Large T-cell granular lyohocytic leulemia is a rare form of chronic lymphocytic leukemia that makes up 2 to 3% of CLL. Approximately 85 percent of? LGLs are CD3 positive, CD57 positive, CD56 negative T cells, representing in vivo antigen-activated effector-memory cytotoxic T cells and teh reamidner are CD3 negative NK-cell lineage. The rarity of these diseases and the lack of randomized trials mean that there is no consensus about optimal therapy for T- and NK-cell neoplasms and recommendations are therefore based on small case series, phase II trials and expert opinion. First line treatment ususally includes Cytoxan and methotrexate. For second line, Campath and pentostatin have been reported to be active.
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Dearden CE, Johnson R, Pettengell R, et al. Guidelines for the management of mature T-cell and NK-cell neoplasms (excluding cutaneous T-cell lymphoma). Br J Haematol 2011; 153:451.
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J Mercieca, E Matutes, C Dearden, K MacLennan and D Catovsky The role of pentostatin in the treatment of T-cell malignancies: analysis of response rate in 145 patients according to disease subtype.
[High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H
Blood Sep 15, 2001:1721-1726
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