Many people who were told that they have T-cell CLL in fact have LGL leukemia. In the Blood of 10th March there was an article on the disease by Thomas Loughran Jnr from Penn State Hershey Cancer Institute, the world expert on the condition.
LGL leukemia is characterized by a clonal expansion of either CD3+ cytotoxic T cells or CD3- NK cells. The phenotype of the cells suggests a chronic antigen driven immune response as initiator of the disease. LGL survival is promoted by the growth factors PDGF and IL-15 which produce resistance to apoptosis.
Clinical features of LGL leukemia include neutropenia, anemia and associated rheumatoid arthritis (RA). Treatment usually becomes necessary because of symptomatic neutropenia or severe RA and is usually begun with low-dose methotrexate. Failure of this agent generally means a switch to low dose cyclophosphamide and then to cyclsporin.
LGL leukemia is usually an indolent condition and m,any cases do not require treatment. Death, which is rare, is usually from neutropenic sepsis.