Large T-cell granular lymphocytic 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 the reminder 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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[High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H
Blood Sep 15, 2001:1721-1726