNot knownen_US
pubs.embargo.termsNot knownen_US
dc.contributor.icrauthorDearden, Claireen_US

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