Clonal diseases of large granular lymphocytes.
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.
Loughran
T P
TP
Veterans Administration Hospital, Syracuse, NY 13210.
D017252:CD3 Complex / Q000032:analysis; D002999:Clone Cells; D005260:Female; D006410:Hematopoiesis; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D015461:Leukemia, Prolymphocytic, T-Cell / Q000473:pathology* / Q000628:therapy; D007958:Leukocyte Count; D016131:Lymphocyte Subsets; D008232:Lymphoproliferative Disorders / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D013601:T-Lymphocytes / Q000473:pathology*
D017252:CD3 Complex