Medline ® Abstract for Reference 1
of 'Clinical manifestations, pathologic features, and diagnosis of T cell large granular lymphocyte leukemia'
Clonal diseases of large granular lymphocytes.
Loughran TP Jr
Three distinct clinical syndromes occur in patients with increased numbers of circulating LGL. Patients with T-LGL leukemia have clonal proliferations of CD3+ LGL typically associated with chronic neutropenia and autoimmune features. NK-LGL leukemia is characterized by clonal CD3- LGL proliferation with an acute clinical presentation marked by massive hepatosplenomegaly and systemic illness. However, most patients with increased numbers of CD3- LGL do not have clinical features of NK-LGL leukemia and have a chronic clinical course. X-linked gene analyses have supported a polyclonal LGL lymphocytosis in this syndrome. Further studies are needed to determine whether clonal progression can occur in these patients.
Veterans Administration Hospital, Syracuse, NY 13210.